his127 slide anemia

7/28/2019 His127 Slide Anemia

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AnemiaAnemia

zzDefinition :Definition :

Condition in which the concentration ofCondition in which the concentration ofHemo lobinHemo lobin or the red cell mass isor the red cell mass is

reduced below normalreduced below normal

1


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Table 1. Normal red blood cel l values in chi ldren

AGEAGE MeanMean -- 2 SD2 SD MeanMean -- 2 SD2 SD

Birth (cord blood)Birth (cord blood) 16.516.5 13.513.5 108108 9898

11--3 days (capil lary)3 days (capillary)

1 week1 week

2 weeks2 weeks

18.518.5

17.517.5

16.516.5

14.514.5

13.513.5

12.512.5

108108

107107

105105

9595

8888

8686

2 months2 months

33--6 months6 months

0.50.5--2 years2 years

..

11.511.5

11.511.5

12.012.0

..

9.09.0

9.59.5

10.510.5

9696

9191

7878

7777

7474

7070

22--6 years6 years66--12 years12 years

1212--18 years, female18 years, female

--

12.512.513.513.5

14.014.0

11.511.511.511.5

12.012.0

81818686

9090

75757777

7878

,,

1818--49 years, female49 years, female

1818--49 years, male49 years, male

..

14.014.0

15.515.5

..

12.012.0

13.513.5

9090

9090

8080

8080 2Hasting, C. Anemia , In: Hematology/oncology handbook, 2002 ; 3


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Blood Smear

H ocromic

Microcytic

MCV LOW

( Red Cell Size < 70 fl)

MCV HIGH

( Red Cell Size > 85 fl )

MCV NORMAL

( Red Cell Size 72-79 fl )Acu te bl ood loss

Thalassemia

Sideroblastic anemia

Chronic disease

(Infection, Cancer,inflamation, renal disease)

5. Lead toxicity

o rma new orn

Increased erythropoisi s

Postsplenectomy

Liver disease

Obstructive jaundice

Infection

Renal failure

Conective tissue disorder

Liver desease

6. Hemoglobin E trait

7. Atransferrinemia

8. In bo rn er ro rs o f i ro n m et ab ol ism

9. Copper deficiency

10. Sev ere maln utr is io n

Apl asti c anemi aHypothyroidism

Megaloblastic anemia

Down syndrome

Syndrome with elevatedHigh F

Early iron deficiency

Apl asti c anemi a

Bone marrowinfiltration

Dyserytropoitic anemia

Myelodysplastic syndr ome

Diamond-Blackfan syndro me

Fanconi anemia

Dyskeratosis Congenital

Pearson s ndrome

Hemolysis

RBC enzym deficiency

RBC membrane defectsHypersplenism

Drugs

3Lanzkowsky P,Manual of Pediatric Hematology andOncology 4 th eds.


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Laboratory Studies often Helpful in InvestigationLaboratory Studies often Helpful in Investigation

of a patient with anemiaof a patient with anemiazz Usual Initial studiesUsual Initial studies

Hemoglobin and hematocrit determinationHemoglobin and hematocrit determinationErythrocyte count and red cell indices, including MCV and RDWErythrocyte count and red cell indices, including MCV and RDWReticu ocyte countReticu ocyte count

Study of stained blood smearStudy of stained blood smearLeucocyte count and differential countLeucocyte count and differential countPlateled countPlateled count

Free erytrocyte protoporphyrinFree erytrocyte protoporphyrinSerum ferittin levelsSerum ferittin levels

Stool for occult bloodStool for occult blood --

Endoscopy (upper and lower bowel)Endoscopy (upper and lower bowel)-- if indicatedif indicated

zz Suspected Vitamin B12 or folic acidSuspected Vitamin B12 or folic acidBone MarrowBone Marrow

erum v tam n eveerum v tam n eveSerum folate levelSerum folate levelGastric analysis after histamine injectionGastric analysis after histamine injection

