pediatric hematology lecture

7/25/2019 Pediatric Hematology Lecture

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The Child with Hematologicor Immunologic Dysfunction

Chapter 26


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Assessment of

Hematologic Function Complete blood count History and assessment ndings

Childs energy and acti!ity le!el

"rowth patterns


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Anemia

The most common hematologic disorder ofchildhood

Decrease in number of #$Cs and%or hemoglobinconcentration below normal

Decreased o&ygen'carrying capacity of blood


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Classication of Anemias

(tiology and physiology

#$C and%or Hgb depletion

)orphology

Characteristic changes in #$C si*e+ shape+ and%or

color


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Conse,uences of Anemia

Decrease in o&ygen'carrying capacity of bloodand decreased amount of o&ygen a!ailable totissues

-hen anemia de!elops slowly+ child adapts


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(.ects of Anemiaon Circulatory /ystem Hemodilution Decreased peripheral resistance Increased cardiac circulation and turbulence

)ay ha!e murmur

)ay lead to cardiac failure Cyanosis "rowth retardation


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Diagnostic (!aluation

C$C

Decreased #$Cs

Decreased Hbg and Hct

0ther tests for particular type of anemia


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Therapeutic

)anagement Treat underlying cause Transfusion after hemorrhage if

needed

Nutritional intervention for deciencyanemias

Supportive care

IV uids to replace intravascular

volume Oxygen

Bed rest


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1ursing Considerations

repare child and family for laboratory tests

Decrease o&ygen demands

re!ent complications

/upport family


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Iron Deciency Anemia

Caused by inade,uate supply of dietary iron

"enerally pre!entable

Iron'fortied cereals and formulas for infants

/pecial needs of premature infants

Adolescents at ris3 due to rapid growth and pooreating habits


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Most common nutritional prolem in !S "aused y any numer of factors that

decrease the supply of #e$ impair itsasorption$ increase the ody%s needfor #e$ or a&ect the synthesis ofhemogloin'

(arge mil) inta)e *ith lo* solid foodsource

+apid gro*th

Inade,uate diet$ menses$ impairedasorption


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athophysiology facts Birth- full term infant

#e supply./00mg

-1uring the last

trimester-#e transferfrom mom 2 rateof 3mg4day

Maternal #e storesare ade,uate for upto 3-5 months

If diet doesn%tsupplement thematernal4fetalstores


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Iron Deciency AnemiaClinical )anifestations

Mil) ay-over*eight Mil) is a poor source of iron$

Vit'"$ 6inc 7 #louride

8ale$ porcelain-li)e s)in !nder*eight 8oor muscle dev$edema$

retarded gro*th$ Irritale$ tachycardic$ fatigue$

)oilonychia Social and cognitive ailities


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re!ention%/creening%Teaching Dietary counseling Iron Fortied formula%cereal up to 42 m Iron rich foods upon solids Fe supplementation 5Ferrous sulfate 'gi!e b%w meals%7it C to absorption

'8eep no more than 4m supply9to&ic

Mgmt4Nursing


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/ic3le Cell Anemia

A hereditary hemoglobinopathy

(thnicity

0ccurs primarily in blac3s

0ccurrence 4 in :;< infants born in =/ 4 in 42 ha!e sic3le cell trait

0ccasionally also in persons of)editerranean descent

Also seen in /outh American+ Arabian+and (ast Indian descent


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(tiology of /ic3le Cell

In areas of world where malaria is common+indi!iduals with sic3le cell trait tend to ha!esur!i!al ad!antage o!er those without trait

Autosomal recessi!e disorder

4 in 42 blac3s are carriers 5ha!e sic3le celltrait

5If both parents ha!e trait+ each o.springwill ha!e 4 in > li3elihood of ha!ing disease


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athophysiology

artial or complete replacement of normal Hgbwith abnormal hemoglobin / 5Hgb /

Hemoglobin in the #$Cs ta3es on an elongated?sic3le@ shape

/ic3led cells are rigid and obstruct capillary bloodow

)icroscopic obstructions lead to engorgement andtissue ischemia

Hypo&ia occurs and causes sic3ling


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/ic3led Hemoglobin

=nder conditions of dehydration+ acidosis+hypo&ia+ and temperature ele!ations+ Hgb/

changes its structure in the cell membrane froma pliable dis3 to a crescent or sic3le shaped #$CB

/ic3ling response is re!ersible with o&ygenation hydration


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athophysiology

arge tissue infarctions occur

Damaged tissues in organsE impaired function

/plenic se,uestration

)ay re,uire splenectomy at early age


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rognosis

No cure 9except possily one marro*transplants:

Supportive care4prevent sic)lingepisodes

#re,uent acterial infections may occurdue to immunocompromise Bacterial infection is leading cause of

death in young children *ith sic)le celldisease

Stro)es in ;


