interventions for clients with hematologic problems ii

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Interventions for Interventions for Clients with Clients with Hematologic Hematologic Problems II Problems II

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Page 1: Interventions for Clients with Hematologic Problems II

Interventions for Interventions for Clients with Clients with Hematologic Hematologic Problems IIProblems II

Page 2: Interventions for Clients with Hematologic Problems II

White blood cellsWhite blood cells White blood cells (WBCs), or White blood cells (WBCs), or leukocytes, leukocytes, provide protection from invading provide protection from invading

non-self cells and cancer cells in several ways. non-self cells and cancer cells in several ways.

These protective These protective functions depend onfunctions depend onmaintaining normal numbers maintaining normal numbers and ratios of many specific and ratios of many specific mature, circulating leukocytes. mature, circulating leukocytes.

When any one type of When any one type of WBC is present in eitherWBC is present in either abnormally high or abnormally abnormally high or abnormally low amounts, hematopoieticlow amounts, hematopoietic function and immune functionfunction and immune function may be altered to some degree, may be altered to some degree, placing clients at risk for specificplacing clients at risk for specific complicationscomplications

Page 3: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIA OverviewOverview The leukemias are a group of malignant disorders involving abnormal The leukemias are a group of malignant disorders involving abnormal

overproduction of a specific WBC type, usually at an immature stage, overproduction of a specific WBC type, usually at an immature stage, in the bone marrow.in the bone marrow.

Leukemia Leukemia may be may be acute, acute, with a sudden onset and short duration, or with a sudden onset and short duration, or chronic, chronic, with a slow onset and persistent symptoms over a period of with a slow onset and persistent symptoms over a period of yearsyears

Leukemias are categorized by the specific maturational pathway from Leukemias are categorized by the specific maturational pathway from which the abnormal cells arise. which the abnormal cells arise.

Leukemias in which the abnormal cells arise from within the Leukemias in which the abnormal cells arise from within the committed lymphoid maturational pathways are committed lymphoid maturational pathways are lymphocytic lymphocytic or or lymphoblastic. lymphoblastic.

Leukemias in which the abnormal cells arise within the committed Leukemias in which the abnormal cells arise within the committed myeloid maturational pathways are myeloid maturational pathways are myelocytic myelocytic or or myelogenous. myelogenous.

Several subtypes exist for each of these diseases, which are classified Several subtypes exist for each of these diseases, which are classified according to the degree of maturity of the abnormal cell and the according to the degree of maturity of the abnormal cell and the specific cell type involvedspecific cell type involved

Page 4: Interventions for Clients with Hematologic Problems II
Page 5: Interventions for Clients with Hematologic Problems II

Differentiating characteristics of the four types of leukemia

Page 6: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIA

PathophysiologyPathophysiology The basic problem in leukemia is a malignant transformation of The basic problem in leukemia is a malignant transformation of

the stem cells or early committed precursor leukocyte cells, the stem cells or early committed precursor leukocyte cells, causing an abnormal proliferation of a specific type of leukocyte. causing an abnormal proliferation of a specific type of leukocyte.

The functionally and structurally abnormal immature leukocytes, The functionally and structurally abnormal immature leukocytes, produced in excessive quantities in the bone marrow, essentially produced in excessive quantities in the bone marrow, essentially shut down normal bone marrow production of erythrocytes, shut down normal bone marrow production of erythrocytes, platelets, and other mature leukocytes. platelets, and other mature leukocytes.

This situation leads to anemia, thrombocytopenia, and leukopenia This situation leads to anemia, thrombocytopenia, and leukopenia of the unaffected WBC types, even though the number of of the unaffected WBC types, even though the number of immature, abnormal WBCs in the circulation is greatly elevated. immature, abnormal WBCs in the circulation is greatly elevated.

Without treatment, the client usually dies of infection or Without treatment, the client usually dies of infection or hemorrhage. For clients with acute leukemias, these pathologic hemorrhage. For clients with acute leukemias, these pathologic changes occur rapidly and, without intervention, progress quickly changes occur rapidly and, without intervention, progress quickly to death. Chronic leukemia may be present for many years before to death. Chronic leukemia may be present for many years before overt pathologic changes occurovert pathologic changes occur

Page 7: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIA

EtiologyEtiology ionizing radiationionizing radiation chemicals and drugschemicals and drugs marrow marrow hypoplasiahypoplasia (slow functioning with (slow functioning with less than the normal less than the normal production rate of blood cells)production rate of blood cells) environmental interactionsenvironmental interactions genetic factorsgenetic factors viral factorsviral factors immunologic factorsimmunologic factors the interaction of these factors the interaction of these factors

Page 8: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIAHistoryHistory The nurse asks the client about: The nurse asks the client about:

– risk factors and causative factors; risk factors and causative factors; – age; age; – occupation and hobbies; occupation and hobbies; – previous illnesses and the medical history (may indicate previous illnesses and the medical history (may indicate

exposure to ionizing radiation or medications that increase exposure to ionizing radiation or medications that increase risk); risk);

– frequency and severity of infectious processes (such as colds, frequency and severity of infectious processes (such as colds, influenza, pneumonia, bronchitis, or unexplained episodes of influenza, pneumonia, bronchitis, or unexplained episodes of fever) during the preceding 6 months;fever) during the preceding 6 months;

– any overt or hidden excessive bleeding episodes, such as the any overt or hidden excessive bleeding episodes, such as the following:following:

a tendency to bruise easilya tendency to bruise easily nosebleedsnosebleeds increased menstrual flowincreased menstrual flow bleeding from the gumsbleeding from the gums rectal bleedingrectal bleeding hematuria (blood in the urine)hematuria (blood in the urine) prolonged bleeding after minor abrasions or lacerationsprolonged bleeding after minor abrasions or lacerations

Page 9: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIA If the client has experienced such an episode, the nurse asks whether this If the client has experienced such an episode, the nurse asks whether this

type and extent of bleeding constitute the usual response to injury or type and extent of bleeding constitute the usual response to injury or represent a change.represent a change.

The client is asked whether he or she has The client is asked whether he or she has experienced any of the following:experienced any of the following:

– HeadachesHeadaches– Behavior changesBehavior changes– Increased somnolenceIncreased somnolence– Decreased alertnessDecreased alertness– Decreased attention spanDecreased attention span– Lethargy, muscle weaknessLethargy, muscle weakness– Diminished appetiteDiminished appetite– Weight lossWeight loss– Increased fatigueIncreased fatigue

Listing activities in the previous 24 hours may disclose additional Listing activities in the previous 24 hours may disclose additional information about activity intolerance, changes in behavior, and information about activity intolerance, changes in behavior, and unexplained fatigue. unexplained fatigue.

The nurse determines how long the client has had any of these debilitating The nurse determines how long the client has had any of these debilitating symptomssymptoms

Page 10: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIA

Page 11: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIA

Laboratory assessmentLaboratory assessment decreased hemoglobin and hematocrit levelsdecreased hemoglobin and hematocrit levels decreased platelet countdecreased platelet count altered white blood cell (WBC) count. altered white blood cell (WBC) count.

The WBC count may be low, normal, or elevated but usually The WBC count may be low, normal, or elevated but usually is quite elevated; counts of 20,000 to 100,000 are common. is quite elevated; counts of 20,000 to 100,000 are common. The client with a higher WBC count on diagnosis has a The client with a higher WBC count on diagnosis has a poorer prognosispoorer prognosis

The definitive test for leukemia includes various The definitive test for leukemia includes various examinations of cells obtained from bone marrow aspiration examinations of cells obtained from bone marrow aspiration and biopsy. The bone marrow is full of leukemic and biopsy. The bone marrow is full of leukemic blast blast phase cells phase cells (immature cells that are dividing). (immature cells that are dividing).

Page 12: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIA The composition of various proteins The composition of various proteins (antigens) (antigens) on the on the

surfaces of the leukemic cells helps diagnose the type of surfaces of the leukemic cells helps diagnose the type of leukemia. Such markers include the T11 protein, the leukemia. Such markers include the T11 protein, the enzyme enzyme terminal deoxynucleotidyl transferase (TDT), terminal deoxynucleotidyl transferase (TDT), and the and the common acute lymphoblastic leukemia common acute lymphoblastic leukemia antigen (CALLA). antigen (CALLA). These markers also indicate the These markers also indicate the prognosis.prognosis.

Blood-clotting times and factors are usually abnormal for the Blood-clotting times and factors are usually abnormal for the client with acute leukemia. Reduced levels of fibrinogen and client with acute leukemia. Reduced levels of fibrinogen and other coagulation factors are typical. Whole blood-clotting other coagulation factors are typical. Whole blood-clotting time (Lee-White clotting test) is increased, as is the time (Lee-White clotting test) is increased, as is the activated partial thromboplastin time (aPTT).activated partial thromboplastin time (aPTT).

