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Approach to hemolytic anemia

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Approach to hemolytic anemia

Approach to hemolytic anemia

Candidate: Dr SARATH MENON.R

K.B.ILLAVA HEMATOLOGY DIVISION DEPT.MEDICINE, MGM MEDICAL COLLEGE,INDORE

objectivesLab indication of hemolysis

Intravascular v/s extravascular hemolysis

D/D of hemolytic anemia

Diagnose hemo.anemia with peripheral smear & ancillary lab tests

Hemolytic Anemia

Definition:Those anemias which result from an increase in RBC destruction coupled with increased erythropoiesis

Classification:Congenital / HereditaryAcquired

CLASSIFICATION OF HEMOLYTIC ANEMIASINTRACORPUSCULAR DEFECTSEXTRACORPUSCULAR FACTORSHEREDITARYHEMOGLOBINOPATHIES

ENZYMOPATHIES

MEMBRANE-CYTOSKELETAL DEFECTSFAMILIAL HEMOLYTIC UREMIC SYNDROMEACQUIREDPAROXYSMAL NOCTURNAL HEMOGLOBINURIAMECHANICAL DESTRUCTION [MICROANGIOPATHIC] TOXIC AGENTSDRUGSINFECTIOUSAUTOIMMUNE

Classification MAHATransfusion rxPNHInfectionsSnake biteHemoglobinopathiesEnzymopathiesMembrane defectsAIHAIntravascular hemolysisExtravascular hemolysis

How is Hemolytic Anemia Diagnosed?

Two main principles

One is to confirm that it is hemolysis

Two is to determine the etiology

How to diagnose hemolytic anemiaNew onset pallor or anemia

Jaundice

Splenomegaly

Gall stones

Dark colored urine

Leg ulcers

GENERAL FEATURES OF HEMOLYTIC DISORDERSGENERAL EXAMINATION - JAUNDICE, PALLOR BOSSING OF SKULLPHYSICAL FINDINGS - ENLARGED SPLEENHEMOGLOBIN - FROM NORMAL TO SEVERELY REDUCEDMCV - USUALLY INCREASEDRETICULOCYTES - INCREASEDBILIRUBIN - INCREASED[MOSTLY UNCONJUGATED]LDH - INCREASEDHAPTOGLOBULIN - REDUCED TO ABSENT

Hemolytic facies- chipmunk facies

Laboratory Evaluation of HemolysisExtravascularIntravascularHEMATOLOGICRoutine blood filmReticulocyte countBone marrow examinationPolychromatophilia

Erythroid hyperplasiaPolychromatophilia

Erythroid hyperplasiaPLASMA OR SERUMBilirubinHaptoglobinPlasma hemoglobinLactate dehydrogenase Unconjugated , Absent N/ (Variable) UnconjugatedAbsent (Variable)URINEBilirubinHemosiderinHemoglobin +00 +++ severe cases

POLYCHROMATOPHILIC CELLS

The key to the etiology of hemolytic anemia The history The peripheral blood film

Patient History

Acute or chronicMedication/Drug precipitants G6PD AIHAFamily historyConcomitant medical illnessesClinical presentation

Case 13 yr old male child presenting with pallor,jaundice,Severe pain of long bones, feverCBC-anemia,reticulocytosis,increased WBCLAB - LDH -600 (normal upto 200) S.bilirubin- 5mg%

Peripheral smear

What is the diagnosis ?SICKLE CELL ANEMIA

DIAGNOSIS OTHER TESTS

Hemoglobin electrophoresis -HbS >80% -HbF -1-20% -HbA2 -2- 4.5% Sickling test POSITIVE

Sickle cell diseaseMutn .beta globin-6 Glu Val. Deoxy HbS (polymerised)

Ca influx, K leakage

stiff,viscous sickle cell

venocclusion dec.RBC survival

microinfarctions,isch.pains anemia,jaundice, autoinfarct.spleen gallstones,leg ulcers

Clinical manifestationsHemo.anemia,reticulocytosis,granulocytosisVasoocclusion-protean Painful crisesSplenic sequestration crisesHand foot syndromeAcute chest syndrome

Diagnosis?

SICKLE THALASSEMIA`

Clinical features of sickle hemoglobinopathiesConditionClinical abnormHb level g%MCV,flHb electrophoSickle cell traitNone,rare painlss hematurianormalnormalHbS/A: 40/60 Sickle cell anemiaVasocclusive crises,AVN,gallstones, priapism7-1080-100HbS/A:100/0HbF;2-25%S/beta0thalasssemiaVasoocclusiveCrises,AVN7-1060-80HbS/A-100/0HbF; 1-10%S/beta+ thalassemiaRare crises,AVN10-1470-80HbS/A:60/40HbSC--do--, retinopathy 10-14 80-100HbS/A;50/0HbC;50%

CASE 26 yr old child presenting with severe pallor,jaundice growth delayAbnormal facies,hepatosplenomegaly+h/o recurrent blood transfusionsCBC-Hb -3gm%, MCV-58FL(Nl-86-98), -MCH- 19pg (nl-28-33) P.S- MICROCYTIC,HYPOCHROMIA with target cells +

Diagnosis?

Target cells

thalassemiaOther diagnosis test-Hb electrophoresisDNA analysis for mutationsAlpha thalassemia & beta thalassemiaBeta thalassemia- major - intermedia - minor

Beta thalassemiaMutn. Beta globin expression

M.C- derange splicing of m-RNA

HYPOCHROMIA ,MICROCYTIC anemia

Beta thalassemia majorSevere homozygousChildhood, growth delaySevere anemia,hepatosplenomegaly,r/r transfusionIron overload-endo.dysfnct

P.Smear- severe microcytosis,target cells

Hb electro- HbF - 90-96 % HbA2- 3.5 %- 5.5% HbA - 0 %

Beta thalassemia intermediaSimilar stigmata like majorSurvive without c/c transfusion Less severe than majorModerate anemia,microcytosis,hypochromia Hb electrophor- HbF - 20-100% HbA2 -3.5%-5.5% HbA 0-30%

Beta thalassemia minorProfound microcytosis,target cellsMinimal anemiaSimilar bld picture of iron def.anemiaLab inv: MCV

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