Vitamin B12 absorption test (radioactive cobalt) (Schilling testVitamin B12 absorption test (radioactive cobalt) (Schilling test))

zz Suspected hemolytic anemiaSuspected hemolytic anemia


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ContinuedContinuedzz Suspected hemolytic anemiaSuspected hemolytic anemia

Evidence of red cell breakdownEvidence of red cell breakdownoo smearoo smear

Serum billirubin levelSerum billirubin levelUrinary urobilinogenUrinary urobilinogenHemoglobinuriaHemoglobinuriaerum ap og o nerum ap og o n

Evidence of red cell regenerationEvidence of red cell regenerationReticulocyte countReticulocyte countBlood smearBlood smear

eta ra ograp seta ra ograp s

Evidence of type of hemolytic anemia: corpuscularEvidence of type of hemolytic anemia: corpuscular

MembraneMembraneBlood smearBlood smearOsmotic fragility testOsmotic fragility test

Autohemolysis testAutohemolysis test

HemoglobinHemoglobinSickle testSickle test

Hemoglobin electrophoresisHemoglobin electrophoresisHemoglobin F determinitationHemoglobin F determinitationKleihauerKleihauer--Betke smearBetke smearHeatHeat--stability teststability test

Enzyme assayEnzyme assay


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ContinuedContinued

Evidence of type of hemolytic anemia : extracorpuscularEvidence of type of hemolytic anemia : extracorpuscularImmuneImmuneAntiglobulin testAntiglobulin testAcid serum lysis testAcid serum lysis test

DonathDonath--Landsteiner antibodyLandsteiner antibodyANAANA

Suspected aplastic or leukemiaSuspected aplastic or leukemiaBone marrow (aspiration or biopsy)Bone marrow (aspiration or biopsy)--cytocemistry, immunologiccytocemistry, immunologic

markers, cromosome analysismarkers, cromosome analysise a ra ograp se a ra ograp s

Other test often used espicially to diagnose the primary diseaseOther test often used espicially to diagnose the primary disease, . ., . .

ANA, complemant, CH50ANA, complemant, CH50Blood urea, creatinin, T4,TSH, Tissue biopsy (skin, lympnode,liver)Blood urea, creatinin, T4,TSH, Tissue biopsy (skin, lympnode,liver)


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Investigation of AnemiaAnemia

History, physical examination, CBC

MCV

Reticulocyte count

Low Normal or high

Compatible with iron deficiency

Peripheral

smear

Peripheral

smear

Low

esNo

high

Response to

Trial of iron

Neutrophils,

platelets Investigate

o

HemolysisNo

Hemolysis

Blood loss

Specific tests

yes No

Hemolysis

ron e c ency,

physical, & red cell

morphology

of microcytic anemia

Pure red cell aplasia or

megaloblatic anemia

Bone marrow failure

8Low


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Table 3. Physical examination of the anemic child

Physical findingsPhysical findings Consider ConsiderSkinSkin PallorPallor

JaundiceJaundice

Severe anemiaSevere anemia

Hemolytic anemia, acute & chronic Hepatitis, aplasticHemolytic anemia, acute & chronic Hepatitis, aplastic

anemiaanemia

Auto immune haemol ti c anemia wi th th romboc to eniaAuto immune haemol ti c anemia wi th th romboc to enia,,

HemolyticHemolytic--uremic syndromeuremic syndrome

Bone marrow aplasia or infil trationBone marrow aplasia or infil tration

HeentHeent Cavernous hemangiomaCavernous hemangioma

Microangiopathic hemolytic anemiaMicroangiopathic hemolytic anemia

ron a oss ng, prom nen ma ar ron a oss ng, prom nen ma ar

maxillary bonesmaxillary bones

Icteric scleraeIcteric sclerae

x rame u ary ema opo es s a assem a ma or,x rame u ary ema opo es s a assem a ma or,

congenital hemolytic anemia)congenital hemolytic anemia)

