hematology handouts

Medical and Surgical Nursing

Hematology Lecture Notes

Prepared by: Mark Fredderick R. Abejo RN,, MAN

1 MS Abejo

MEDICAL AND SURGICAL NURSING

Hematology

Lecturer: Mark Fredderick R. Abejo RN, MAN

OVERVIEW OF THE STRUCTURE AND FUNCTION OF

THE HEMATOLOGIC SYSTEM

HEMATOLOGY – the scientific study of the structure and

functions of blood in health and in disease.

BLOOD – is the circulatory fluid of the CV system which is

circulating constantly through a closed circuit of tubes.

FUNCTIONS:

► supply oxygen from the lungs and absorbed nutrients

from the GIT to the cells

► remove waste products from tissues to the kidneys,

skin and lungs for excretion

► transport hormones from their origin in the endocrine

glands to other parts of the body

► protect the body form dangerous microorganism

► promote Hemostasis ( to stop bleeding)

► regulate body temperature by heat transfer –

vasoconstriction and vasodilation

Hematopoiesis

Process of blood cell production.

At birth, it is accomplished in the liver, spleen,

thymus, lymph nodes and red bone marrow.

After birth, it is confined in the red bone marrow ( but

some WBCs are still produced in the lymphatic

tissues).

During childhood, all blood cells are essentially

produced in marrow sites of the flatbones of the skull,

clavicle, sternum, ribs, vertebrae, and pelvis

After puberty, hematopoiesis becomes localized

within the flatbones of the sternum, ilium, ribs, and

vertebrae, sometimes occurring in the proximal ends

of long bones (humerus and femur)

All formed elements come from one stem cell or the

HEMOCYTOBLAST. Cell differentiation gives rise

to the cell lines with the help of growth factors.

Adult bone marrow produces :

175 b RBCs

70 b neutrophils

175 b platelets

Blood Vessels

Veins

Arteries

Capillaries

Blood Forming Organs

Liver

Thymus

Spleen

Bone Marrow

Lymph nodes

Lymphoid organ

CHARACTERISTICS: Color

o Arterial Blood

o Venous Blood

Fraction of body weight 8 %

Volume Female: 4 -5 L

Male: 5 - 6 L

Temperature 38 C ( 100.4 F )

pH 7.35 - 7.45

Viscosity (relative to water)

Whole blood: 4.5-5.5

Plasma: 2.0

Specific gravity - 1.048 to 1.066 ( 1.055 – 1.065)

Composition:

Liquid phase: PLASMA (55%)

► - A light yellow substance which is one of

the major fluids of the body. Major function

is to maintain the blood volume within the

vascular compartment

► 92% Water

► Serum

► Plasma Proteins – all produced in the LIVER

Albumin – most abundant, maintains osmotic

pressure

Globulin Alpha – transports bilirubin, steroids and

hormones

Beta – transports iron and copper

Gamma – transports immunoglobulins

Prothrombin – clotting factor

Fibrinogen – clotting factor

► Electrolytes ( Na+,,Ca 2+,, HCO3ˉ, CIˉ),

► Miscellaneous (less than 1%): sugars, fats, vitamins,

hormones




2 MS Abejo

Solid phase: FORMED ELEMENTS /

CELLELAR COMPONENTS (45%)

RBC (Erythrocytes) – only component which is

anucleated

N = 4-6 million/mm3

Are biconcave discs (AKA discocytes) which are less

than 7.5 micrometers in diameter.

ERYTHROPOIESIS process of formation of RBC

ERYTHROPOETIN hormone produced primarily by

the kidney; necessary for erythropoiesis

HEMOGLOBIN iron-containing protein of RBC,

delivers oxygen to tissue

Carries about 200-300 million molecules of

hemoglobin(heme-globin-iron) that attach oxygen

within each RBC, responsible for 97% of O2 transport

Molecules of Hgb (carries oxygen)

( Ave. 12 - 18 g/dL)

Female: 12-16 g/dL

Male: 13-18 g/dL

HEMATOCRIT – red cell percentage in whole blood

(three times of normal Hemoglobin)

FEMALES: 36-42%

MALES: 42-48%

Substances needed for maturation of RBC

1. FOLIC ACID – prevents neural tube deficit;

needed in the FIRST trimester of pregnancy

2. IRON – needed in the THIRD trimester

3. VIT B12 (Cyanocobalamin)

4. VIT C (Ascorbic Acid)

5. VIT B6 (Pyridoxine)

6. INTRINSIC FACTOR (released in stomach’s

parietal cells)

Normal lifespan – 80-120 days

Spleen – kills RBCs in the red pulp

WBCs (Leukocytes)

N = 5,000-10,000/mm3

Granulocytes

1. Neutrophils – most abundant, 60-70% of total

WBCs

First line of defense, most common type of

leukocyte but a short lifespan of only 10-12

hours making them ineffective in destroying

infectious agents

Helpful in localizing the infection and in

immobilizing the pathogens until other

WBCs arrive

for acute inflammation

2. Eosinophils– allergic reactions

Weak phagocytic action, elevated during

asthma attacks.