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/ystems A.ected


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/ic3le Cell Crisis

recipitating factors Anything that increases bodys need for o&ygen or

alters transport of o&ygen Trauma Infection+ fe!er

hysical and emotional stress Increased blood !iscosity due to dehydration Hypo&ia

From high altitude+ poorly pressuri*ed airplanes+hypo!entilation+!asoconstriction due tohypothermia


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/ic3le Cell Crisis 5contd

Acute e&acerbations that !ary in se!erity andfre,uency

Types

7aso'occlusi!e 70CG thrombotic )ost common type of crisis!ery painful

/tasis of blood with clumping of cells inmicrocirculationischemiainfarction

/ignsJ fe!er+ pain+ tissue engorgement


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/ic3le Cell Crisis 5contd

Types 5contd

/plenic se,uestration

ife threateningdeath can occur within hours

$lood pools in the spleen

/igns rofound anemia+ hypo!olemia+ and shoc3


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Diagnosis of /ic3le Cell

Cord blood in newborns

1ewborn screening done in >: states

"enetic testing to identify carriers and childrenwho ha!e disease

/ic3le'turbidity test

Kuic3 screening purposes in children L6 mos of age


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)edical )anagement

Aggressi!e treatment of infection

ossible prophylactic antibiotics from 2 mos'< yrs


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)edical )anagement

5contd )onitor reticulocyte count regularly to e!aluatebone marrow function

$lood transfusion+ if gi!en early in crisis+ mayreduce ischemia


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)edical )anagement

5contd Fre,uent transfusion decreases hemosiderosis5iron in tissues

Treat with iron'chelation such as fero&amine M!itamin C to promote iron e&cretion


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1ursing )anagement

)onitor childs growthwatch for failure to thri!e

Careful multi'system assessment

Assess pain

0bser!e for presence of inammation or possibleinfection

Carefully monitor for signs of shoc3


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sychosocial 1eeds

Coping mechanisms

/upport with genetic counseling

Financial needs

Caregi!er role strain

i!ing with chronic illness in the family


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Hemophilia

A group of hereditary bleeding disorders thatresult from deciencies of specic clotting factors


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Hemophilia

N lin3ed recessi!e disorder

Carried on female chromosomeE males are

a.ected Deciency of Factor O+ produced by the

li!er+ necessary for the formation ofthromboplastin for clotting


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Types of Hemophilia

Hemophilia A

?Classic hemophilia@

Deciency of factor 7III

Accounts for OPQ of cases of hemophilia

0ccurrenceJ 4 in


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Hemophilia A'/e!erity

Degrees /e!ere'/pontaneously bleed w%o trauma )oderate'$leed with trauma

)ild'se!ere trauma%surgery


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Types of Hemophilia

5contd Hemophilia $ Also 3nown as Christmas disease

Caused by deciency of factor IN

Accounts for 4


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)anifestations of

Hemophilia $leeding tendencies range from mild tose!ere

/ymptoms may not occur until 6 mos of age

)obility leads to inRuries from falls andaccidents

Hemarthrosis

$leeding into Roint spaces of 3nee+ an3le+

elbow leading to impaired mobility (cchymosis


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)anifestations 5contd

(pista&is

$leeding after procedures

)inor trauma+ tooth e&traction+ minor surgeries

arge subcutaneous and intramuscular hemorrhages

may occur $leeding into nec3+ chest+ mouth may compromise

airway


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Clinical Therapy

Can be diagnosed through amniocentesis

"enetic testing of family members to identifycarriers

Diagnosis on basis of h&+ labs+ and e&am

SabsJ ow le!els of factor 7III or IN+ prolonged TT

S1ormalJ platelet count+ T+ and brinogen


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)edical )anagement

11>V8 IV "auses ?-3 @ increase in factor

VIII activity !sed for mild hemophilia

+eplace missing clotting factors Transfusions

>t home *ith prompt interventionto reduce complications

#ollo*ing maAor or minorhemorrhages


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rognosis

Historically+ most died by < yrs age

1ow mild to moderate hemophilia patients li!enear normal li!es

"ene therapy for future

Infused carrier organisms act on target cells topromote manufacture of decient clotting factor


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Inter!entions

"lose supervision and safeenvironment

1ental procedures in controlled

situation

Shave only *ith electric ra6or

Supercial leedingapplypressure for at least =; minutesC ice to vasoconstrict

If signicant leeding occurs$transfuse for factor replacement


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)anaging Hemarthrosis

During bleeding episodes+ ele!ate and immobili*eRoint

Ice Analgesics #0) after bleeding stops to pre!ent contractures T A!oid obesity to minimi*e Roint stress


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7on -illebrand Disease

> hereditary leeding disorder$ involvingdeciency of Von Dillerand factor$ 9aplasma protein$ and the carrier forfactor VIII:

Von Dillerand factor is necessary forplatelet adhesion

Transmitted as autosomal dominant trait

Occurs in males and females

Eene for disease is located onchromosome =?