Congenital hemolytic anemia & or hyperhemolyticCongenital hemolytic anemia & or hyperhemolytic

crisescrises

ngu ar s oma sngu ar s oma s

GlossitisGlossitis

Iron deficiencyIron deficiency

Vit. B12 or iron defic iencyVit. B12 or iron defic iency

ChestChest Rales, gallop rhythmRales, gallop rhythm Congestive heart failure, acute or severe anemiaCongestive heart failu re, acute or severe anemia

TachycardiaTachycardia

ExtremitiesExtremities Radial limb dysplasiaRadial limb dysplasiaSpoon nailsSpoon nails

Tr i halan eal thumbsTr i halan eal thumbs

Fanconis anemiaFanconis anemia

Iron deficiencyIron deficiency

Red cell a lasiaRed cell a lasia

SpleenSpleen SplenomegalySplenomegaly Congenital hemolytic anemia, infection, hematologicCongenital hemolytic anemia, infection, hematologic

malignancies, portal hypertension with resultantmalignancies, portal hypertension with resultanthypersplenismhypersplenism 7Hasting, C. Anemia , In: Hematology/oncology handbook, 2002 ; 5


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IRON DEFICIENCY ANEMIARON DEFICIENCY ANEMIAzz

zzAnemia resulting from lack of sufficient iron forAnemia resulting from lack of sufficient iron forsynthesis of hemoglobin.synthesis of hemoglobin.

zz Prevalence :Prevalence :zz The most common cause of anemia worlwideThe most common cause of anemia worlwide

zzAn estimated 30% of the worlds population :An estimated 30% of the worlds population :

zz4.5 bill ion4.5 bill ion @ anemicanemiczz500500 600 million600 million @Iron def. anemiaIron def. anemia

zz North American (1970) : 30North American (1970) : 30 -- 75% of child 175% of child 1 2 years2 years

of ageof agezz Israell i : 25% females (aged 16Israell i : 25% females (aged 16 -- 17 yrs)17 yrs)

zz n ones a :n ones a : age yrsage yrs

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Iron cycle 12


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Causes of Iron Deficiency AnemiaCauses of Iron Deficiency Anemia

I. Deficient intakeI. Deficient intakeDietary ( milk,0,5Dietary ( milk,0,5--1,5 iron /L)1,5 iron /L)

II. Increased demandII. Increased demandGrowth ( low birth weight, prematury, lowGrowth ( low birth weight, prematury, low--birthbirth--weigh twins or multiple brths,weigh twins or multiple brths,

, ,, ,

III.Blood lossIII.Blood lossA.A. PerinatalPerinatal

1.Placental (Transplacental bleeding into maternal Circulation,1.Placental (Transplacental bleeding into maternal Circulation,

Retroplacental, Intraplacental, Fetal blood loss at before birth)Retroplacental, Intraplacental, Fetal blood loss at before birth)

2.Umbilicus (rupture umbilical cord,In adequat cord tying, post exchange2.Umbilicus (rupture umbilical cord,In adequat cord tying, post exchangetransfusion)transfusion)

B.B. PostnatalPostnatal

Gastrointestinal tract, Hepatobiliary system,Pulmonary hemocideration,Gastrointestinal tract, Hepatobiliary system,Pulmonary hemocideration,

recurent epistaxisrecurent epistaxis

IV.Impaired absorptionIV.Impaired absorption

, ,, ,diared,postgastrectomy, inflamatory bowel desease, Helicobacter pyloridiared,postgastrectomy, inflamatory bowel desease, Helicobacter pyloriinfection associated chronic gastritisinfection associated chronic gastritis


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CLINICAL MANIFESTATIONSLINICAL MANIFESTATIONSConsequences of anemia :Consequences of anemia :

@ .. -- @compensatory mechanism (+)compensatory mechanism (+)


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Nonhematologic consequence :Nonhematologic consequence :