Usually activated during parasitic invasion

(Schistosomes / blood flukes)

Lifespan= hours to 3 days

Modulates or reduce IgE mediated allergic

reactions

3. Basophils – not phagocytic in nature, they are

mediators in inflammatory process.

Involved in the release of chemical

mediators

Prostaglandin

Serotonin

Histamine

Bradykinin

For inflammation

Non-granulocytes (agranulocytes)

1. Monocytes – largest WBC (macrophage)

Upon release in the bone marrow and travel

to the different tissues, it is just a hypoactive

phagocytic cell, become a Macrophage

when it attaches to the endothelium of

organs and performs its full phagocytic

function.

Long term phagocytosis (months)

KUPFFER – kidneys

HISTOCYTES – skin and subcutaneous

ALVEOLAR macrophage – lungs

MICROGLIA – CSF

MACROPHAGE - blood

2. Lymphocytes

B cells (bone marrow)

differentiated in the bone marrow, antibody-

mediated immune response (Humoral)

For immunity

T cells (Thymus)

For immunity

Differentiated in the Thymus and lives

long cell-mediated response

Target site of HIV

AIDS incubation period: 6 mos –

5 years; window period 6 mos

AZT ZIDOVUDINE or

RETROVIR : drug of choice for

aids

WESTERN BLOT – confirmatory

test for aids

Kaposi’s SARCOMA

NK cells

Natural killer cells

Anti-tumor and anti-viral properties

Humoral (Antibody-Mediated) Immune Response

B Cells

– Matures into Plasma Cells responsible for Antibody

production

5 Classes of Immunoglobulins (MADGE) :

– Immunoglobulin M (IgM) • 1st immunoglobulin produced in an immune

responsepresent in plasma, too big to cross

membrane barriers

– Immunoglobulin A (IgA) • Sound in body secretions like saliva, tears, mucus,

bile, milk & colostrum

– Immunoglobulin D (IgD) • Present only in the plasma & is always attached to the

B Cell

– Immunoglobilin G (IgG) • 80% of circulating antibodies

• Can cross the placenta and provide passive immunity

• Present in all body fluids

– Immunoglobulin E (IgE) • Responsible for Allergic & hypersensitivity reactions

• Stimulates Mast cells & Basophils to release

Histamine which mediates inflammation & the

allergic response

PLATELETS (Thrombocytes)

N = 150-450 thousand mm3

Promotes hemostasis prevention of blood loss

promote clotting mechanisms

MEGAKARYOCYTES – immature/baby platelets;

target site of DHF

Normal lifespan: 9-12 days




3 MS Abejo

Hemostasis (Blood Clotting)

Three Major Phases

1. Platelet Plug Formation

– Platelets adhere and stick to vessel lining that are

damaged forming a Platelet Plug or White

Thrombus

– Platelets release chemicals to attract more platelets

to the injured site

2. Vascular Spasms

– Platelets release Serotonin causing spasms of the

blood vessel, constricting it & decreasing blood

flow

3. Coagulation or Blood Clotting

– Thromboplastin is released by damaged cells

– plasma Clotting Factors form an activator that

triggers the Clotting Cascade

– a Blood Clot is formed

– Serum is squeezed out within the hour pulling the

ruptured edges together

Plasma Clotting Factors I Fibrinogen

II Prothrombin

III Tissue Thromboplastin

IV Calcium

V Proacelerin

VII Proconvertin

VIII Antihemophilic Factor

IX Christmas Factor

X Stuart – Prower Factor

XI Plasma Thromboplastin

Antecedent

XII Hageman Factor

XIII Fibrin Stabilizing Factor

Compatible Blood Types

Assessment of the Hematologic System

1. Risk Factor Analysis

Non-Modifiable

a. Age

immune response is diminished in both very young and

very old

anemia prevalence increases with age

folic acid deficiency in growth spurt (infants and

adolescents)

because some laboratory results are age- specific

b. Sex

women have lower hemoglobin and hematocrit levels,

more prevalence of agranulocytosis

hemophilia, bleeding expressed among males

because some laboratory results are sex-specific

c. Race

Blacks have lower hemoglobin levels than whites (more

prevalent sickle cell anemia)

d. Family history

Because some hematologic disorders are inherited:

– Anemia

– Thrombocytopenia

– Bleeding disorders (hemophilia and Von

Willebrand’s Dse),

– Congenital Blood Disorder (Sickle Cell

anemia)

– Jaundice, infections, delayed healing,

– Cancer

– Autoimmune dse (aplastic anemia, pernicious

anemia)

e. Congenital lack of the intrinsic factor

Modifiable

a. Exposure to certain chemical and drugs

Radiation overexposure

Anti-neoplastic drugs/ chemotherapy

Chemical Oxidants (e.g benzene, nitrites, lead, arsenic,

etc.)