Chromosome analysis of the malignant bone marrow cells Chromosome analysis of the malignant bone marrow cells may identify specific marker chromosomes to assist in the may identify specific marker chromosomes to assist in the diagnosis of the type of leukemia, predict the prognosis, and diagnosis of the type of leukemia, predict the prognosis, and determine the effectiveness of therapy. An example is the determine the effectiveness of therapy. An example is the Philadelphia chromosome, which is important in the Philadelphia chromosome, which is important in the diagnosis of chronic myelogenous leukemia (CML)diagnosis of chronic myelogenous leukemia (CML)

Page 13: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIARadiographic assessmentRadiographic assessment Specific symptoms determine the need for specific tests. Specific symptoms determine the need for specific tests.

In a client with dyspnea,In a client with dyspnea, a chest x-ray study is needed a chest x-ray study is needed to determine whether leukemic to determine whether leukemic infiltrates are present in the lung. infiltrates are present in the lung.

Skeletal x-ray films may Skeletal x-ray films may help to determine whetherhelp to determine whether bone bone resorption resorption (loss of (loss of bone minerals and density) bone minerals and density) is presentis present

Page 14: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIARisk for infectionRisk for infection Infection is a major cause of death in the Infection is a major cause of death in the immunosuppressed client, and septicemia is immunosuppressed client, and septicemia is a common complication. a common complication. Infection of the client with leukemia occursInfection of the client with leukemia occurs through both autocontamination (normal through both autocontamination (normal flora overgrows and penetrates the internal flora overgrows and penetrates the internal environment) and cross-contamination (microorganisms from environment) and cross-contamination (microorganisms from

another person or the environment are transmitted to the another person or the environment are transmitted to the client). client).

The three most common sites of infection are the skin, The three most common sites of infection are the skin, respiratory tract, and gastrointestinal tract.respiratory tract, and gastrointestinal tract.

Gram-negative bacteria are most often the cause of infection, Gram-negative bacteria are most often the cause of infection, although gram-positive and fungal infections do occur. although gram-positive and fungal infections do occur.

Interventions aim to interrupt or halt the process of infection Interventions aim to interrupt or halt the process of infection and control specific infections early.and control specific infections early.

Page 15: Interventions for Clients with Hematologic Problems II
Page 16: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIA DRUG THERAPY FOR LEUKEMIADRUG THERAPY FOR LEUKEMIA. Drug therapy for clients with . Drug therapy for clients with

AML is divided into three distinctive phases: in duction, AML is divided into three distinctive phases: in duction, consolidation, and maintenance.consolidation, and maintenance.

Induction therapy. Induction therapy. Induction therapy is intensive and consists of Induction therapy is intensive and consists of combination chemotherapy initiated at the time of diagnosis. This combination chemotherapy initiated at the time of diagnosis. This therapy is aimed at achieving a rapid, complete remission of all therapy is aimed at achieving a rapid, complete remission of all manifestations of disease. A typical course of aggressive manifestations of disease. A typical course of aggressive chemotherapy includes IV administration of cytosine arabinoside chemotherapy includes IV administration of cytosine arabinoside for 7 days with concomitant administration of daunorubicin for the for 7 days with concomitant administration of daunorubicin for the first 3 days.first 3 days.

A major side effect of these agents is severe bone marrow A major side effect of these agents is severe bone marrow suppression. As a result, the client becomes even more vulnerable suppression. As a result, the client becomes even more vulnerable to infection than before the treatment started. Prolonged to infection than before the treatment started. Prolonged hospitalizations are common while the client is immunosuppressed. hospitalizations are common while the client is immunosuppressed.

Recovery of bone marrow function requires at least 2 to 3 weeks, Recovery of bone marrow function requires at least 2 to 3 weeks, during which time the client must be protected from life-during which time the client must be protected from life-threatening infections. threatening infections.

Other adverse reactions include nausea, vomiting, diarrhea, Other adverse reactions include nausea, vomiting, diarrhea, alopecia (hair loss), stomatitis (mouth sores), kidney toxicity, liver alopecia (hair loss), stomatitis (mouth sores), kidney toxicity, liver toxicity, and cardiac toxicity.toxicity, and cardiac toxicity.

Page 17: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIA

Consolidation therapy. Consolidation therapy. Consolidation therapy usually Consolidation therapy usually consists of another course of either the same agents consists of another course of either the same agents used for induction at a different dosage or a different used for induction at a different dosage or a different combination of chemotherapeutic agents. This combination of chemotherapeutic agents. This treatment occurs early in remission, and its intent is to treatment occurs early in remission, and its intent is to cure. At some institutions, consolidation therapy is a cure. At some institutions, consolidation therapy is a single course of chemotherapy; at others, it involves single course of chemotherapy; at others, it involves regularly scheduled, repeated courses of regularly scheduled, repeated courses of chemotherapy for 1 to 2 years.chemotherapy for 1 to 2 years.

Maintenance therapy. Maintenance therapy. Maintenance therapy may be Maintenance therapy may be prescribed for months to years after successful prescribed for months to years after successful induction and consolidation therapies. It is commonly induction and consolidation therapies. It is commonly indicated for clients with acute lymphocytic leukemia indicated for clients with acute lymphocytic leukemia (ALL). The purpose is to maintain the remission (ALL). The purpose is to maintain the remission achieved through induction and consolidation. achieved through induction and consolidation. Maintenance agents are milder and are often given Maintenance agents are milder and are often given orally for 2 to 5 years.orally for 2 to 5 years.

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LEUKEMIALEUKEMIA

DRUG THERAPY FOR INFECTION. DRUG THERAPY FOR INFECTION. Drug Drug therapy is the primary defense against therapy is the primary defense against infections that develop in clients infections that develop in clients undergoing therapy for AML. undergoing therapy for AML.

Agents used depend on the sensitivity of Agents used depend on the sensitivity of the specific organism causing the the specific organism causing the infection, as well as the extent of the infection, as well as the extent of the infection, and are categorized by infection, and are categorized by specificity as specificity as

antibacterial, antiviral, or antifungal. antibacterial, antiviral, or antifungal.

Page 19: Interventions for Clients with Hematologic Problems II
Page 20: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIA Antibiotic and antibacterial agents.Antibiotic and antibacterial agents. Antibiotic and Antibiotic and

antibacterial agents used for prophylaxis or antibacterial agents used for prophylaxis or treatment of infection in clients with AML usually treatment of infection in clients with AML usually include at least one of the aminoglycoside antibiotics include at least one of the aminoglycoside antibiotics (amikacin, gentamicin, and tobramycin) and a (amikacin, gentamicin, and tobramycin) and a systemic penicillin. Additional, powerful antibiotics systemic penicillin. Additional, powerful antibiotics used may include vancomycin and drugs from the used may include vancomycin and drugs from the tetracycline and third-generation cephalosporin tetracycline and third-generation cephalosporin classes.classes.

Antifungal agents.Antifungal agents. Systemic antifungal agents, used Systemic antifungal agents, used when a fungal infection has been diagnosed or is when a fungal infection has been diagnosed or is strongly sug gested, include amphotericin B, strongly sug gested, include amphotericin B, ketoconazole (Nizoral), andketoconazole (Nizoral), and nystatin (Mycostatin, nystatin (Mycostatin, Nadostine, Nilstat). In neutropenic clients, antifungal Nadostine, Nilstat). In neutropenic clients, antifungal creams (e.g., miconazole nitrate) are admin istered creams (e.g., miconazole nitrate) are admin istered intravaginally to prevent yeast infectionsintravaginally to prevent yeast infections

Page 21: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIA

Antiviral agents.Antiviral agents. Antiviral agents are commonly used Antiviral agents are commonly used in clients with leukemia to prevent and treat viral in clients with leukemia to prevent and treat viral infections. Acyclovir is administered either orally or infections. Acyclovir is administered either orally or parenterally before the initiation of antineoplastic parenterally before the initiation of antineoplastic agents, especially in clients who are cytomegalovirus agents, especially in clients who are cytomegalovirus (CMV) positive. If a viral infection is suspected or (CMV) positive. If a viral infection is suspected or diagnosed with positive cultures, pharmacologic diagnosed with positive cultures, pharmacologic treatments may include ganciclovir, foscarnet, or treatments may include ganciclovir, foscarnet, or steroids. The antivirals, although helpful in steroids. The antivirals, although helpful in combating severe infections, are associated with a combating severe infections, are associated with a wide range of serious adverse effects, especially wide range of serious adverse effects, especially ototoxicity ototoxicity (disruption of hearing and/or balance) (disruption of hearing and/or balance) and and nephrotoxicity nephrotoxicity (disruption of kidney function). (disruption of kidney function). The nurse carefully monitors the client treated with The nurse carefully monitors the client treated with such drugs for signs of hearing impairment and renal such drugs for signs of hearing impairment and renal insufficiencyinsufficiency

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LEUKEMIALEUKEMIA

INFECTION PROTECTIONINFECTION PROTECTION SKIN CARESKIN CARE RESPIRATORY CARERESPIRATORY CARE BONE MARROW BONE MARROW

TRANSPLANTATIONTRANSPLANTATION

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Page 24: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIA Sources of stem cells.Sources of stem cells. BMT originated with the use of BMT originated with the use of allogeneic bone marrow allogeneic bone marrow

transplantation transplantation (transplantation of identical bone marrow from a sibling) and has (transplantation of identical bone marrow from a sibling) and has advanced to the use of human leukocyte antigen (HLA)-matched stem cells from the advanced to the use of human leukocyte antigen (HLA)-matched stem cells from the umbilical cords of unrelated donors.umbilical cords of unrelated donors.