CLINICAL MANIFESTATIONS

zz Pica :Pica :zzPhagophagia (compulsive eating of ice)Phagophagia (compulsive eating of ice)

zzGeophagia : interest in dirt consumptionGeophagia : interest in dirt consumption @riskriskfor parasitic infestation & lead poisoning)for parasitic infestation & lead poisoning)

zz Ephitelial changes : Koilonychia, atrophy of lingualEphitelial changes : Koilonychia, atrophy of lingual

papilpapil

zz Exercise intoleranceExercise intolerancezz Behavioral changesBehavioral changes

zz norma ermogenes snorma ermogenes s

zzAltered host responseAltered host response 15


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Koilonychia 16


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LABORATORIUM FINDINGSABORATORIUM FINDINGSzz Red blood cells (RBCs)Red blood cells (RBCs)

zzChronic iron def.Chronic iron def. @ red cell indicesred cell indices (MCV, MCH,(MCV, MCH,

MCHC) :MCHC) : parallels with Hb concentrationparallels with Hb concentrationzzReticulocytes : slightlyReticulocytes : slightly zzRed cell morphology :Red cell morphology :

zzMild iron def.Mild iron def.@ hypochromic, anisocytosishypochromic, anisocytosiszzChronic iron def.Chronic iron def.@ oic loc tosis tar etsoic loc tosis tar ets

cells, ovalocytes, mycrocytes & cellcells, ovalocytes, mycrocytes & cell

fragments)fragments)

zzRBCs survival : shortenedRBCs survival : shortened17


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Poikylocytosis Anisocytosis 20


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Laboratorium findings Laboratorium findings

zz PlateletsPlatelets :: varies trombocytopenia to trombositosisvaries trombocytopenia to trombositosis

zz Iron metabolism :Iron metabolism :zz Serum iron :Serum iron : zz TIBC :TIBC : zz zz Serum ferritin : < 10Serum ferrit in : < 10 --12 g/dl12 g/dl

zz Factor VIII activity :Factor VIII activity : 22 3 fold.3 fold.

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DIAGNOSISIAGNOSISzz Based of :Based of :

zz HistoryHistory

zz

Physical examinationPhysical examinationzz Laboratorium findingsLaboratorium findings

zz Diagnostic criterias for iron deficiency anemia (IDA) :Diagnostic criterias for iron deficiency anemia (IDA) :

Criteria of IDA by WHO :Criteria of IDA by WHO :

1.1. Hb concentration < normal (age dependent)Hb concentration < normal (age dependent)

2.2. Mean Hb concentration < 31% ( N: 32Mean Hb concentration < 31% ( N: 32 -- 35%)35%)3.3. Serum iron < 50 Ug/dl (N: 80Serum iron < 50 Ug/dl (N: 80--180 ug/dl)180 ug/dl)

4.4. rans err n sa . < :rans err n sa . < : --

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Diagnosis Diagnosis

Criteria of IDA b Cook & MonsenCriteria of IDA b Cook & Monsen : :1.1. Microcytic hypochromic anemiaMicrocytic hypochromic anemia

.. ..

3.3. FEP levels > 100 ug/dl eritrositFEP levels > 100 ug/dl eritrosit

..

.... (Transferrin sat. serum ferrit in & FEP) are present.(Transferrin sat. serum ferrit in & FEP) are present.

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Diagnosis Diagnosis

Criteria of IDA by Lanzkowsky :Criteria of IDA by Lanzkowsky :

1. Peripheral smears :1. Peripheral smears :

-- Microcytic hypochromicMicrocytic hypochromic-- MCV MCH MCHC :MCV MCH MCHC : -- RDW > 17%RDW > 17%

3. Serum ferritin :3. Serum ferritin :

.. ,, , ., .5. Response to iron preparation5. Response to iron preparation

.. -- 24


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DIFFERENTIAL DIAGNOSISIFFERENTIAL DIAGNOSISIFFERENTIAL DIAGNOSISIFFERENTIAL DIAGNOSIS

zz Thalassemia traitThalassemia trait

zz Lead poisoningLead poisoning

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Disorders Associated with HypochromiaDisorders Associated with Hypochromia

. ron e c ency. ron e c ency2.Hemoglobinopathies2.Hemoglobinopathies

ThalassemiaThalassemia --, Hemoglobin Koln, Hemoglobin Koln--LeoporeLeopore--HH--EE..