Drugs (chloramphenicol, sulfonamide, anti-convulsant,

streptomycin, hair dyes

2. History – Chief complaint Disorders of the hematologic system often affects all

organs and tissues

Determine:

o Onset

abrupt or gradual?

since childhood or recent

o Allergen triggered response? Seasonal?

o How long do the allergic manifestations last? Relieved

or persist once the allergen is removed?

o Quality and quantity

How severe? Massive bleeding? How long does

it last?

How long do the bleeding episodes last and how

severe they are?

Does blood ooze from a site or does sudden

massive bleeding occur?

How often do bleeding episodes occur and how

long do they last?

What does the client do to stop them?

Is there any break in skin integrity?

Swelling? Edema? Fever? Pain? Tenderness?

Pruritus? Redness? Or drainage?

Note allergic manifestations such as rhinitis,

sneezing, nasal stuffiness, postnasal drip, sore

throat, voice changes, hoarseness, wheezing,

persistent cough, dyspnea, malaise, fatigue,

tearing or altered hearing acuity.

o Severity and location

Rest can alleviate fatigue? Bleeding of joints?

Can rest alleviate fatigue? (s/sx of anemia)

Ask how activities and activity tolerance changed

over time

Does the client bruise easily?

Has bleeding in the joints?

Number and saturation of sanitary pads

Anaphylactic reactions? Or simple allergic

response?




4 MS Abejo

o Precipitating factors

Anticoagulant? – bleeding

Bone Marrow suppression – anemia, leukemia,

and thrombocytopenia

Antineoplastic drugs? Antibiotics? Radiation?

Infectious agents? Corticosteroids or

immunosuppressive drugs?

Allergic triggers (inhalants such as pollens and

dust, contact agents such as dyes and cosmetics,

ingested agents such as foods and drugs,

injectable agents such as drugs, vaccine and

insect venom)

o Aggravating and relieving factors

salicylates containing OTC may aggravate

bleeding

what relieves allergic manifestations

3. Past Medical History

a. Major illnesses and hospitalization

Previous hematologic problems

Surgical procedures that may affect the hematologic

system

Liver problems

Any bleeding disorders

o How long was the bleeding problem? Do any

members of the family have a history of

bleeding?

o Is bleeding linked with any specific event or

procedure? Does it occur with menses or

following minor trauma? Any frequency of nose

bleeding? Does he bruise easily? Any petechiae?

o How severe are any of the bleeding episodes?

What is the durations?

o Any history of hepatic/ splenic or renal disease?

Recently taken medications?

b. Medications- aspirin, chloramphenicol, antineoplastic drugs

c. Allergies- Hx of allergies, BT and/reactions

d. Family History

4. Psychosocial Hx and Lifestyle

a. Occupation- exposure to chemicals and radiation

b. Habits- nutritional, substance abuse, alcohol abuse

Review of Systems/ Physical Examination

1. SKIN

– pallor

– ruddy skin

– jaundice

– dry skin, brittle, spoon shaped with longitudinal ridges

2. EYES – visual disturbances (anemia and polycythemia)

– blindness (retinal hemorrhage related to

thrombocytopenia and bleeding do)

– scleral jaundice (hemolytic anemia)

3. EARS – vertigo, tinnitus (severe anemia)

– bleeding in auditory canal

– (bleeding do)

4. NOSE – epistaxis (thrombocytopenia and bleeding disorders)

5. MOUTH – smooth, glossy, bright red tongue and sore tongue

(pernicious and Fe def. anemia)

– gingival bleeding (thrombocytopenia and bleeding

disorders)

6. LUNGS – dyspnea, orthopnea (anemia)

7. CARDIOVASCULAR SYSTEM – tachycardia, palpitation, murmurs, angina (anemia)

8. GIT – dysphagia (mucous membrane atrophy due to iron def.

anemia)

– abdominal pain( bleeding)

– hepatomegaly, splenomegaly (hemolytic anemia)

– hematemesis, melena (thrombocytopenia and bleeding

disorders

9. GUT – hematuria (bleeding disorders)

– amenorrhea and menorrhagia

– (iron def. and bleeding do)

10. MUSCULOSKELETAL – joint pain (hemophilia)

– back pain

– sternal tenderness and bone pain

– (sickle cell crisis)

–

11. NERVOUS SYSTEM – headache, confusion

– (anemia, polycythemia)

– brain hemorrhage

– (thrombocytopenia and bleeding disorders)

– peripheral neuropathy,

– paresthesis, loss of balance (pernicious anemia)