Transplants can be classified based on the source of stem cells. Transplants can be classified based on the source of stem cells. In In autologous transplants, autologous transplants, the clients receive their own stem cells, which were the clients receive their own stem cells, which were

collected before therapy. collected before therapy. Syngeneic transplants Syngeneic transplants are rare and involve the client's own identical twin as the are rare and involve the client's own identical twin as the

donor of stem cells. donor of stem cells. In In allogeneic transplants, allogeneic transplants, a closely a closely HLA-matched sibling or an unrelated donor provides HLA-matched sibling or an unrelated donor provides the stem cells. Stem cells for transplantation may bethe stem cells. Stem cells for transplantation may be obtained by one of the following methods: bone obtained by one of the following methods: bone marrow harvest, peripheral stem cell pheresis, or marrow harvest, peripheral stem cell pheresis, or umbilical cord blood stem cellumbilical cord blood stem cell

Transplantation procedures have five phases:Transplantation procedures have five phases: stem cell procurement, conditioning regimen, stem cell procurement, conditioning regimen, transplantation, engraftment, and posttransplantation transplantation, engraftment, and posttransplantation recoveryrecovery

Page 25: Interventions for Clients with Hematologic Problems II
Page 26: Interventions for Clients with Hematologic Problems II

Timing and steps of allogenic bone marrow transplantation

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LEUKEMIALEUKEMIA

HOME CARE MANAGEMENTHOME CARE MANAGEMENT Planning for home care for the client with leukemia Planning for home care for the client with leukemia begins as soon as a client achieves remission. begins as soon as a client achieves remission. He or she will need assistance at home until theHe or she will need assistance at home until the condition improves. condition improves. The nurse assesses the available support mechanisms. Many The nurse assesses the available support mechanisms. Many

clients require the services of a visiting nurse to assist with clients require the services of a visiting nurse to assist with dressing changes for central venous catheters, to assist with dressing changes for central venous catheters, to assist with hyperalimentation infusions, to transfuse platelets, and to answer hyperalimentation infusions, to transfuse platelets, and to answer questions. Occasionally they may also require home transfusion questions. Occasionally they may also require home transfusion therapy for one or more blood components.therapy for one or more blood components.

The home care team is critical for the client receiving stem cell The home care team is critical for the client receiving stem cell transplantation in the home setting. Potential candidates are transplantation in the home setting. Potential candidates are evaluated in advance. Criteria include a knowledgeable caregiver, evaluated in advance. Criteria include a knowledgeable caregiver, a clean home environment, close proximity to the hospital, a clean home environment, close proximity to the hospital, telephone access, and emotional stability on the part of the client telephone access, and emotional stability on the part of the client and caregiver.and caregiver.

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LEUKEMIALEUKEMIA In one sample program, clients receive their daily In one sample program, clients receive their daily

dose of chemotherapy in the outpatient clinic in the dose of chemotherapy in the outpatient clinic in the morning and then receive a home visit in the morning and then receive a home visit in the evening. evening.

Home care nurses administer chemotherapy and Home care nurses administer chemotherapy and monitor the client for complications. monitor the client for complications.

Nurses visit the client once or twice a day and spend Nurses visit the client once or twice a day and spend between 4 and 8.5 hours per day in the home. between 4 and 8.5 hours per day in the home.

The client receives the stem cell transplant infusion The client receives the stem cell transplant infusion in the outpatient clinic. in the outpatient clinic.

Nursing care is similar to that provided Nursing care is similar to that provided in the hospital. If serious complications suchin the hospital. If serious complications such as sepsis or venoocclusive disease occur, as sepsis or venoocclusive disease occur, the client is admitted to the inpatient facilitythe client is admitted to the inpatient facility

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LEUKEMIALEUKEMIAHEALTH TEACHINGHEALTH TEACHING The client and the family The client and the family

need to be educated about need to be educated about the importance of continuing the importance of continuing therapy and appropriate therapy and appropriate medical follow-up, despite medical follow-up, despite the unpleasant side effects the unpleasant side effects of therapy. of therapy.

Many clients go home with a Many clients go home with a central venous catheter in central venous catheter in place and require place and require instructions about its care instructions about its care and maintenance. These and maintenance. These guidelines may be altered guidelines may be altered depending on the home depending on the home setting, assistance available, setting, assistance available, and agency policyand agency policy

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LEUKEMIALEUKEMIA

Protecting the client from infection after Protecting the client from infection after discharge from the hospital is just as important as discharge from the hospital is just as important as it was during hospitalization. it was during hospitalization.

The nurse urges the client to use proper hygiene The nurse urges the client to use proper hygiene and to avoid crowds or others with infections. and to avoid crowds or others with infections. Neither the client nor any household member Neither the client nor any household member should receive live virus immunization should receive live virus immunization (poliomyelitis, measles, or rubella) for 2 years (poliomyelitis, measles, or rubella) for 2 years after transplantation. after transplantation.

The client should continue mouth care regimens The client should continue mouth care regimens at home. at home.

The nurse emphasizes that the client should The nurse emphasizes that the client should immediately notify the physician if he or she immediately notify the physician if he or she experiences fever or any other sign of infection. experiences fever or any other sign of infection.

Page 31: Interventions for Clients with Hematologic Problems II
Page 32: Interventions for Clients with Hematologic Problems II

LEUKEMIALEUKEMIA

Because platelet recovery is usually slower than recovery of Because platelet recovery is usually slower than recovery of white blood cells (WBCs), many clients return home still at white blood cells (WBCs), many clients return home still at risk for bleeding. risk for bleeding.

Thrombocytopenia may be present for 6 months following Thrombocytopenia may be present for 6 months following transplantation. transplantation.

The nurse reinforces the safety and bleeding precautions The nurse reinforces the safety and bleeding precautions initiated in the hospital, emphasizing that the client must initiated in the hospital, emphasizing that the client must follow these precautions until the platelet count is above follow these precautions until the platelet count is above 50,000. 50,000.

The client and family are instructed to assess for petechiae, The client and family are instructed to assess for petechiae, avoid trauma and sharp objects, apply pressure to wounds avoid trauma and sharp objects, apply pressure to wounds for 10 minutes, and report any unusual symptoms, for 10 minutes, and report any unusual symptoms, including blood in the stool or urine, or headache that does including blood in the stool or urine, or headache that does not respond to acetaminophen. not respond to acetaminophen.

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Page 34: Interventions for Clients with Hematologic Problems II

MALIGNANT LYMPHOMAMALIGNANT LYMPHOMA

– Malignant lymphomas occur as a result of abnormal overgrowth Malignant lymphomas occur as a result of abnormal overgrowth of one type of leukocyte (lymphocytes); they differ from the of one type of leukocyte (lymphocytes); they differ from the leukemias in the degree of maturation of the affected cells and leukemias in the degree of maturation of the affected cells and the location of cell production. the location of cell production.

– Lymphomas are malignancies characterized by a proliferation of Lymphomas are malignancies characterized by a proliferation of committed lymphocytes rather than stem cell precursors (as in committed lymphocytes rather than stem cell precursors (as in leukemia). leukemia).

This proliferation occurs not in bone marrow but in This proliferation occurs not in bone marrow but in other lymphoid tissues scattered throughout the other lymphoid tissues scattered throughout the body, especially the lymph nodes and spleen. body, especially the lymph nodes and spleen.