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)anifestations of7on -illebrand Disease (asy bruising

(pista&is

"ingi!al bleeding

(&cessi!e bleeding with lacerations or surgeries

)enorrhagia


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Diagnosisaboratory Findings Decreased 7on -illebrand factor le!els

7on -illebrand antigen le!els

Decreased platelet agglutination

rolonged bleeding time

TT may be normal or prolonged


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Treatment of7on -illebrand Disease Infusion of 7on -illebrands protein concentrate

DDA7 infusion prior to surgery or to treatbleeding episode 5synthetic !asopressin

Aminocaproic acid 5Amicar to treat bleeding in

mucous membranes 5in some cases


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Inter!entions

A!oid aspirin or 1/AIDs 5increased bleeding timeand inhibit platelet function

)anage bleeding episodes with prompt infusiontherapy

Children with 7on -illebrands ha!e normal lifee&pectancy if well managed


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IdiopathicThrombocytopenic urpura5IT An ac,uired hemorrhagic disorder characteri*ed

by

ThrombocytopeniaJ e&cessi!e destruction ofplatelets

urpuraJ discoloration caused by petechiae beneaththe s3in


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IT Forms

Acute self'limiting

0ften follows =#I or other infection

Chronic 5L6 months duration


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IT

Diagnostic e!aluation

Therapeutic management

rognosis

1ursing considerations


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(pista&is 51osebleeding

Isolated and transient epista&is is common inchildhood

#ecurrent or se!ere episodes may indicateunderlying disease

7ascular abnormalities+ leu3emia+ thrombocytopenia+clotting factor deciency diseases 57on -illebranddisease and hemophilia


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1ursing Considerations(pista&is #emain calm+ 3eep child calm

Ha!e child sit up and lean forward

ressure to nose

Further e!aluation if bleeding continues


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1eoplastic Disorders

eading cause of death from disease in childrenpast infancy

Almost half of all childhood cancers in!ol!e bloodor blood'forming organs


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eu3emias

)ost common form of childhood cancer

:'> cases per 4PP+PPP caucasian children 4< yrsold

)ore fre,uent in males L4 yr old

ea3 onset between 2 and 6 yrs of age

/ur!i!ability


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Classication of

eu3emias eu3emiaJ A broad group of malignant disease ofbone marrow and lymphatic system Comple& disease with !arying heterogeneity

Classications are increasingly comple&


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)orphology

Acute lymphoid leu3emia 5A

Acute non'lymphoid 5myelogenous leu3emia5A1 or A)

/tem cell or blast cell leu3emia


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/ymptoms

AJ lymphatic+ lymphocytic+ lymphoblastic+ andlymphoblastoid leu3emia

A)J granulocytic+ myelocytic+ monocytic+myelogenous+ monoblastic+ and non'myeloblastic

leu3emia


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rognosis

A'SSinitial -$C countSS5


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athophysiology

eu3emia is an unrestricted proliferation ofimmature -$Cs in the blood'forming tissues ofthe body

i!er and spleen are the most se!erely a.ected

organs


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athophysiology 5contd

Although leu3emia is an o!erproduction of -$Cs+often acute form causes low leu3ocyte count

Cellular destruction ta3es place by inltration andsubse,uent competition for metabolic elements

C f


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Conse,uences of

eu3emia Anemia from decreased #$Cs Infection from neutropenia

$leeding tendencies from decreased plateletproduction

/pleen+ li!er+ and lymph glands show mar3edinltration+ enlargement+ and brosis


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Diagnostic (!aluation

$ased on history+ physical manifestations

eripheral blood smear

Immature leu3ocytes

Fre,uently low blood counts

to e!aluate C1/ in!ol!ement $one marrow aspiration or biopsy


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)gmtB%Follow rotocols

Chemotherapeutic agents

Cranial irradiation 5in some cases


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)gmtB%Follow rotocols

"hemotherapy9antineoplasticagents:

+adiotherapy Immunotherapy

3 phasesF Induction Intensication4co

nsol' "NS prophylax Maintenance

BMTFallogeneic4autologous

Prepare families for dx/txprocedures

Provide cont emotionalsupport

Reverse isolation FYI=BMA center of the

one is hollo! andcontains the cells that laterecome !or"in#$ loodcells or leu"emic cells