DIAGNOSTIC PROCEDURES

1. COMPLETE BLOOD COUNT

a. RBC count- # of RBCs/ mm3 of blood, to diagnose anemia

and ploycythemia

b. Hemoglobin- # of grams of hgb/ 100ml of blood; to measure

the oxygen-carrying capacity of the blood

c. Hematocrit – expressed in %; measures the volume of RBCs

in proportion to plasma; used also to diagnose anemia and

ploycythemia and abnormal hydration states

d. RBC indices- measure RBC size and hemoglobin content

a. MCV (mean corpuscular volume)

b. MCH (mean corpuscular hemoglobin)

c. MCHC (mean corpuscular hemoglobin

concentrarion)

e. Platelet count- # of Platelet/ mm3; to diagnose

thrombocytopenia and subsequent bleeding tendencies

f. WBC count- of WBCs/ mm3 of blood; to detect infection or

inflammation

g. WBC Differential count- determines proportion of each

WBC in a sample of 100 WBCs; used to classify leukemias

Normal Values

RBC: Women – 4.2-5.4 million/mm3

Men – 4.7-6.1 million/mm3

Hgb: Women – 12-16 g/dl

Men – 13-18 g/dl

Hct : Women – 36-42%

Men – 42-48%

WBC: 5000-10,000/mm3

Granulocytes Neutrophils: 55-70%

Eosinophils: 1-4%

Basophils: 0.5-1.0%

Agranulocytes Lymphocytes: 20-40%

Monocytes: 2-8%

Platelets: 150,000-450,000/mm3




5 MS Abejo

2. PERIPHERAL BLOOD SMEAR

To determine the variations/ abnormality in RBCs, WBCs and

Platelets: normal size and shape (normocytes) and normal color

(normochromic)

3. DIRECT ANTIGLOBULIN EST (Coomb’s Test)

Used in cross matching blood when transfusion reaction occurs,

test umbilical cord for Erythroblastosis fetalis and diagnose

acquired hemolytic anemia

4. INDIRECT ANTIGLOBULIN TEST

Identifies antibodies to RBC antigens in the serum of clients

who have greater than normal chance of developing transfusion

reactions.

5. RETICULOCYTE COUNT

Used to determine the responsiveness of the bone marrow to the

depletion of circulating RBCs (probably due to hemolytic

anemia or hemorrhage)

6. BONE MARROW ASPIRATION and BIOPSY

Used to determine size and shape of RBCs, WBCs and platelet

precursors and to examine various maturational abnormalities.

Nursing Responsibility

Preprocedure - explain the purpose, obtain consent

- inform client of pain or of what to

expect

- give sedatives as ordered

Procedure - place patient in lateral position, with

site of aspiration uppermost

- clean pt’s skin with antiseptic sol’n

- administer local anesthesia to numb

skin and subcutaneous tissues

- apply ice on the contralateral side to

relieve pain

Postprocedure - apply pressure until bleeding stops

- check site frequently for bleeding

- give pain relievers to relieve pain

7. COAGULATION SCREENING TESTS

a. Bleeding Time – measures the ability to stop bleeding after

small puncture wound

b. Partial Thromboplastin Time (PTT) – used to identify

deficiencies of coagulation factors, prothrombin and fibrinogen;

monitors heparin therapy.

c. Prothrombin Time (Pro-time) – determines activity and

interaction of the Prothrombin group: factors V (preacclerin),

VII (proconvertin), X (Stuart-Power factor), prothrombin and

fibrinogen; used to determine dosages of oral anti-coagulant.

Normal Values

Reticulocytes: 25-75 x 10 9/L

Bleeding Time: 2.75-8 min

Partial Thromboplastin Time (PTT): 20-35 sec.

Prothrombin Time (PT): 12-14 sec.

BLOOD DISORDERS

I. IRON DEFICIENCY ANEMIA (IDA) – chronic

microcytic anemia due to inadequate absorption of iron

leading to hypoxemic tissue injury

A. INCIDENT RATE

1. Developed countries (d/t high intake of cereals

and milk)

2. Accidents (adults)

3. Tropical areas (blood sucking parasites)

4. Women 15-35 (reproductive age)

5. Common among the poor (poor nutrition)

B. PREDISPOSING FACTORS

1. Chronic blood loss

Trauma

Menstruation

GIT bleeding

Hematemesis

Melena (UGIB)

Hematochezia (LGIB) (d/t E.

histolytica DOC: metronidazole)

2. Inadequate intake of iron rich food

3. Inadequate absorption of iron due to

Chronic diarrhea

R/t increased cereal intake with decreased

animal CHON ingestion, related to subtotal

gastrectomy

Malabsorption syndrome

4. Improper cooking of foods

C. SIGNS AND SYMPTOMS

1. Usually asymptomatic, first sign: weakness and

fatigue

2. Headache, dyspnea, dizziness, palpitations, cold

sensitivity, generalized body malaise, pallor

3. Brittleness of hair, spoon shaped nails

(koilonychia 180 degrees ang normal) d/t

hypoxia atrophy of epidermal cells

4. Atrophic glossitis, stomatitis, dysphagia

D. DIAGNOSTICS: ALL DECREASED!

1. RBC

2. Hgb

3. Hct

4. Reticulocytes

5. Iron

E. NURSING MANAGEMENT

1. Monitor for signs of bleeding of all hema test

including urine, stool and GIT

2. Enforce CBR so as not to overtire patient

3. Encourage increased iron diet (Damo! green

leafy vegetables, California raisins, organ meat,

legumes, yolk, dried foods




6 MS Abejo

4. Avoid tannates in tea and coffee because it

impairs iron absorption

5. Administer medications as ordered

Oral iron preparations (300mg OD)

