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Approach to hemolytic anemia

Approach to hemolytic anemia. Guide : 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

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Approach to hemolytic anemia

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  1. Approach to hemolytic anemia Guide : Dr MANOJ GUPTA Candidate: Dr SARATH MENON.R HEMATOLOGY DIVISION.DEPT.MEDICINE, MGM MEDICAL COLLEGE,INDORE

  2. objectives • Lab indication of hemolysis • Intravascular v/s extravascularhemolysis • D/D of hemolytic anemia • Diagnose hemo.anemia with peripheral smear & ancillary lab tests

  3. Hemolytic Anemia • Definition: • Those anemias which result from an increase in RBC destruction coupled with increased erythropoiesis • Classification: • Congenital / Hereditary • Acquired

  4. CLASSIFICATION OF HEMOLYTIC ANEMIAS

  5. Classification Intravascular hemolysis Extravascularhemolysis • MAHA • Transfusion rx • PNH • Infections • Snake bite • Hemoglobinopathies • Enzymopathies • Membrane defects • AIHA

  6. How is Hemolytic Anemia Diagnosed? Two main principles • One is to confirm that it is hemolysis • Two is to determine the etiology

  7. How to diagnose hemolytic anemia • New onset pallor or anemia • Jaundice • Splenomegaly • Gall stones • Dark colored urine • Leg ulcers

  8. GENERAL FEATURES OF HEMOLYTIC DISORDERS • GENERAL EXAMINATION - JAUNDICE, PALLOR BOSSING OF SKULL • PHYSICAL FINDINGS - ENLARGED SPLEEN • HEMOGLOBIN - FROM NORMAL TO SEVERELY REDUCED • MCV - USUALLY INCREASED • RETICULOCYTES - INCREASED • BILIRUBIN - INCREASED[MOSTLY UNCONJUGATED] • LDH - INCREASED • HAPTOGLOBULIN - REDUCED TO ABSENT

  9. Hemolytic facies- chipmunk facies

  10. POLYCHROMATOPHILIC CELLS

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

  12. Patient History • Acute or chronic • Medication/Drug precipitants G6PD AIHA • Family history • Concomitant medical illnesses • Clinical presentation

  13. Case 1 • 3 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%

  14. Peripheral smear

  15. What is the diagnosis ? • SICKLE CELL ANEMIA

  16. DIAGNOSIS – OTHER TESTS • Hemoglobin electrophoresis -HbS >80% -HbF -1-20% -HbA2 -2- 4.5% • Sickling test POSITIVE

  17. Sickle cell disease • Mutn .beta globin-6 Glu Val. • DeoxyHbS (polymerised) • Ca influx, K leakage • stiff,viscous sickle cell • venocclusion dec.RBC survival microinfarctions,isch.painsanemia,jaundice, autoinfarct.spleengallstones,leg ulcers

  18. Clinical manifestations • Hemo.anemia,reticulocytosis,granulocytosis • Vasoocclusion-protean • Painful crises • Splenic sequestration crises • Hand foot syndrome • Acute chest syndrome

  19. Diagnosis? SICKLE THALASSEMIA`

  20. Clinical features of sickle hemoglobinopathies

  21. CASE 2 • 6 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 +

  22. Diagnosis?

  23. Target cells

  24. thalassemia • Other diagnosis test-Hb electrophoresis • DNA analysis for mutations • Alpha thalassemia & beta thalassemia • Beta thalassemia- major - intermedia - minor

  25. Beta thalassemia • Mutn. Beta globin expression • M.C- derange splicing of m-RNA • HYPOCHROMIA ,MICROCYTIC anemia

  26. 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 electro- HbF - 90-96 % HbA2- 3.5 %- 5.5% HbA - 0 %

  27. Beta thalassemiaintermedia • Similar stigmata like major • Survive without c/c transfusion • Less severe than major • Moderate anemia,microcytosis,hypochromia • Hbelectrophor- HbF - 20-100% HbA2 -3.5%-5.5% HbA – 0-30%

