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