Lymphomas are actually solid tumors rather than Lymphomas are actually solid tumors rather than cellular suspensions within the blood andcellular suspensions within the blood and bone bone marrow, and they fall into two major categories marrow, and they fall into two major categories among adults: Hodgkin's lymphoma and non-among adults: Hodgkin's lymphoma and non-Hodgkin's lymphomaHodgkin's lymphoma

Page 35: Interventions for Clients with Hematologic Problems II

Hodgkin's LymphomaHodgkin's Lymphoma Hodgkin's lymphoma is a cancer that can affect any age group, Hodgkin's lymphoma is a cancer that can affect any age group,

although incidence peaks first in people in their mid-to-late 20s and although incidence peaks first in people in their mid-to-late 20s and then in people older than 50 years of age. then in people older than 50 years of age.

Men and women are affected equally in the first group, but the Men and women are affected equally in the first group, but the disease is more prevalent in men in the older group.disease is more prevalent in men in the older group.

Factors implicated as possible causes of Hodgkin's lymphoma Factors implicated as possible causes of Hodgkin's lymphoma include viral infections and previous exposure to alkylating chemical include viral infections and previous exposure to alkylating chemical agents. agents.

This cancer usually originates in a single lymph node or a single This cancer usually originates in a single lymph node or a single chain of nodes. The lymphoid tissues within the node undergo chain of nodes. The lymphoid tissues within the node undergo malignant transformation, usually initiating some inflammatory malignant transformation, usually initiating some inflammatory processes. These nodes contain a specific transformed cell type, the processes. These nodes contain a specific transformed cell type, the Reed-Sternberg cell, Reed-Sternberg cell, a marker for Hodgkin's lymphoma. a marker for Hodgkin's lymphoma.

The disease first The disease first metastasizes metastasizes (spreads) to other nearby lymphoid (spreads) to other nearby lymphoid structures and eventually invades nonlymphoid tissuesstructures and eventually invades nonlymphoid tissues

Page 36: Interventions for Clients with Hematologic Problems II

Hodgkin's LymphomaHodgkin's Lymphoma

AssessmentAssessment Assessment most often reveals a greatly enlarged but painless Assessment most often reveals a greatly enlarged but painless

lymph node or nodes, usually the earliest manifestation of Hodgkin's lymph node or nodes, usually the earliest manifestation of Hodgkin's lymphoma. The client also often experiences fever, malaise, and lymphoma. The client also often experiences fever, malaise, and night sweats. More specific clinical manifestations depend on the site night sweats. More specific clinical manifestations depend on the site (or sites) of malignancy and the extent of disease.(or sites) of malignancy and the extent of disease.

Diagnosis and grade are established when biopsy of a node or mass Diagnosis and grade are established when biopsy of a node or mass reveals Reed-Sternberg cells. reveals Reed-Sternberg cells.

The client then undergoes extensive staging procedures to The client then undergoes extensive staging procedures to determine the exact extent of disease. Staging must be detailed and determine the exact extent of disease. Staging must be detailed and accurate because the treatment regimen is determined by the accurate because the treatment regimen is determined by the extent of disease. extent of disease.

Staging procedures for Hodgkin's lymphoma include biopsies of Staging procedures for Hodgkin's lymphoma include biopsies of distant lymph nodes, computed tomography (CT) of the thorax and distant lymph nodes, computed tomography (CT) of the thorax and abdomen, staging laparotomy, a complete blood count (CBC), liver abdomen, staging laparotomy, a complete blood count (CBC), liver function studies, and bilateral bone marrow biopsiesfunction studies, and bilateral bone marrow biopsies

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Manifestations and staging criteria for Hodgkin's lymphoma

Page 38: Interventions for Clients with Hematologic Problems II

Hodgkin's LymphomaHodgkin's Lymphoma

InterventionsInterventions Hodgkin's lymphoma is now one of the most Hodgkin's lymphoma is now one of the most

curable types of cancer. curable types of cancer.

Generally, for stage I and stage II disease without Generally, for stage I and stage II disease without mediastinal node involvement, the treatment of mediastinal node involvement, the treatment of choice is extensive external radiation of involved choice is extensive external radiation of involved lymph node regions. lymph node regions.

With more extensive disease, radiation coupled With more extensive disease, radiation coupled with an aggressive multiagent chemotherapy with an aggressive multiagent chemotherapy regimen is most effective in achieving a cureregimen is most effective in achieving a cure

Page 39: Interventions for Clients with Hematologic Problems II

Hodgkin's Hodgkin's LymphomaLymphoma

Specific nursing management of the client undergoing Specific nursing management of the client undergoing treatment for Hodgkin's lymphoma focuses on the side treatment for Hodgkin's lymphoma focuses on the side effects of therapy, especially the following:effects of therapy, especially the following:– Drug-induced pancytopenia, which results in increased risk for Drug-induced pancytopenia, which results in increased risk for

infection, bleeding, and anemiainfection, bleeding, and anemia– Severe nausea and vomitingSevere nausea and vomiting– Skin irritation and breakdown at the site of radiationSkin irritation and breakdown at the site of radiation – Impaired hepatic function either by metastasis to the liver or Impaired hepatic function either by metastasis to the liver or

by multiagent chemotherapyby multiagent chemotherapy– Permanent sterility for male clients receiving radiation to the Permanent sterility for male clients receiving radiation to the

abdominopelvic region in the pattern of an inverted Y in abdominopelvic region in the pattern of an inverted Y in combination with specific chemotherapeutic agents (the client combination with specific chemotherapeutic agents (the client should be informed of this side effect and given the option to should be informed of this side effect and given the option to store sperm in a sperm bank be store sperm in a sperm bank be fore treatment)fore treatment)

– Secondary malignancies for clients receiving radiation alone or Secondary malignancies for clients receiving radiation alone or chemotherapy (Long-term follow-up should include screening chemotherapy (Long-term follow-up should include screening for recurrence, as well as the possible development of a for recurrence, as well as the possible development of a secondary cancer.)secondary cancer.)

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Non-Hodgkin's Non-Hodgkin's LymphomaLymphoma

Non-Hodgkin's lymphoma is the classification for all cancers originating Non-Hodgkin's lymphoma is the classification for all cancers originating from lymphoid tissues that are not diagnosed as Hodgkin's lymphoma. from lymphoid tissues that are not diagnosed as Hodgkin's lymphoma.

There are more than 12 subtypes of non-Hodgkin's lymphoma, including There are more than 12 subtypes of non-Hodgkin's lymphoma, including low-grade, intermediate, and high-grade lymphomas.low-grade, intermediate, and high-grade lymphomas.

The low-grade lymphomas usually arise from B-cell lymphocytes and The low-grade lymphomas usually arise from B-cell lymphocytes and progress slowly. Although clients with low-grade lymphomas have longer progress slowly. Although clients with low-grade lymphomas have longer survival rates, the diseases are less responsive to treatment and, survival rates, the diseases are less responsive to treatment and, consequently, cures are rare.consequently, cures are rare.

At the other end of the spectrum are the high-grade lymphomas, which are At the other end of the spectrum are the high-grade lymphomas, which are aggressive tumors of usually mixed cellularity with rapid doubling times. aggressive tumors of usually mixed cellularity with rapid doubling times. High-grade lymphomas are more responsive to chemotherapy, and the High-grade lymphomas are more responsive to chemotherapy, and the chances for a longterm cure are greater.chances for a longterm cure are greater.

Most non-Hodgkin's lymphomas arise from lymph nodes, but they can Most non-Hodgkin's lymphomas arise from lymph nodes, but they can originate in virtually any tissue or organ. A low-grade lymphoma also can originate in virtually any tissue or organ. A low-grade lymphoma also can convert to a higher-grade lymphoma. Definitive causes are unknown, but convert to a higher-grade lymphoma. Definitive causes are unknown, but viral infection, exposure to ionizing radiation, autoimmune disorders, and viral infection, exposure to ionizing radiation, autoimmune disorders, and exposure to toxic chemicals have all been implicatedexposure to toxic chemicals have all been implicated

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Non-Hodgkin's Non-Hodgkin's LymphomaLymphoma

Because lymphomas may arise from lymphoid cells in any tissue Because lymphomas may arise from lymphoid cells in any tissue and because the malignancy can spread to any organ, assessment and because the malignancy can spread to any organ, assessment reveals no specific clinical manifestations other than reveals no specific clinical manifestations other than lymphadenopathy common to all types of lymphoma. lymphadenopathy common to all types of lymphoma.

Diagnosis is made from the histologic features apparent on biopsy Diagnosis is made from the histologic features apparent on biopsy of any suspicious node or mass. of any suspicious node or mass.