FeSO4, Fe Fumarate, Fe Gluconate

NURSING MANAGEMENT

1. Administer with meals to lessen

GIT irritation 2. Use straw for liquid form

3. Administer with orange juice or

vitamin C to facilitate absorption 4. Inform client of SE/monitor for

a. Anorexia

b. Nausea and vomiting c. Abdominal pain

d. Diarrhea/constipation

e. Melena

Parenteral Iron Preparations

Iron Dextran IM or IV

Sorbitex IM

NURSING MANAGEMENT

1. Administer using z-tract method to prevent discomfort,

discoloration and leakage

2. Avoid massaging of injection site instead encourage pt. to ambulate

to facilitate absorption

3. Monitor SE a. Pain at injection site

b. Localized abscess

c. Lymphadenopathy d. Fever and chills

e. Pruritus and urticaria

Hypotension anaphylactic shock

epinephrine

RBC (80-120 days) destroyed in Spleen Hgb

Hemoglobin breaks into: Globin

Heme

A. Ferrous 1. Bilirubin

2. Biliverdin

B. Ferritin Early sign of anaphylactic shock: dyspnea

II. PERNICIOUS ANEMIA – chronic anemia resulting from

deficiency of intrinsic factor leading to hypochlorhydria

(decreased HCl secretion); IDIOPATHIC

A. PREDISPOSING FACTORS

1. Subtotal gastrectomy

2. Hereditary factors

3. Inflammatory disorders of the ileum

4. Autoimmune

5. Strictly vegetarian diet

Stomach (widest area of alimentary canal) Argentaffin/oxyntic/parietal cells in stomach

produces IF promotes reabsorption of vit B12 (Cyanocobalamin) maturation of

RBC

If absent IF dyspepsia weight loss

so increase calories in diet

Secretes HCl acid it aids in digestion

Immature RBCs sequestered in spleen bilirubinemia jaundice

B. SIGNS AND SYMPTOMS

1. Headache, dizziness, dyspnea, palpitation, cold

sensitivity, pallor and generalized body malaise

2. GIT changes

Mouth sores, Red beefy tongue, Dyspepsia

or indigestion, Weight loss, Jaundice

3. CNS changes – PA is the most dangerous form of

anemia

Tingling sensation, Paresthesia, Ataxia,

Psychosis

C. DIAGNOSTICS

1. SCHILLING’S TEST – indicates decreased

reabsorption of vitamin B12; confirms presence

of pernicious anemia

D. NURSING MANAGEMENT

1. Enforce complete bed rest (consistent to all types

of anemia)

2. Administer Vit B12 injections at MONTHLY

intervals for lifetime as ordered; common site:

dorso and ventrogluteal, no drug toxicity because

it is water soluble and is easily excretable; oral

forms might develop tolerance.

3. Increase caloric intake, CHON, CHO, Fe, Vit C

4. Encourage client to use soft bristled toothbrush

and avoid irritating mouthwashes (remember

there are mouthsores!)

5. Avoid heat application (there is numbness

remember?) may lead to burns

III. APLASTIC ANEMIA – stem cell disorder leading to bone

marrow depression pancytopenia (all blood cells

decreased) anemia, leucopenia, thrombocytopenia


1. Chemicals

Benzene and its derivatives

2. Irradiation

3. Immunologic injury

4. Drugs

Broad spectrum antibiotics

Chloramphenicol

Sulfonamides (Bactrim…)

Chemotherapeutic Agents

Nitrogen Mustard (Anti-metabolite)

Vincristine (plant alkaloid)

Methotrexate (alkylating agent)

Phenylbutazones


1. Headache, dizziness, dyspnea, palpitations,

pallor, cold sensitivity, generalized body malaise

2. Leukopenia (increased susceptibility to

infections)

3. Thrombocytopenia Petechiae

Ecchymoses Oozing of blood from venipuncture sites

C. DIAGNOSTICS

1. CBC – pancytopenia

2. Bone Marrow Biopsy or Aspirate

Posterior iliac crest

Would reveal fat necrosis in the bone

marrow

D. NURSING MANAGEMENT

1. Removal of underlying cause

2. BT as ordered

3. Enforce complete BR

4. Administer O2 inhalation

5. Reverse isolation

6. Monitor for signs of infection

7. Avoid IM, SQ or any venipuncture sites

8. instruct: use electric razor when shaving

9. Medications as ordered

Immunosuppressants via central venous

catheter

Anti-lymphocyte globulin (ALG) –

given within 6 days – 3 weeks to

achieve maximum therapeutic effect




7 MS Abejo

IV. SICKLED CELL ANEMIA - is a life-long blood disorder

characterized by red blood cells that assume an abnormal,

rigid, sickle shape


1. Hereditary factors

2. African ,South/Central American people and

Mediterranean countries


Related to anemia:

1. Shortness of breath

2. Dizziness

3. Headache

4. Coldness in the hands and feet

5. Pale skin

6. Chest pain

Related to pain:

1. Sickle Cell Crisis - Sickle cell crises often affect

the bones, lungs, abdomen, and joints.

