APPROACH TO HEMOLYTIC ANEMIAGuide : Dr MANOJ GUPTA Candidate: Dr
SARATH MENON.R HEMATOLOGY DIVISION.DEPT.MEDICINE, MGM MEDICAL
COLLEGE,INDORE
OBJECTIVES
Lab 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:y
Those anemias which result from an increase in RBC destruction
coupled with increased erythropoiesis
Classification:Congenital / Hereditary y Acquiredy
CLASSIFICATION OF HEMOLYTIC ANEMIASINTRACORPUSCULAR DEFECTS
EXTRACORPUSCULAR FACTORS FAMILIAL HEMOLYTIC UREMIC SYNDROME
HEREDITARY
HEMOGLOBINOPATHIES ENZYMOPATHIES MEMBRANECYTOSKELETAL
DEFECTS
ACQUIRED
PAROXYSMAL NOCTURNAL HEMOGLOBINURIA
MECHANICAL DESTRUCTION [MICROANGIOPATHIC] TOXIC AGENTS DRUGS
INFECTIOUS AUTOIMMUNE
CLASSIFICATIONIntravascular hemolysis Extravascular
hemolysis
MAHA Transfusion rx PNH Infections Snake bite
Hemoglobinopathies Enzymopathies Membrane defects AIHA
HOW IS HEMOLYTIC ANEMIA DIAGNOSED?
Two main principlesy
One is to confirm that it is hemolysis
y
Two is to determine the etiology
HOW TO DIAGNOSE HEMOLYTIC ANEMIA
New onset pallor or anemia Jaundice Splenomegaly Gall stones
Dark colored urine Leg ulcers
GENERAL FEATURES OF HEMOLYTIC DISORDERS
GENERAL EXAMINATION
- JAUNDICE, PALLOR BOSSING OF SKULL
PHYSICAL FINDINGS - ENLARGED SPLEEN HEMOGLOBIN MCV RETICULOCYTES
BILIRUBIN LDH HAPTOGLOBULIN - FROM NORMAL TO SEVERELY REDUCED -
USUALLY INCREASED - INCREASED - INCREASED[MOSTLY UNCONJUGATED] -
INCREASED - REDUCED TO ABSENT
HEMOLYTIC FACIES- CHIPMUNK FACIES
Laboratory Evaluation of HemolysisExtravascular
IntravascularHEMATOLOGIC
Routine blood film Reticulocyte count Bone marrow
examinationPLASMA OR SERUM
Polychromatophilia Erythroid hyperplasia
Polychromatophilia Erythroid hyperplasia
Bilirubin Haptoglobin Plasma hemoglobin Lactate dehydrogenase
URINE Bilirubin Hemosiderin Hemoglobin
Unconjugated , Absent N/ (Variable)
Unconjugated Absent (Variable)
+ 0 0
+ + + severe cases
POLYCHROMATOPHILIC CELLS
THE KEY TO THE ETIOLOGY OFHEMOLYTIC ANEMIA
The
history peripheral blood film
The
PATIENT HISTORY
Acute or chronic Medication/Drug precipitants G6PD AIHA
Family history Concomitant medical illnesses Clinical
presentation
CASE 13 yr old male child presenting with pallor,jaundice,
Severe pain of long bones, fever
CBC-anemia,reticulocytosis,increased WBC LAB - 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 anemia,jaundice, gallstones,leg ulcers
microinfarctions,isch.pains autoinfarct.spleen
CLINICAL
MANIFESTATIONSHemo.anemia,reticulocytosis,granulocytosis
Vasoocclusion-protean Painful crises Splenic sequestration crises
Hand foot syndrome Acute chest syndrome
DIAGNOSIS?SICKLE THALASSEMIA`
CLINICAL FEATURES OF SICKLEHEMOGLOBINOPATHIESCondition Sickle
cell trait Sickle cell anemia Clinical abnorm None,rare painlss
hematuria Hb level g% normal MCV,fl normal Hb electropho HbS/A:
40/60 HbS/A:100/0 HbF;2-25%
Vasocclusive 7-10 crises,AVN,ga llstones, priapism Vasoocclusive
Crises,AVN Rare crises, AVN --do--, retinopathy 7-10
80-100
S/beta0 thalasssemia S/beta+ thalassemia HbSC
60-80
HbS/A-100/0 HbF; 1-10% HbS/A: 60/40 HbS/A;50/0 HbC;50%
10-14 10-14
70-80 80-100
CASE 26 yr old child presenting with severe pallor,jaundice
growth delay Abnormal facies,hepatosplenomegaly+ h/o recurrent
blood transfusions CBC-Hb -3gm%, MCV-58FL(Nl-86-98), -MCH- 19pg
(nl-28-33) P.S- MICROCYTIC,HYPOCHROMIA with target cells +
DIAGNOSIS?
TARGET CELLS
THALASSEMIA Other diagnosis test-Hb electrophoresis DNA analysis
for mutations Alpha thalassemia & beta thalassemia Beta
thalassemia- major - intermedia - minor
BETA THALASSEMIA
Mutn. Beta globin expression M.C- derange splicing of m-RNA
HYPOCHROMIA ,MICROCYTIC anemia
BETA THALASSEMIA MAJOR
Severe homozygous Childhood, growth delay Severe
anemia,hepatosplenomegaly,r/r transfusion Iron
overload-endo.dysfnct P.Smear- severe microcytosis,target cells Hb
electroHbF - 90-96 % HbA2- 3.5 %- 5.5% HbA - 0 %
BETA THALASSEMIA INTERMEDIASimilar stigmata like major Survive
without c/c transfusion Less severe than major Moderate
anemia,microcytosis,hypochromia Hb electrophor- HbF - 20-100% HbA2
-3.5%-5.5% HbA 0-30%
BETA THALASSEMIA MINORProfound microcytosis,target cells Minimal
anemia Similar bld picture of iron def.anemia Lab inv: MCV