Classification of the specific lymphoma subtype is based on a Classification of the specific lymphoma subtype is based on a complex grading of surface markers, cytogenetic features, cell complex grading of surface markers, cytogenetic features, cell size, and expression of viral antigens. Staging is similar to that size, and expression of viral antigens. Staging is similar to that

for Hodgkin's lymphoma.for Hodgkin's lymphoma.

Treatment consists of radiation therapy and multiagent Treatment consists of radiation therapy and multiagent chemotherapy. Nursing care needs are similar to thosechemotherapy. Nursing care needs are similar to those for clients with Hodgkin's lymphoma, with additional for clients with Hodgkin's lymphoma, with additional organ-specific problems taken into account if the disease is widely organ-specific problems taken into account if the disease is widely

disseminateddisseminated

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COAGULATION COAGULATION DISORDERSDISORDERS

Coagulation disorders are synonymous with Coagulation disorders are synonymous with bleeding disorders and are characterized by bleeding disorders and are characterized by abnormal or increased bleeding resulting from abnormal or increased bleeding resulting from defects in one or more components regulating defects in one or more components regulating hemostasis. hemostasis.

Bleeding disorders may be spontaneous or Bleeding disorders may be spontaneous or traumatic, localized or generalized, lifelong or traumatic, localized or generalized, lifelong or acquired. acquired.

They can originate from a defectThey can originate from a defect in the hemostatic processes at in the hemostatic processes at the vascular, platelet, or clotting the vascular, platelet, or clotting factor level.factor level.

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PLATELET DISORDERSPLATELET DISORDERS Platelets play a vital role in hemostasis. For both the intrinsic and the Platelets play a vital role in hemostasis. For both the intrinsic and the

extrinsic pathways, blood clotting starts with platelet adhesion and the extrinsic pathways, blood clotting starts with platelet adhesion and the formation of a platelet plug. Any condition that either reduces the formation of a platelet plug. Any condition that either reduces the number of platelets or interferes with their ability to adhere (to one number of platelets or interferes with their ability to adhere (to one another, blood vessel walls, collagen, or fibrin threads) can be another, blood vessel walls, collagen, or fibrin threads) can be manifested as increased bleeding. manifested as increased bleeding.

Platelet disorders can be inherited, acquired, or temporarily induced by Platelet disorders can be inherited, acquired, or temporarily induced by the ingestion of substances that limit platelet production or inhibit the ingestion of substances that limit platelet production or inhibit aggregation.aggregation.

A drop in the number of platelets below the level needed for normal A drop in the number of platelets below the level needed for normal coagulation is called coagulation is called thrombocytopenia. thrombocytopenia. Thrombocytopenia may Thrombocytopenia may occur as a result of other conditions or treatments that suppress occur as a result of other conditions or treatments that suppress general bone marrow activity. It also can occur through processes that general bone marrow activity. It also can occur through processes that specifically limit platelet formation or increase the rate of platelet specifically limit platelet formation or increase the rate of platelet destruction. destruction.

The two thrombocytopenic conditions affecting adults are autoimmune The two thrombocytopenic conditions affecting adults are autoimmune thrombocytopenic purpura and thrombotic thrombocytopenic purpurathrombocytopenic purpura and thrombotic thrombocytopenic purpura

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Autoimmune Thrombocytopenic Autoimmune Thrombocytopenic PurpuraPurpura

Clients with idiopathic thrombocytopenic purpura make an Clients with idiopathic thrombocytopenic purpura make an antibody directed against the surface of their own platelets antibody directed against the surface of their own platelets (an antiplatelet antibody). This antibody coats the surface (an antiplatelet antibody). This antibody coats the surface of the platelets, making them more susceptible to of the platelets, making them more susceptible to attraction and destruction by phagocytic leukocytes, attraction and destruction by phagocytic leukocytes, especially macrophages. especially macrophages.

Because the spleen contains a large concentration of Because the spleen contains a large concentration of macrophages and because the blood vessels of the spleen macrophages and because the blood vessels of the spleen are long and twisted, antibody-coated platelets are are long and twisted, antibody-coated platelets are destroyed primarily in the spleen. destroyed primarily in the spleen.

When the rate of platelet destruction When the rate of platelet destruction exceeds that of production, the numberexceeds that of production, the number of circulating platelets decreases and of circulating platelets decreases and blood clotting slows.blood clotting slows.

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Autoimmune Autoimmune Thrombocytopenic PurpuraThrombocytopenic Purpura

AssessmentAssessment Clinical manifestations associated with ITP are generally Clinical manifestations associated with ITP are generally

limited to the skin and mucous membranes: large limited to the skin and mucous membranes: large ecchymoses ecchymoses (bruises) on the arms, legs, upper chest, and (bruises) on the arms, legs, upper chest, and neck or a petechial rash. neck or a petechial rash.

Mucosal bleeding occurs easily. If the client has experienced Mucosal bleeding occurs easily. If the client has experienced significant blood loss, signs of anemia may also be present.significant blood loss, signs of anemia may also be present.

A rare complication is an intracranial bleeding-induced stroke. A rare complication is an intracranial bleeding-induced stroke.

The nurse assesses for neurologic function and mental status. The nurse assesses for neurologic function and mental status.

Family members or significant others are asked if the client's Family members or significant others are asked if the client's behavior and responses to the mental status examination are behavior and responses to the mental status examination are typical or represent a change from usual reactions.typical or represent a change from usual reactions.

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Autoimmune Thrombocytopenic Autoimmune Thrombocytopenic PurpuraPurpura

Idiopathic thrombocytopenic purpura is Idiopathic thrombocytopenic purpura is diagnosed by a decreased platelet count diagnosed by a decreased platelet count and large numbers of megakaryocytes in and large numbers of megakaryocytes in the bone marrow. the bone marrow.

Antiplatelet antibodies Antiplatelet antibodies may be present in detectablemay be present in detectablelevels in peripheral blood. levels in peripheral blood. If the client experiences If the client experiences any episodes of bleeding,any episodes of bleeding,hematocrit and hemoglobin hematocrit and hemoglobin levels also are lowlevels also are low

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Autoimmune Autoimmune Thrombocytopenic PurpuraThrombocytopenic Purpura

NONSURGICAL MANAGEMENTNONSURGICAL MANAGEMENT DRUG THERAPY. DRUG THERAPY. Agents used to control ITP include drugs Agents used to control ITP include drugs

that suppress immune function to some degree. The that suppress immune function to some degree. The premise for the use of agents such as corticosteroids and premise for the use of agents such as corticosteroids and azathioprine (Imuran) is to inhibit immune system synthesis azathioprine (Imuran) is to inhibit immune system synthesis of antiplatelet autoantibodies. More aggressive therapy can of antiplatelet autoantibodies. More aggressive therapy can include low doses of chemotherapeutic agents, such as the include low doses of chemotherapeutic agents, such as the an-timitotic agents and cyclophosphamidean-timitotic agents and cyclophosphamide

BLOOD REPLACEMENT THERAPY. BLOOD REPLACEMENT THERAPY. For the client For the client with a platelet count of less than 20,000/mmwith a platelet count of less than 20,000/mm33 who who is experiencing an acute life-threatening bleeding is experiencing an acute life-threatening bleeding episode, a platelet transfusion may be required. episode, a platelet transfusion may be required. Platelet transfusions are not performed routinely, Platelet transfusions are not performed routinely, because the donated platelets are just as rapidly because the donated platelets are just as rapidly destroyed by the spleen as the client's own destroyed by the spleen as the client's own platelets.platelets.

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Autoimmune Thrombocytopenic Autoimmune Thrombocytopenic PurpuraPurpura

MAINTAINING A SAFE ENVIRONMENT. MAINTAINING A SAFE ENVIRONMENT. The nurse's The nurse's major objectives are to protect the client from major objectives are to protect the client from situations that can lead to bleeding and to closely situations that can lead to bleeding and to closely monitor the amount of bleeding that is occurring.monitor the amount of bleeding that is occurring.

SURGICAL MANAGEMENT. For the client who does SURGICAL MANAGEMENT. For the client who does not respond to drug therapy, splenectomy may not respond to drug therapy, splenectomy may be the treatment of choice. Because the be the treatment of choice. Because the leukocytes in the spleen perform many different leukocytes in the spleen perform many different

immunodefensive functions, the client immunodefensive functions, the client who has undergone a splenectomy is who has undergone a splenectomy is at increased risk for infectionat increased risk for infection

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Thrombotic Thrombocytopenic PurpuraThrombotic Thrombocytopenic Purpura

Thrombotic thrombocytopenic purpura (TTP) is a rare Thrombotic thrombocytopenic purpura (TTP) is a rare disorder in which platelets clump together inappropriately disorder in which platelets clump together inappropriately in the microcirculation and insufficient platelets remain in in the microcirculation and insufficient platelets remain in the systemic circulation. the systemic circulation.