- A sickle cell crisis occurs when sickled red

blood cells form clumps in the bloodstream.

These clumps of cells block blood flow through

the small blood vessels in the limbs and organs.

This can cause pain and organ damage

Complications of Sickle Cell Crisis Hand-Foot Syndrome

Splenic Crisis

Infections

Acute Chest Syndrome

Pulmonary Arterial Hypertension

Delayed growth and puberty

Stroke

Eye problem

Priapism

Gallstone

Ulcers of the leg

Multiple organ failure

C. DIAGNOSIS

1. CBC reveals hgb of 6-8g/dl , increase

reticulocyte count, low hct

2. HemoglobinElectrophoresis, confirmatory

diagnosis for sickled cell anemia

3. Urinalysis – UTI

4. Chest Xray and CT scan – pulmonary

complication

E. MEDICAL MANAGEMENT

1. Children born with sickle-cell disease will

undergo close observation.

2. Patients will take a 1 mg dose of folic acid daily

for life.

3. From birth to five years of age, they will also

have to take penicillin daily due to the immature

immune system that makes them more prone to

early childhood illnesses.

4. Painful crises are treated symptomatically with

analgesics; pain management requires opioid

administration at regular intervals until the crisis

has settled

5. The first approved drug for the causative

treatment of sickle-cell anaemia, hydroxyurea,

was shown to decrease the number and severity

of attacks

6. Bone marrow transplants have proven to be

effective in children

F. NURSING MANAGEMENT

1. Administer O2 & Blood Transfusion as Rx

2. Maintain adequate hydration

3. Avoid tight clothing that could impair

circulation.

4. Keep wounds clean and dry.

5. Provide bed rest to decrease energy expenditure

and oxygen use.

6. Encourage patient to eat foods high in calories,

CHON, with folic acid supplementation.

7. Analgesics:

o Acetaminophen

o Morphine

o avoid aspirin as it enhances acidosis,which

promotes sickling

8. Avoid anticoagulants( sludging is not due to

clotting ).

9. Antibiotics.

10. Avoid activities that require so much energy.

11. Keep arms and legs from extreme cold.

12. Decrease emotional stress.

13. Provide good skin care

V. THALASSEMIA MAJOR (Cooley’s anemia)

B - thalassemia refers to an inherited hemolytic

anemia, characterized by reduction or absence of the

B-globulin chain in Hgb synthesis

Fragile RBC & short life span

Autosomal recessive pattern of inheritance

Insufficient B-globulin chain synthesis allows large

amounts of unstable chains to accumulate

Precipitates of alpha chains that form cause RBC’s to

be rigid & easily destroyed, leading to severe

hemolytic anemia = chronic hypoxia

Skeletal deformities: pathologic fractures

Hemosiderosis – excess iron supply, which leads to

iron deposits in the organ tissues leading to decreased

function

A. CLINICAL MANIFESTATIONS

1. onset is usually insidious

2. Sx are primarily related to progressive anemia,

expansion of marrow cavities of the bone &

developmemnt of hemosiderosis

3. Early Sx often include progressive pallor, poor

feeding & lethargy

4. Further signs: hemorrhage, bone pain, exercise

intolerance, jaundice, & protuberant abdomen

5. hemosiderosis of the eye and lungs

B. DIAGNOSTIC EVALUATION

• Decrease hemoglobin

• RBC= increase in number

• Hgb elctrophoresis

– elevated levels of HgF ( doesn’t hold O2

well )