  28. Beta thalassemia minor • Profound microcytosis,target cells • Minimal anemia • Similar bld picture of iron def.anemia • Lab inv: MCV<75,Hct <30-33% Hbelectr: HbA2-3.5-7.5%,HbA-80-95%,HbF-1-5%

  29. Alpha thalassemias

  30. CASE 3 • 45 yr old male came to opd in a remote PHC with burning micturition • Urine R/M shows numerous pus cells++++ • UTI diagnosed & medical officer gave cotrimoxazole 2 bd X 5days • 1 wk later,pt developed severe pallor,palpitation,jaundice • Lab- increased LDH, S.BILIRUBIN,RETIC COUNT • P.S- shows irreg cells like

  31. Blister cells

  32. Heinz bodies

  33. Diagnosis? • G-6PD DEFICIENCY • INVESTIGATION- • Peripheral smear- bite cells,heinz bodies, - polychromasia G-6PD LEVEL BEUTLER FLUORESCENT SPOT TEST- Positive-if blood spot fails to flouresce in U V

  34. Clinical Features: Acute hemolysis: Drugs,infections,asso with diabetic acidosis Favism Neonatal jaundice Congenital nonspherocytic hemolytic anemia

  35. 2. PyruvateKinase Deficiency AR Deficient ATP production, Chronic hemolytic anemia Clinical features hydropsfetalis neonatal jaundice compensated hemolytic anemia Inv; P. Smear: PRICKLE CELLS ( Contracted rbc with spicules) Decreased enzyme activity

  36. Prickle cell

  37. CASE 4 • 14 YR old female present with anemia, jaundice • Rthypochondrial pain • o/e- vitals stable.pallor+,icterus+,splenomegaly + • Usg- cholilithiasis • Lab; elevated ,LDH, S.Bilirubin • Peripheral smear shows-

  38. Differential diagnosis • Hereditary spherocytosis • Autoimmune hemolytic anemia • Other diagnostic tests- osmotic fragility - coombs test

  39. Red Cell Membrane Defects 1.Hereditary Spherocytosis • Usually inherited as AD disorder • Defect: Deficiency of Beta Spectrin or Ankyrin Loss of membrane surface area becomes more spherical Destruction in Spleen

  40. C/F: Pallor Jaundice Splenomegaly Pigmented gall stones- 50%

  41. Complications • Clinical course may be complicated with Crisis: • Hemolytic Crisis: associated with infection • Aplastic crisis: associated with Parvovirus infection

  42. Inv: Test will confirm Hemolysis P Smear: Spherocytes Osmotic Fragility: Increased Screen family members

  43. Autoimmune Hemolytic Anemia • Result from RBC destruction due to RBC autoantibodies: Ig G, M, E, A • Most commonly-idiopathic • Classification • Warm AI hemolysis:Ab binds at 37degree Celsius • Cold AI Hemolysis: Ab binds at 4 degree Celsius

  44. 1.Warm AI Hemolysis: Can occurs at all age groups F > M Causes: 50% Idiopathic Rest - secondary causes: 1.Lymphoid neoplasm: CLL, Lymphoma, Myeloma 2.Solid Tumors: Lung, Colon, Kidney, Ovary, Thymoma 3.CTD: SLE,RA 4.Drugs: Alpha methyl DOPA, Penicillin , Quinine, Chloroquine 5. UC, HIV

  45. Inv: hemolysis, MCV decreased P Smear: microspherocytosis, Confirmation: Direct Coomb’s Test / Antiglobulin test

  46. 2. Cold AI HemolysisUsually Ig M directed at the RBC I antigen Infection: Mycoplasma pneumonia, Infec Mononucleosis Neoplasms : waldenstrommacroglobulinemia , lymphoma,CLL,kaposi sarcoma, myeloma. C/F: Elderly patients Exacerbations in the winter Cold , painful & often blue fingers, toes, ears, or nose ( Acrocyanosis)

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