The client experiences inappropriate clotting, yet the blood The client experiences inappropriate clotting, yet the blood fails to clot properly when trauma occurs. fails to clot properly when trauma occurs.

The underlying cause of TTP appears to be an autoimmuneThe underlying cause of TTP appears to be an autoimmune reaction in blood vessel cells (endothelial cells) that makes reaction in blood vessel cells (endothelial cells) that makes platelets clump together in very small blood vessels. As a platelets clump together in very small blood vessels. As a result, tissues become ischemic. result, tissues become ischemic.

Common manifestations include renal failure, myocardial Common manifestations include renal failure, myocardial infarction, and stroke. If left untreated, this condition is fatal infarction, and stroke. If left untreated, this condition is fatal within 3 months in 90% of clientswithin 3 months in 90% of clients

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Thrombotic Thrombocytopenic Thrombotic Thrombocytopenic PurpuraPurpura

Treatment for the client with TTP focuses on Treatment for the client with TTP focuses on inhibiting the inappropriate platelet aggregation inhibiting the inappropriate platelet aggregation and disrupting the underlying autoimmune and disrupting the underlying autoimmune process. process.

Primary treatment consists of Primary treatment consists of plasmapheresis with the infusion ofplasmapheresis with the infusion of fresh frozen plasma. This treatment provides the fresh frozen plasma. This treatment provides the

necessary platelet aggregate inhibitors. necessary platelet aggregate inhibitors. Drugs that inhibit platelet clumping, such Drugs that inhibit platelet clumping, such as aspirin, alprostadil (Prostin), and plicamycin,as aspirin, alprostadil (Prostin), and plicamycin, also may be helpful. Immunosuppressive also may be helpful. Immunosuppressive therapy reduces the intensity of this disordertherapy reduces the intensity of this disorder

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CLOTTING FACTOR DISORDERSCLOTTING FACTOR DISORDERS Coagulation or bleeding disorders can result from a clotting Coagulation or bleeding disorders can result from a clotting

factor defect, including the inability to produce a specific factor defect, including the inability to produce a specific clotting factor, production of insufficient quantities, or a less clotting factor, production of insufficient quantities, or a less active form of clotting factor.active form of clotting factor.

Most clotting factor disorders are congenitally transmitted Most clotting factor disorders are congenitally transmitted gene abnormalities of one clotting factor. gene abnormalities of one clotting factor.

The few acquired clotting factor disorders are related to an The few acquired clotting factor disorders are related to an inability to synthesize many clotting factors at the same inability to synthesize many clotting factors at the same time as a result of liver damage or an insufficiency of time as a result of liver damage or an insufficiency of clotting cofactors and clotting cofactors and

precursor products. precursor products. Common congenital disorders that result in defects at theCommon congenital disorders that result in defects at the clotting factor level include hemophilias A and B and von clotting factor level include hemophilias A and B and von Willebrand's disease. Willebrand's disease. Disseminated intravascular coagulation (DIC) may be Disseminated intravascular coagulation (DIC) may be considered an acquired clotting disorder but is more closely considered an acquired clotting disorder but is more closely associated with septic shockassociated with septic shock

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HemophiliaHemophilia

Hemophilia comprises two hereditary bleeding disorders Hemophilia comprises two hereditary bleeding disorders resulting from deficiencies of specific clotting factors. resulting from deficiencies of specific clotting factors.

Hemophilia A (classic hemophilia) results from a Hemophilia A (classic hemophilia) results from a deficiency of factor VIII and accounts for 80% of cases of deficiency of factor VIII and accounts for 80% of cases of hemophilia. hemophilia.

Hemophilia B (Christmas disease) is a deficiency of factor Hemophilia B (Christmas disease) is a deficiency of factor IX and accounts for 20% of cases.IX and accounts for 20% of cases.

The incidence of both is 1 in 10,000. The incidence of both is 1 in 10,000. Hemophilia is an X-linked recessive trait. Female carriers Hemophilia is an X-linked recessive trait. Female carriers

have a 50% chance of transmitting the gene for have a 50% chance of transmitting the gene for hemophilia to their daughters (who then are carriers) and hemophilia to their daughters (who then are carriers) and to their sons (who will have overt hemophilia). to their sons (who will have overt hemophilia).

Hemophilia A is, with rare exceptions, a disease Hemophilia A is, with rare exceptions, a disease affecting males, none of whose sons will have the affecting males, none of whose sons will have the gene for hemophilia and all of whose daughters gene for hemophilia and all of whose daughters will be will be carriers carriers (able to pass on the gene without (able to pass on the gene without actually having the disorder). In about 30% of actually having the disorder). In about 30% of clients with hemophilia, there is no family history, clients with hemophilia, there is no family history, and it is presumed that their disease is the result and it is presumed that their disease is the result of a new mutation.of a new mutation.

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HemophiliaHemophilia

The bleeding disorder associated with hemophilia A is so The bleeding disorder associated with hemophilia A is so severe that before blood transfusions were available, children severe that before blood transfusions were available, children with hemophilia rarely survived past age 3 years. With the with hemophilia rarely survived past age 3 years. With the availability of blood transfusion and factor VIII therapy, mean availability of blood transfusion and factor VIII therapy, mean survival time has increased so greatly that hemophilia now is survival time has increased so greatly that hemophilia now is commonly seen among adult clientscommonly seen among adult clients

The clinical pictures of hemophilia A and B are identical. The The clinical pictures of hemophilia A and B are identical. The client has abnormal bleeding in response to any trauma client has abnormal bleeding in response to any trauma because of an absence or deficiency of the specific clotting because of an absence or deficiency of the specific clotting factor. Clients with hemophilia do not bleed more frequently factor. Clients with hemophilia do not bleed more frequently or even more rapidly that those without the disease, but they or even more rapidly that those without the disease, but they do bleed for a longer period. do bleed for a longer period.

Hemophiliacs form platelet plugs at the bleeding site, but the Hemophiliacs form platelet plugs at the bleeding site, but the clotting factor deficiency impairs the hemostatic response clotting factor deficiency impairs the hemostatic response and the capacity to form a stable fibrin clot. This produces and the capacity to form a stable fibrin clot. This produces abnormal bleeding, which may be mild, moderate, or severe, abnormal bleeding, which may be mild, moderate, or severe, depending on the degree of factor deficiencydepending on the degree of factor deficiency

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HemophiliaHemophilia

AssessmentAssessment Assessment of the client with hemophilia reveals the following:Assessment of the client with hemophilia reveals the following:

– Excessive hemorrhage from minor cuts or abrasions caused by Excessive hemorrhage from minor cuts or abrasions caused by abnormal platelet functionabnormal platelet function

– Joint and muscle hemorrhages that lead to disabling long-term Joint and muscle hemorrhages that lead to disabling long-term sequelaesequelae

– A tendency to bruise easilyA tendency to bruise easily– Prolonged and potentially fatal postoperative hemorrhage Prolonged and potentially fatal postoperative hemorrhage

The laboratory test results for a client with hemophilia The laboratory test results for a client with hemophilia demonstrate a prolonged partial thromboplastin time (PTT), a demonstrate a prolonged partial thromboplastin time (PTT), a normal bleeding time, and a normal prothrombin time (PT). normal bleeding time, and a normal prothrombin time (PT).

The most common health problem associated with hemophilia The most common health problem associated with hemophilia is degenerating joint function resulting from chronic bleeding is degenerating joint function resulting from chronic bleeding into the joints, especially at the hip and kneeinto the joints, especially at the hip and knee

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HemophiliaHemophiliaInterventionsInterventions The bleeding problems of hemophilia The bleeding problems of hemophilia

A can be well managed by either A can be well managed by either regularly scheduled IV administration regularly scheduled IV administration of factor VIII cryoprecipitate or of factor VIII cryoprecipitate or intermittent administration as intermittent administration as needed, depending on the individual's needed, depending on the individual's activity level activity level

and injury probability.and injury probability.

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TRANSFUSION THERAPYTRANSFUSION THERAPY PRETRANSFUSION PRETRANSFUSION RESPONSIBILITIESRESPONSIBILITIES

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TRANSFUSION TRANSFUSION RESPONSIBILITIESRESPONSIBILITIES

– The nurse takes the vital signs, including temperature, The nurse takes the vital signs, including temperature, immediately before initiating the transfusion. immediately before initiating the transfusion.