– limited amount of HgA




8 MS Abejo

C. COMPLICATIONS

1. Splenomegaly

2. Growth retardation in the second decade

3. Endocrine abnormalities : – delayed development of secondary sex

characteristics – most boys fail to undergo

puberty, girls – menstruation problems

– DM – due to iron deposits in the pancreas

– Hypermetabolic rates

4. Skeletal complications – Frontal & parietal bossing (Enlargement)

– Maxillary hypertrophy – leading to

occlusion

– Premature closure of epiphyses of long

bones

– Osteoporosis & pathologic fractures

5. Cardiac problems: pericarditis & CHF – usual

cause of death

D. MANAGEMENT

1. Frequent and regular transfusion of packed

RBC’s to maintain Hgb levels above 10 g/dL

2. Iron chelation therapy with deferoxamine

(Desferal) – reduces toxic effects of excess iron

& increases iron excretion thru urine & feces

3. Splenectomy

4. Supportive management of symptoms

5. Bone marrow transplant

6. Prognosis and Survival rate is poor because of no

known cure

7. Often fatal in late adolescence or early adulthood

VI. POLYCYTHEMIA VERA Underlying cause is unknown

Hyperplasia of all bone marrow elements

> increase RBC mass

> increase blood volume viscosity

> decrease marrow iron reserve

> Splenomegaly

A. ASSESSMENT

1. Reddish purple hue of skin & mucosa,

pruritus

2. Splenomegaly, hepatomegaly

3. Epigastric discomfort, abdominal discomfort

4. Painful fingers & toes from paresthesias

5. Altered mentation

6. Weakness, fatigue, night sweats, bleeding

tendency

7. Hyperuricemia – from increased RBD

formation and destruction

B. DX TESTS 1. CBC

2. BONE MARROW ASPIRATION & Biopsy

C. MANAGEMENT

1. HYPERVISCOSITY

= phlebotomy @ intervals determined by CBC

results to decrease RBC mass

=generally 250-500ml removal @ a time

2. HYPERPLASIA

= myelosuppressive therapy,

= generally using hydroxyurea or IV radioactive

phosphorus (32P), biologic response

modifier, ie alpha interferon

3. HYPERURICEMIA= allupurinol

(Zyloprim)

4. PRURITUS = antihistamines (cimitidine),

low dose acetyl salicylic acid; certain anti-

depressants (paroxetin), phototherapy,

cholestyramine

D. NURSING INTERVENTION

1. Encourage/assist ambulation

2. Assess for early S/Sx of thromboembolic

complications : swelling of limbs, increased

warmth, pain

3. Monitor CBC & assist with phlebotomy as

ordered

Patient Education

Educate about risk of thrombosis; encourage

patient to maintain normal activity pattern &

avoid long periods of rest

Avoid hot showers

Report @ regular intervals for follow up

blood

VII. HEMOPHILIA

Hereditary coagulation defect, usually

transmitted to affected male by female carrier

through sex – linked recessive gene, resulting in

prolonged clotting time.

Most common type is Hemophilia A or Classic

Hemophilia - factor VIII deficiency (called

Antihemophilic Factor / AHF)

Hemophilia B or Christmas Disease – factor IX

deficiency (called the Christmas Factor)

Male inherits hemophilia from their mothers, and

females inherit the carrier status from their

fathers.

– Found predominantly, but not exclusive, in

male offsprings

Bleeding occurs due to impaired ability to form

fibrin clot

A. ASSESSMENT

1. Abnormal bleeding in response to trauma or

surgery. (muscles/joints)

2. Joint bleeding causing pain, tenderness,

swelling, and limited range of motion.

3. Tendency to bruise easily.

4. Epistaxis

5. Hemarthrosis (bleeding in joints causing pain,

swelling and limited movement)

B. IMPLEMENTATION

1. Administer factor VIII concentrate.

2. Monitor for bleeding and maintain bleeding

precautions.

3. Monitor for joint pain; IMMOBILIZE the

affected extremity if joint pains occur.

4. Monitor urine for hematuria.




9 MS Abejo

5. Instruct the parents regarding activities for the

child, emphasizing the avoidance of contact

sports.

6. Instruct the parents on how to control bleeding

(direct/indirect pressure)

7. DDVAP (Desmopressin) – promotes the release

of Factor VIII in hemophilia A

8. Use soft toothbrush and point out need for

regular dental checkups

9. Refer to National Hemophilia Association

10. Emphasize avoidance of Aspirin

11. Provide diet information as excess weight places

further stress on joints

R - Rest

I - Immobilize

C - Cold Compress

E - Elevate

VIII. DISSIMENATED INTRAVASCULAR

COAGULATION

DIC is a disorder of diffuse activation of the clotting

cascade that results in depletion of clotting factors in

the blood.

occurs when the blood clotting mechanisms are

activated all over the body instead of being localized

to an area of injury.

grave coagulopathy resulting from overstimulation of

clotting & anticlotting processess in response to

disease & injury

Small blood clots form throughout the body, and

eventually the blood clotting factors are used up and

not available to form clots at sites of tissue injury.

Clot - dissolving mechanisms are also increased

stimulated by many factors including infection in the

blood & severe tissue injury – burns and head injury,

reactions to blood transfusions, carcinomas and

obstetrical complications such as retained placenta

after delivery.

A. ASSESSMENT

1. purpura on lower extremities & abdomen

2. hemorrhagic bullae, acral cyanosis, focal

gangrene in skin

B. Dx Tests:

1. marked decrease of blood platelets

2. low levels of fibrinogen & other clotting factors

3. prolonged prothrombin & partial thromboplastin

times & abnormal erythrocyte

morphologic characteristics

C. NURSING MANAGEMENT

1. Monitor for signs of bleeding of all hema tests :

blood, urine, stool, git

2. Administer IV fluid replacement as ordered

3. Administer oxygen inhalation as ordered

4. Administer medications as ordered Vitamin K

Heparin Pitressin (Vasopressin) via heplock

5. Provide Heplock, avoid IM, SQ and any

venipunctures

6. Institute NGT decompression

Iced saline solution

Cold saline solution

Lavage: 500-1000cc of water; monitor NGT

output

7. Prevent complications

Hypovolemic shock (first sign: cold clammy

skin) (+) Anuria

IX. IDIOPATHIC THROMBOCYTOPENIA PURPURA

Increased destruction of platelets with resultant

platelet count of less that 100,000/mm3 characterized

by petechiae and ecchymoses of the skin.

