4a..hemolytic anemia

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4a..hemolytic anemia

  1. 1. HEMATOLOGYHemolytic AnemiaDr. Rafi Ahmed GhoriFCPSProfessor Medicine
  2. 2. "Pleasure in the job"Pleasure in the jobputsputsperfection in the work."perfection in the work."-- Aristotle-- Aristotle
  3. 3. Blood Smear - Normal
  4. 4. C.B.C / FBC / Hemogram Haemoglobin - 15±2.5, 14 ±2.5 - g/dl PCV - 0.47 ±0.07, 0.42 ±0.05 - l/l (%) Haematocrit, Total RBC volume - better RBC count - 5.5 ±1, 4.8 ± 1 x1012/l MCHC - Hb/PCV - 30-36 - g/dl Hb synthesis within RBC MCH - Hb/RBC - 29.5 ± 2.5 pg/l Average Hb in RBC MCV - PCV/RBC 85 ± 8 – fl RBC Maturation
  5. 5. RBC disorders (Anemias) :““Anemia is decreased red cell massAnemia is decreased red cell massaffecting tissue oxygenation”affecting tissue oxygenation”* Low Hb <13.5 (males), <11.5 (females)* Low Hb <13.5 (males), <11.5 (females)Acquired / Congenital disorders:Decreased production / Increased loss
  6. 6. Haemolytic An. IntroductionAnemia due to Increased RBC destructionDecreased life span (<120d)Breakdown  ↑Bilirubin (Unconj)  JaundiceIncreased RBC production - ↑ reticulocytesLow Haptoglobins – Hb carrier proteins.
  7. 7. Ketabolism of Hb:
  8. 8. Polychromasia - HemolyticAn.
  9. 9. Blood Film Features: Abnormal shape Polychromasia Nucleated RBC Plt may be low.
  10. 10. Clinical Features: Pallor mild – mucosal Jaundice - Mild fluctuating Splenomegaly No bile in urine (dark on standing-UBG) Pigment gall stones – in chronic forms Crisis – aplastic, hemolytic, vascular Ankle ulcers
  11. 11. Hemolytic Anemia - Types: Immune lysis• Warm & Cold Ab, Auto & Allo immune Mechanical Damage• Valve, Microangiopathy (DIC), prosthesis, march Hereditary Defects• Membrane, Hb & Enzyme defect Infection induced• Clostridia, malaria, septicemia
  12. 12. ClinicalFeaturesof SickleCellDisease
  13. 13. Congenital RBC Disorders:Membrane Disorders: Spherocytosis, ElliptocytosisHemoglobin Disorders: Hemoglobinopathies - Sickle cell, HbC etc. Thalassemia Syndromes - α, β, δEnzyme disorders: G6PD, PK deficiency
  14. 14. Laboratory Evaluation: Features of RBC breakdown: Hyperbilirubinemia Increased Urine UBG & Faecal stercobilinogen. Low or absent Haptoglobins Features of increased RBC Production: Reticulocytosis Marrow erythroid hyperplasia – bone changes Damaged RBC Morphology, Osmotic Fragility Decreased RBC survival – 51Cr labelling. Hemoglobin electrophoresis, enzyme abnormality
  15. 15. Laboratory Evaluation:Intravascular Haemolysis: Haemoglobinaemia, Haemoglobinuria Haemosiderinuria – Renal tubular cells Methhaemalbuminaemia – Schumm’s test.Molecular genetics Hb Electrophoresis Globin synthesis studies.
  16. 16. CBC Analyzer Report
  17. 17. Blood Smear Interpretation:A B C DE F G HI JA. NormalB. Micro/hypoC. MacroD. TargetE. SpheroF. Heinz bodyG. SchistocyteH. nRBCI. PolychromJ. Teardrop
  18. 18. Hb Electrophoresis – New born_SFABarts+CSAFpH 6.3pH 8.6
  19. 19. ““Seeing much, suffering muchSeeing much, suffering muchand studying much are theand studying much are thethree pillars of learning.”three pillars of learning.”–Benjamin Disraeli
  20. 20. MOLECULAR PATHOLOGY : Normal adult blood contain 3 types of Hb. The major component is HbA - α2ß2. The minor component fetal Hb (α2γ2) and HbA2 (α2δ2)Structure & Synthesis of Haemoglobin:Hb in adult Hb A Hb F Hb A2Structure α2ß 2α2γ2α2δ2Normal % 96-98 0.5-0.8 1.5-3.2
  21. 21. Introduction to Haemoglobins:Introduction to Haemoglobins:
  22. 22. GENE CLUSTER & HB SWITCH
  23. 23. EFFECTS OF EXCESS α CHAINS
  24. 24. Thalassemia Syndromes:Group of disorders with decreased productionof α or β chains.Features: Low Hb – depending on type. Microcytic Hypochromic RBC Unlike IDA, uniform - low RDW Target forms typical. No pencil forms. Heinz bodies – globin deposits HBH inclusions – Golf ball cells
  25. 25. Thalassemia Syndromes:Etiologically α, β, δβ thalassemia, HbH dis.Clinically classified into Hydrops fetalis(α) – IU death Thalassemia major (β) – transfusion dep Thalassemia intermedia (αβ) –spleenomegaly, Fe Thalassemia minor (αβ) - symptomless.
  26. 26. α-Thalassemia:Decreased production of α chains.Classification α0– (four gene deletion) - Hydrops fetalis α+2/3 gene deletion – Thal. Intermedia, HbH dis. α0trait, α+trait – Thal Minor, HPFHNo α thalassemia major.HbH inclusions can be demonstrated in somecases. (less in trait, more in intermedia)
  27. 27. Hydrops Fetalis:
  28. 28. α-Thalassemia:
  29. 29. Blood Smear & HbH PreparationBlood Smear & HbH Preparation
  30. 30. Thalassemia Trait:
  31. 31. Thalassemia Major:
  32. 32. ß Thalassemia Major:
  33. 33. Sickle Cell Disease:
  34. 34. G6PD Def - Heinz bodies:
  35. 35. Her. Spherocytosis:
  36. 36. Hereditary Elliptocytosis:
  37. 37. G6PD Deficiency:
  38. 38. G6PD Deficiency Anemia:
  39. 39. If you dontIf you dontstand for something,stand for something,you will fall for anything…!you will fall for anything…!
  40. 40. Hb Barts levels in Cord blood inHb Barts levels in Cord blood in αα thalassemiathalassemiaPhenotype Equivalent Noof FunctionalGenes% BartsNormal 4 0α thal trait(mild)3 0-1α thal trait(severe)2 2-8*Hb H disease 1 10-40Hb Barts Hydrops 0 ~80Higgs DR et al Blood 73, 1081, 1989* Some references mention upto 15%

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