– Infusion begins slowly. Infusion begins slowly. – A nurse remains with the client for the first 15 to 30 minutes. A nurse remains with the client for the first 15 to 30 minutes. – Any severe reaction usually occurs with administration of the first Any severe reaction usually occurs with administration of the first

50 mL of blood. 50 mL of blood. – The nurse assesses vital signs 15 minutes after initiation of the The nurse assesses vital signs 15 minutes after initiation of the

transfusion to detect signs of a reaction. If there are none, the transfusion to detect signs of a reaction. If there are none, the infusion rate can be increased to transfuse 1 unit in about 2 hours infusion rate can be increased to transfuse 1 unit in about 2 hours (depending on the client's cardiovascular status). (depending on the client's cardiovascular status).

The nurse takes the vital signs every hour The nurse takes the vital signs every hour throughout the transfusion or as specified by throughout the transfusion or as specified by agency policy.agency policy.

Blood components without large amounts of red Blood components without large amounts of red blood cells (RBCs) can be infused more quickly. The blood cells (RBCs) can be infused more quickly. The identification checks are the same as for RBC identification checks are the same as for RBC transfusions. transfusions.

Physiologic changes in older clients may necessitate Physiologic changes in older clients may necessitate that blood products be transfused at a slower rate.that blood products be transfused at a slower rate.

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Red Blood Cell Red Blood Cell TransfusionsTransfusions

RBCs are administered to replace erythrocytes lost as a result of RBCs are administered to replace erythrocytes lost as a result of trauma or surgical interventions. Clients with clinical conditions that trauma or surgical interventions. Clients with clinical conditions that result in the destruction or abnormal maturation of RBCs may also result in the destruction or abnormal maturation of RBCs may also benefit from RBC transfusions. benefit from RBC transfusions.

Packed RBCs, supplied in 250-mL bags, are a concentrated source of Packed RBCs, supplied in 250-mL bags, are a concentrated source of RBCs and are the most common component administered to RBC-RBCs and are the most common component administered to RBC-deficient clients. deficient clients.

Packed RBCs are administered to individuals with a hemoglobin Packed RBCs are administered to individuals with a hemoglobin concentration less than 6 g/dL (or a hemoglobin value of 6 to 10 g/dL if concentration less than 6 g/dL (or a hemoglobin value of 6 to 10 g/dL if clinical symptoms are present).clinical symptoms are present).

Blood transfusions are actually transplantations of tissue from one Blood transfusions are actually transplantations of tissue from one person to another. The donor and recipient blood must thus be person to another. The donor and recipient blood must thus be carefully checked for compatibility to prevent potentially lethal carefully checked for compatibility to prevent potentially lethal reactions. reactions.

Compatibility is determined by two different types of antigen systems Compatibility is determined by two different types of antigen systems (cell surface proteins): the ABO system antigens and the Rh antigen, (cell surface proteins): the ABO system antigens and the Rh antigen, present on the membrane surface of RBCspresent on the membrane surface of RBCs

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Red Blood Cell Red Blood Cell TransfusionsTransfusions

RBC antigens are inherited.RBC antigens are inherited. For the ABO antigen system, a For the ABO antigen system, a

person inherits one of the following:person inherits one of the following:– A antigen (type A blood)A antigen (type A blood)– B antigen (type B blood)B antigen (type B blood)– Both A and B antigens (type AB blood)Both A and B antigens (type AB blood)– No antigens (type O blood)No antigens (type O blood)

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Platelet TransfusionsPlatelet Transfusions Platelets are administered to clients with platelet counts below 20,000 mmPlatelets are administered to clients with platelet counts below 20,000 mm33 and to and to

clients with thrombocytopenia who are actively bleeding or are scheduled for an clients with thrombocytopenia who are actively bleeding or are scheduled for an invasive procedure. invasive procedure.

Platelet transfusions are usually pooled from as many as 10 donors and do not have Platelet transfusions are usually pooled from as many as 10 donors and do not have to be of the same blood type as the client. For clients who are candidates for bone to be of the same blood type as the client. For clients who are candidates for bone marrow transplantation (BMT) or who require multiple platelet transfusions, single-marrow transplantation (BMT) or who require multiple platelet transfusions, single-donor platelets may be ordered. donor platelets may be ordered.

Single-donor platelets are obtained from one person Single-donor platelets are obtained from one person and decrease the amount of antigen exposure to the and decrease the amount of antigen exposure to the recipient, helping prevent the formation of platelet antibodies. recipient, helping prevent the formation of platelet antibodies. The chances of allergic transfusion reactions to future platelet The chances of allergic transfusion reactions to future platelet transfusions are thus reduced.transfusions are thus reduced.

Platelet infusion bags usually contain 300 mL for pooled Platelet infusion bags usually contain 300 mL for pooled platelets and 200 mL for single-donor platelets. Because the platelets and 200 mL for single-donor platelets. Because the platelet is a fragile cell, platelet transfusions are administeredplatelet is a fragile cell, platelet transfusions are administered rapidly after being brought to the client's room, usually over rapidly after being brought to the client's room, usually over a 15- to 30-minute period. a 15- to 30-minute period.

A special transfusion set with a smaller filter and shorter tubing is used.A special transfusion set with a smaller filter and shorter tubing is used.

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Platelet TransfusionsPlatelet Transfusions Standard transfusion sets are not used with platelets because the Standard transfusion sets are not used with platelets because the

filter traps the platelets, and the longer tubing increases platelet filter traps the platelets, and the longer tubing increases platelet adherence to the lumen. adherence to the lumen.

Additional platelet filters help remove white blood cells (WBCs) in the Additional platelet filters help remove white blood cells (WBCs) in the platelet concentrate. These filters are connected directly to the platelet concentrate. These filters are connected directly to the platelet transfusion set and are used for clients who have a history of platelet transfusion set and are used for clients who have a history of febrile reactions or who will require multiple platelet transfusions.febrile reactions or who will require multiple platelet transfusions.

The nurse takes the vital signs before the infusion, 15 minutes after The nurse takes the vital signs before the infusion, 15 minutes after the infusion is initiated, and at its completion. The client may be the infusion is initiated, and at its completion. The client may be premedicated with meperidine (Demerol) or hydrocortisone to premedicated with meperidine (Demerol) or hydrocortisone to minimize the chances of a reaction. minimize the chances of a reaction.

He or she can become febrile and experience He or she can become febrile and experience rigors rigors (severe chills) (severe chills) during transfusion, but these symptoms are not considered a true during transfusion, but these symptoms are not considered a true transfusion reaction. transfusion reaction.

IV administration of amphotericin B (Amphotec, Fungizone), an IV administration of amphotericin B (Amphotec, Fungizone), an antifungal agent given to many clients with leukemia, is discontinued antifungal agent given to many clients with leukemia, is discontinued during platelet transfusion and not resumed for at least 1 hour after during platelet transfusion and not resumed for at least 1 hour after transfusion. Amphotericin B can cause severe allergic reactions that transfusion. Amphotericin B can cause severe allergic reactions that are difficult to distinguish from transfusion reactionsare difficult to distinguish from transfusion reactions

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Plasma Plasma TransfusionsTransfusions

Historically, plasma infusions have been administered to replace Historically, plasma infusions have been administered to replace blood volume, and they are occasionally still used for this purpose. blood volume, and they are occasionally still used for this purpose.

It is more common for plasma to be frozen immediately after It is more common for plasma to be frozen immediately after donation. Freezing preserves the clotting factors, and the plasma donation. Freezing preserves the clotting factors, and the plasma can then be used for clients with clotting disorders. can then be used for clients with clotting disorders.

Fresh frozen plasma (FFP) Fresh frozen plasma (FFP) is infused immediately after thawing is infused immediately after thawing while the clotting factors are still viable. Clients who are actively while the clotting factors are still viable. Clients who are actively bleeding with a prothrombin time (PT) or partial thromboplastin bleeding with a prothrombin time (PT) or partial thromboplastin time (FIT) greater than 1.5 times normal are candidates for an FFP time (FIT) greater than 1.5 times normal are candidates for an FFP infusion.infusion.

ABO compatibility is required for transfusion of plasma ABO compatibility is required for transfusion of plasma products. products. The volume of the infusion bag is approximately The volume of the infusion bag is approximately 200 mL. The infusion takes place as rapidly as the client 200 mL. The infusion takes place as rapidly as the client can tolerate, generally over a 30- to 60-minute period, can tolerate, generally over a 30- to 60-minute period, through a regular Y-set or straight-filtered tubingthrough a regular Y-set or straight-filtered tubing

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CryoprecipitateCryoprecipitate Cryoprecipitate Cryoprecipitate is a product derived from plasma. is a product derived from plasma. Clotting factors VIII and XIII, von Willebrand's factor, Clotting factors VIII and XIII, von Willebrand's factor,

fibronectin, andfibronectin, and fibrinogen are precipitated from pooled fibrinogen are precipitated from pooled plasma to produce cryoprecipitate. plasma to produce cryoprecipitate.