Exact cause unknown; may be autoimmune.

Spleen is the site for destruction of platelets

often triggered by URTI or Childhood communicable

disease – Measles & chickenpox

A. ASSESSMENT:

1. Petechiae

2. Ecchymosis

3. Blood in any body secretions, bleeding form

mucous membranes, nosebleeds.

4. Decreased platelet count

5. Anemia

6. easy bruising

7. blood in stool or urine

8. CBC reveals platelet count below 20,000/mm3

9. Bone marrow aspiration done to rule out

leukemia

B. MEDICAL MANAGEMENT:

Drug therapy:

1. Prednisone – decreases anti-platelet antibodies

(monitor for infection)

2. IVIG (Intravenous Immune Globulin) – helps

to effectively increase platelet count

3. Anti-D Antibody – one dose treatment

Given to pt’s 1 year but less than 19 years

old

Normal WBC and hemoglobin

no active bleeding present

no concurrent infection

Diphenhydramine and hydrocortisine are

made ready for possible allergic reactions

to the medication

Platelet transfusion

Splenectomy

C. NURSING MANAGEMENT

1. Prevent, control and minimize bleeding.

2. Prevent bruising

3. Provide support to client and be sensitive to

change in body image.

4. Protect from infection.

5. Administer analgesics (acetaminophen) as

ordered; avoid aspirin.

6. administer meds orally, rectally, or I.V. rather

than I.M.




10 MS Abejo

BLOOD TRANSFUSIONS

I. OBJECTIVES

A. To replace circulating blood volume

B. Increase oxygen carrying capacity of the blood

C. Combat infections if decreased WBCs

D. Prevent bleeding if decreased PLT

II. NURSING MANAGEMENT/PRINCIPLES

A. Proper refrigeration

B. Proper blood typing and cross-matching

1. Type O – universal donor

2. Type AB – universal receipient

3. 85% of general population is Rh (+)

Blood expiration:

Platelets : 5 days

RBC: 5-7 days, 250 cc

C. Aseptically assemble all materials needed for BT

1. Filter set (BT set)

2. PNSS to prevent hemolysis

3. 18-19 gauge large bore needle to prevent

hemolysis

D. Instruct another RN to re-check the following:

1. Name of patient

2. Bt and ct

3. Expiration date

4. Serial number

E. Check blood unit for presence of bubbles, cloudiness,

sediments and dark color as it may indicate

contamination

F. NEVER WARM BLOOD PRODUCTS! ROOM

TEMPERATURE ONLY 1. Warming only done if you have dewarming devise 2. Warming only done during emergency situations if

there is massive blood loss massive transfusion

G. Transfusion should be completed in 4 HOURS

because blood exposed to room temperature more than

2 hours causes blood deterioration

H. Avoid mixing or administering drug at BT line to

prevent hemolysis

I. Regulate at KVO (10-15 gtts/min) at 100 cc/hour to

prevent circulatory overload J. MONITOR VS BEFORE, DURING, & AFTER

TRANSFUSION ESPECIALLY EVERY 15 MINUTES

FOR THE FIRST HOUR. Majority of BT reactions occurs at these times

K. SIGNS OF BT REACTION (HAPCATCH)

1. Hemolytic reactions life threatening.

PRIORITY

Signs and symptoms

Dizziness, Headache, Dyspnea,

Hypotension, Flushed skin, Lumbar,

flank and sternal pain, diarrhea or

constipation, Portwine urine (red urine)

Nursing Management

Stop BT, Notify MD, Flush with PNSS

Administer Isotonic Solution to

counteract shock and prevent acute

tubular necrosis

Return blood unit to blood bank for re-

examination

Obtain urine and blood sample of client

for re-examination and send to lab

Monitor VS and IO

2. Allergic reactions

Signs and symptoms

Fever, chills, dyspnea, Laryngospasm,

bronchospasm, Bronchial wheezing,

Urticaria, pruritus, skin rashes

Nursing Management

Stop BT, Notify MD, Flush with PNSS

Diphenhydramine administration as

ordered

If (+) to hypotension, it indicates

anaphylactic shock


examination


for re-examination

Monitor VS and IO

3. Pyrogenic reactions

Signs and symptoms

Fever and chills

Headache

Dyspnea

Tachycardia and palpitations

Diaphoresis

Nursing Management

Stop BT

Notify MD

Flush with PNSS

Administer antipyretics and antibiotics

as ordered

Provide hypothermic blanket


examination


for re-examination

Monitor VS and IO

4. Circulatory overload

Signs and symptoms

Dyspnea

Rales/crackles

Orthopnea

Nursing Management

Stop BT

Notify MD

Administer loop diuretics as ordered

NO FLUSHING!

Monitor VS and IO

5. Air embolism

6. Thrombocytopenia

7. Citrate intoxication

8. Hyperkalemia arrhythmia

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