Lead, Pyrazinamide, IsoniazideLead, Pyrazinamide, Isoniazide4.Sideroblastic anemias4.Sideroblastic anemias..

6.Malignancy6.Malignancy7.Hereditary orotic aciduria7.Hereditary orotic aciduria

8.H o8.H o-- or a transferrinemiaor a transferrinemiaCongenitalCongenital

Acquired (e.q,hepatic disorders); malignan disease, proteinAcquired (e.q,hepatic disorders); malignan disease, proteinmalnutrition (decreased transferin synthesis), nephroticmalnutrition (decreased transferin synthesis), nephrotic

9.Copper deficiency9.Copper deficiency10.Inborn error of iron metabolism10.Inborn error of iron metabolism


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TREATMENTREATMENTREATMENTREATMENTzz Treatment of IDA is twofold :Treatment of IDA is twofold :

zz Replenishment of body ironReplenishment of body ironzz Correction of the factors res onsible for theCorrection of the factors res onsible for the

deficiencydeficiency

zz Iron can be administered orally, IM, or IV.Iron can be administered orally, IM, or IV.

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.. Treatment

zz Oral iron therapyOral iron therapy

zz Ferrous salt : adsorbed 3X better than ferric saltsFerrous salt : adsorbed 3X better than ferric salts

zz Ferrous gluconate, ferrous fumarate, & ferrousFerrous gluconate, ferrous fumarate, & ferrous

sucsinat : well adsorbed but >> expensivesucsinat : well adsorbed but >> expensive

zz Dose : 4Dose : 4 6 mgkg of elemental iron in three divided6 mgkg of elemental iron in three divided

doses.doses.

zz n a equa e response o ron erapy :n a equa e response o ron erapy : concentration > 1g/dl in 10 days & reticulocytosisconcentration > 1g/dl in 10 days & reticulocytosis

supplementation.supplementation.

>>>>

temporary stainingtemporary staining of the teethof the teeth 27


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.. Treatment.. Treatment

zz Parenteral iron therapyParenteral iron therapy

zz Indicated : for steadfast noncompliance with oralIndicated : for steadfast noncompliance with oral

medication, iron malabsorption, ongoing iron lossmedication, iron malabsorption, ongoing iron loss

exceed.exceed.

zz repara on : ron ex ranrepara on : ron ex ran @ a comp ex o err ca comp ex o err chydroxide withhydroxide with molecular weight dextrans.molecular weight dextrans.

zz o e ron rema ns a e n ec on s e or o e ron rema ns a e n ec on s e or

monthsmonths @ staining of the skin.staining of the skin...

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.. Treatment

zz ry ropo e nry ropo e nzz Recombinant human erythropoietin (EPO)Recombinant human erythropoietin (EPO)

precursorsprecursors @ in heme synthesis.in heme synthesis. @ @

or SQor SQ

zz Children with very severe anemia (Hb < 5 g/dl)Children with very severe anemia (Hb < 5 g/dl)

exchange transfusionexchange transfusion @ avoid expansion of theavoid expansion of theblood volume.blood volume.

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.. Treatment

zz PreventionPrevention

zz Encouragement of breastEncouragement of breast feedingfeeding

zz Use of ironUse of iron -- fortified infant formulas after weaningfortif ied infant formulas after weaning

zz The introduction of wholes cows milk to 1 years ofThe introduction of wholes cows milk to 1 years of

ageage

zz

Use of ironUse of iron fortified infant cereals & ascorbid acidfortif ied infant cereals & ascorbid acid--r c oo s ar c oo s a mon smon s

zz Supplemental iron for preterm infants after the firstSupplemental iron for preterm infants after the first

..

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his127 slide anemia

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