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Four hases of Therapy

Induction therapyJ >'6 wee3s

C1/ prophylactic therapyJ intrathecalchemotherapy

Intensication 5consolidation therapyJ To

eradicate residual leu3emic cells and pre!entresistant leu3emic clones

)aintenance therapyJ to preser!e remission

H t i ti /t C ll


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Hematopoietic /tem CellTransplantation 5H/CT

Donors may be relati!es or non'relati!es

Antigen'matched or mismatched

eripheral stem cells may be used

/tem cells from umbilical cord blood


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#is3s of H/CT

/ignicant ris3 of morbidity and mortality

"raft !sB host disease 5"7HD

0!erwhelming infection

/e!ere organ damage

Cure after H/CTJ up to 6PQ';PQ


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rognosis

If relapse after H/CTJ dismal prognosis Identied factors for determining prognosis

Initial -$C count Age at time of diagnosis Type of cell in!ol!ed

"ender 8aryotype analysis


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1ursing Considerations

Assessment

1ursing diagnosis

lanning

Implementation

repare child and family for procedures

ain management

re!ent complication of myelosuppression

Increased /usceptibility


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Increased /usceptibilityto Infection At time of diagnosis and relapse

During immunosuppressi!e therapy

After prolonged antibiotic therapy thatpredisposes to the growth of resistant organism


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Infection Control

(n!ironment

Hand hygiene

7isitor restriction

Nursing "are


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Infection Control

Manage prolemsof irradiation anddrug toxicity .

N4V$ anorexia$mucosalulceration$neuropathy$hemorrhagic

cystitis$ alopecia$moon face$ moodchanges$ pain

Nursing "are

provide favored foods

mouth care, soft toothbrush,mouthwash, hydrogen

peroxide, saline

Vincristine, numbness temp

temporary, wigs, hats

decrease Na intake, look

healthyanalgesics

)anaging


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)anagingChemotherapeutic Agents ?7esicants@sclerosing agents e!en in minute

amounts

Inter!entions for e&tra!asation

#is3 for anaphyla&is


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1ursing Diagnoses

#is3 for inRury related to malignant process+treatment

#is3 for uid !olume decit related to nausea+!omiting

Altered nutrition

Impaired s3in integrity

Altered family processes

Fear related to diagnosis+ procedures+treatments


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ymphomas

Hodg3in disease )ore pre!alent in 4 yrs of age


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Hodg3in Disease

1eoplastic disease originating in lymphoid system

0ften metastasi*es to spleen+ li!er+ bone marrow+lungs+ and other tissues

i i


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Diagnostics

Clinical manifestations of Hodg3in disease

ymph node biopsy for diagnosis and staging

resence of #eed'/ternberg cells is characteristicof Hodg3in disease

Therapeutic


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Therapeutic)anagement #adiation

Chemotherapy 5alone or with radiation

rognosis

1ursing considerations

1 H d 3i h


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1on'Hodg3in ymphoma

Appro&imately 6PQ of pediatric lymphomasare 1H

Clinical appearance Disease usually di.use rather than nodular

Cell type undi.erentiated or poorlydi.erentiated Dissemination occurs early+ often+ and

rapidly )ediastinal in!ol!ement and in!asion of

meninges

Immunologic Deciency


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Immunologic DeciencyDisorders

Severe Combined ImmunodeficiencySyndrome (SCID) and Wiskott-AldrichSyndrome-the body is unable to mount an

immune response.he immune system!s function is toreco"ni#e $self% from $nonself% and toinitiate a response to eliminate the

$nonself% or anti"en.Cell surface markers&body cells 'ithspecific cell surface markers uniue to theindividual


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rotecti!e )echanisms

Intact s)in Saliva$ s*eat$

tears$ stomachacids

Snee6ing$coughing

8rimary lymphoidorgans9thymus$BM$liver:

Secondarylymphoid organs9spleen$ lymphnodes$ E>(T:

7ertical Transmission


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7ertical Transmission5erinatal

HI7 M mother to her newborn

Antepartum

IntrapartumJU deli!ery ostpartumJ !ia breastfeeding'minimal in =B/B+

carries a ris3 as high as 2OQ

1 i # ibiliti


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1ursing #esponsibilities

GIt%s the (a*H

M%&' counsel ( offer testin# to those !hoappear at deliver) !ith *+ record of an ,I- testdurin# pre#nanc)

If a !oman declines ,I- testin#. a si#nedoection M%&' e attempted

+ffer testin# to a child. !hose mother has not

een tested 0hilds ,I- status is strictl) confidential. schools

ma) not have info unless parents allo!


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Therapeutic Mgmt'

Assess compliance

Assuring adherence to med. schedule

Promoting weight gainclinical improvementsinclude growth retardation improvements,decrease hepatospleno, encephalopathy, andimmune sys. !x.

Nutritional mgmt to help !""

pediatric hematology lecture

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