Clients with a fibrinogen level less than 100 mg/dL are Clients with a fibrinogen level less than 100 mg/dL are candidates for a cryoprecipitate infusion. candidates for a cryoprecipitate infusion.

This highly concentrated blood product is administered to This highly concentrated blood product is administered to clients with clotting factor disorders at a volume of 10 to 15 clients with clotting factor disorders at a volume of 10 to 15 mL/unit. mL/unit.

Although cryoprecipitate can be infused, Although cryoprecipitate can be infused, it is usually given by IV push within 3 it is usually given by IV push within 3 minutes. minutes. Dosages are individualized, and it is best if theDosages are individualized, and it is best if the cryoprecipitate is ABO compatiblecryoprecipitate is ABO compatible

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TRANSFUSION TRANSFUSION REACTIONSREACTIONS

Clients can experience any of the following Clients can experience any of the following transfusion reactions: hemolytic, allergic, transfusion reactions: hemolytic, allergic, febrile, or bacterial reactions; circulatory febrile, or bacterial reactions; circulatory overload; or transfusion-associated graft-overload; or transfusion-associated graft-versus-host disease (GVHD). versus-host disease (GVHD).

The nurse is vigilant to prevent The nurse is vigilant to prevent serious complications through early serious complications through early detection and initiation of appropriatedetection and initiation of appropriate treatmenttreatment

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Hemolytic Transfusion ReactionsHemolytic Transfusion Reactions

Hemolytic transfusion reactions are caused by blood type or Rh Hemolytic transfusion reactions are caused by blood type or Rh incompatibility. When blood containing antibodies against the recipient's incompatibility. When blood containing antibodies against the recipient's blood is infused, antigen-antibody complexes are formed and released into blood is infused, antigen-antibody complexes are formed and released into the circulation. These complexes can destroy the transfused cells and the circulation. These complexes can destroy the transfused cells and initiate inflammatory responses in the recipient's blood vessel walls and initiate inflammatory responses in the recipient's blood vessel walls and organs. The ensuing reaction may be mild, with fever and chills, or life organs. The ensuing reaction may be mild, with fever and chills, or life threatening, with disseminated intravascular coagulation (DIC) and threatening, with disseminated intravascular coagulation (DIC) and circulatory collapse. Other clinical signs include the following:circulatory collapse. Other clinical signs include the following:– ApprehensionApprehension– HeadacheHeadache– Chest painChest pain– Low back painLow back pain– TachycardiaTachycardia– TachypneaTachypnea– HypotensionHypotension– HemoglobinuriaHemoglobinuria– A sense of impending doomA sense of impending doom

The onset of a hemolytic transfusionThe onset of a hemolytic transfusion reaction may be immediate or may not occurreaction may be immediate or may not occur until subsequent units have been transfused.until subsequent units have been transfused.

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Allergic Transfusion Allergic Transfusion ReactionsReactions

Allergic transfusion reactions are most often seen in clients with a Allergic transfusion reactions are most often seen in clients with a history of allergy. history of allergy.

They may have urticaria, itching, bronchospasm, or occasionally They may have urticaria, itching, bronchospasm, or occasionally anaphylaxis. anaphylaxis.

Onset of this type of reaction usually occurs during or up to 24 Onset of this type of reaction usually occurs during or up to 24 hours after the transfusion. hours after the transfusion.

Clients with a history of allergy Clients with a history of allergy can be given buffy coat-poor or washedcan be given buffy coat-poor or washed red blood cells (RBCs) in which the red blood cells (RBCs) in which the white blood cells (WBCs) and plasmawhite blood cells (WBCs) and plasma have been removed. This procedure have been removed. This procedure minimizes the possibility of an allergic minimizes the possibility of an allergic reaction.reaction.

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Febrile Transfusion Febrile Transfusion ReactionsReactions

Febrile transfusion reactions occur most Febrile transfusion reactions occur most commonly in the client with anti-WBC antibodies, commonly in the client with anti-WBC antibodies, a situation seen after multiple transfusions. The a situation seen after multiple transfusions. The recipient experiences the following:recipient experiences the following:– Sensations of coldSensations of cold– TachycardiaTachycardia– FeverFever– HypotensionHypotension– TachypneaTachypnea

Again, the physician can order buffy coat-poor Again, the physician can order buffy coat-poor RBCs or single-donor HLA-matched platelets. RBCs or single-donor HLA-matched platelets.

Leukocyte filters may also be used to trap WBCs Leukocyte filters may also be used to trap WBCs and prevent their transfusion into the clientand prevent their transfusion into the client

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Bacterial Transfusion ReactionsBacterial Transfusion Reactions

Bacterial transfusion reactions are seen after Bacterial transfusion reactions are seen after transfusion of contaminated blood products. Usually transfusion of contaminated blood products. Usually a gram-negative organism is the source because a gram-negative organism is the source because these bacteria grow rapidly in blood stored under these bacteria grow rapidly in blood stored under refrigeration. Symptoms include the following:refrigeration. Symptoms include the following:– TachycardiaTachycardia– HypotensionHypotension– FeverFever– ChillsChills– ShockShock

The onset of a bacterial transfusion reaction is rapidThe onset of a bacterial transfusion reaction is rapid

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Circulatory OverloadCirculatory Overload Circulatory overload can occur when a blood product is Circulatory overload can occur when a blood product is

administered too quickly. This complication is most common with administered too quickly. This complication is most common with whole-blood transfusions or when the client requires multiple whole-blood transfusions or when the client requires multiple transfusions. Older adults are most at risk for this condition. transfusions. Older adults are most at risk for this condition. Symptoms include the following:Symptoms include the following:– HypertensionHypertension– Bounding pulseBounding pulse– Distended jugular veinsDistended jugular veins– DyspneaDyspnea– RestlessnessRestlessness– ConfusionConfusion

The nurse can both manage and The nurse can both manage and prevent this complication by monitoring prevent this complication by monitoring intake and output, transfusing bloodintake and output, transfusing blood products more slowly, and administeringproducts more slowly, and administering diuretics.diuretics.

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Transfusion-Associated Graft-Versus-Host Transfusion-Associated Graft-Versus-Host DiseaseDisease

Transfusion-associated graft-versus-host disease (TA-GVHD) is Transfusion-associated graft-versus-host disease (TA-GVHD) is an infrequent but life-threatening complication that can occur an infrequent but life-threatening complication that can occur in both immunosuppressed and immunocompetent clients. in both immunosuppressed and immunocompetent clients.

Its cause in immunosuppressed clients is similar to that of Its cause in immunosuppressed clients is similar to that of GVHD associated with allogeneic bone marrow transplantation GVHD associated with allogeneic bone marrow transplantation (BMT), in which donor T-cell lymphocytes attack host tissues.(BMT), in which donor T-cell lymphocytes attack host tissues.

The cause of TA-GVHD in immunocompetent hosts is The cause of TA-GVHD in immunocompetent hosts is uncertain. Reactions are more common when the host and uncertain. Reactions are more common when the host and donor share similar human leukocyte antigens (HLAs), such as donor share similar human leukocyte antigens (HLAs), such as in first-degree relatives or individuals with a similar ethnic in first-degree relatives or individuals with a similar ethnic background. Symptoms typically occur within 1 to 2 weeks and background. Symptoms typically occur within 1 to 2 weeks and include thrombocytopenia, anorexia, nausea, vomiting, chronic include thrombocytopenia, anorexia, nausea, vomiting, chronic hepatitis, weight loss, and recurrent infection.hepatitis, weight loss, and recurrent infection.

TA-GVHD has a 90% mortality rate but can be prevented by TA-GVHD has a 90% mortality rate but can be prevented by using irradiated blood products, which destroy T-cells and their using irradiated blood products, which destroy T-cells and their cytokine productscytokine products

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AUTOLOGOUS BLOOD AUTOLOGOUS BLOOD TRANSFUSIONSTRANSFUSIONS

Autologous blood transfusions involve collection Autologous blood transfusions involve collection and transfusion of the client's own blood. and transfusion of the client's own blood.

Advantages of this type of transfusion are Advantages of this type of transfusion are guaranteed compatibility and elimination of the guaranteed compatibility and elimination of the risk of transmitting diseases such as hepatitis or risk of transmitting diseases such as hepatitis or HIV. HIV.

The four types of autologous blood The four types of autologous blood transfusions are preoperative autologoustransfusions are preoperative autologous blood donation, acute normovolemic blood donation, acute normovolemic hemodilution, intraoperative autologous hemodilution, intraoperative autologous transfusion, and postoperative blood salvagetransfusion, and postoperative blood salvage

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