1. SHEIKHA HEMOLYTIC ANEMIAS

2. SHEIKHA Abnormalities of the Environment of the Red Cells Abnormalities of the Red Cells Premature destruction of the red cells

3. HEMOLYTIC ANEMIAS SHEIKHA Abnormalities of the Red Cells

4. HEMOLYTIC ANEMIAS SHEIKHA Membrane Hb Enzymes 120 days

5. HEMOLYTIC ANEMIAS SHEIKHA Membrane Hb Enzymes HS HE H. Stomatocytosis

6. HEMOLYTIC ANEMIAS SHEIKHA Membrane Hb Enzymes HS HE H.St. Hemoglobinopathies Thalassemia Sickle

7. HEMOLYTIC ANEMIAS SHEIKHA Membrane Hb Enzymes HS HE H.St. Hemoglobinopathies Thalassemia Sickle G6PDH ↓ PK ↓ ALL CONGENITAL

8. HEMOLYTIC ANEMIAS SHEIKHA PNH Paroxysmal Nocturnal Hemoglobinuria ACQUIRED

9. HEMOLYTIC ANEMIAS SHEIKHA Abnormalities of the Environment of the Red Cells

10. HEMOLYTIC ANEMIAS due to ABNORMAL RBC ENVIRONMENT Abnormal Plasma Constituents IMMUNE: DRUGS HDN TOXINS Drugs AIHA Abnormal Physical Environment Fragmentation Hypersplenism Burn SHEIKHA WARMCHAD Infections Malaria Cl. Welchii

11. HEMOLYTIC ANEMIAS due to ABNORMAL RBC ENVIRONMENT Abnormal Plasma Constituents IMMUNE: DRUGS HDN TOXINS Drugs AIHA SHEIKHA WARMCHAD

12. HEMOLYTIC ANEMIAS due to ABNORMAL RBC ENVIRONMENT Abnormal Plasma Constituents IMMUNE: HDN Drugs AIHA SHEIKHA WARMCHAD IMMUNE HEMOLYTIC ANEMIAS

13. HEMOLYTIC ANEMIAS due to ABNORMAL RBC ENVIRONMENT Abnormal Plasma Constituents IMMUNE: DRUGS HDN TOXINS Drugs AIHA SHEIKHA WARMCHAD AIHA WARM IDIOPATHIC Associated with: SLE CLL LYMPHOMA OTHER AUOIMMUNE DISORDERS MISCELANEOUS

14. HEMOLYTIC ANEMIAS SHEIKHA Coomb’s Test

15. HEMOLYTIC ANEMIAS due to ABNORMAL RBC ENVIRONMENT Abnormal Plasma Constituents IMMUNE: DRUGS HDN TOXINS Drugs AIHA SHEIKHA WARMCHAD AIHA CHAD ACUTE CHRONIC ATYPICAL MYCO- PLASMA PNEMONIA IM IDIO- PATHIC LYMPH- OMA

16. HEMOLYTIC ANEMIAS due to ABNORMAL RBC ENVIRONMENT Abnormal Plasma Constituents IMMUNE: DRUGS HDN TOXINS Drugs AIHA SHEIKHA WARMCHAD AIHA WARMCHAD PCH ACUTE Measles Mumps Flu Idiopathic CHRONIC Idiopathic

17. Management of AIHA STEROID SPLENECTOMY RITUXIMAB CYCLOSPORIN FOLATE

18. HEMOLYTIC ANEMIAS due to ABNORMAL RBC ENVIRONMENT Abnormal Physical Environment Fragmentation Hypersplenism Burn SHEIKHA March Hb-uria Abnormal Blood Vessels MAHA Microangiopathic Hemolytic Anemia TTP/ HUS Thrombotic Thrombocytopenic Purpura / Hemolytic Uremic Syndrome Mechanical HA From damaged Valves Malignancy “Mucin Secreting Adeno- Carcinoma” Septicemia Malignant HTN

19. HEMOLYTIC ANEMIAS due to ABNORMAL RBC ENVIRONMENT SHEIKHA Infections Malaria Cl. Welchii

20. HEMOLYTIC ANEMIAS due to ABNORMAL RBC ENVIRONMENT Abnormal Plasma Constituents IMMUNE: DRUGS HDN TOXINS Drugs AIHA Abnormal Physical Environment Fragmentation Hypersplenism Burn SHEIKHA WARMCHAD Infections Malaria Cl. Welchii

21. HEMOLYTIC ANEMIAS SHEIKHA

22. HEMOLYTIC ANEMIAS LABORATORY FINDINGS: Hyperbilirubinemia “mild & unconjugated” Anemia Increased Urinary Urobilinogen Reduced Haptoglobin Reticulocytosis & Polychromasia Increased Marrow Activity RBC shape changes: Spherocytosis Fragmentation SHEIKHA

23. HEMOLYTIC ANEMIAS SHEIKHA CLINICAL FEATURES Jaundice “usually mild” Pallor Acholuric jaundice Splenomegaly Gall stones Leg ulcers Crises: Aplastic Hemolytic Sequestration

24. HEMOLYTIC ANEMIAS SHEIKHA LABORATORY FINDINGS: Hyperbilirubinemia Anemia Increased Urinary Urobilinogen Reduced Haptoglobin Reticulocytosis & Polychromasia Increased Marrow Activity RBC shape changes CLINICAL FEATURES Jaundice “usually mild” Pallor Acholuric jaundice Splenomegaly Gall stones Leg ulcers

25. HEMOLYTIC ANEMIAS SHEIKHA Intravascular Hemolysis HemoglobinemiaHemoglobinuria Hemosiderinuria

26. HEMOLYTIC ANEMIAS SHEIKHA Hemoglobinuria

27. HEMOLYTIC ANEMIAS SHEIKHA Hemosiderinuria

29. HEMOLYTIC ANEMIAS SHEIKHA

30. HEMOLYTIC ANEMIAS SHEIKHA

31. HEMOLYTIC ANEMIAS SHEIKHA

32. HEMOLYTIC ANEMIAS SHEIKHA

33. HEMOLYTIC ANEMIAS SHEIKHA

34. HEMOLYTIC ANEMIAS SHEIKHA

35. HEMOLYTIC ANEMIAS SHEIKHA

36. HEMOLYTIC ANEMIAS SHEIKHA

37. HEMOLYTIC ANEMIAS SHEIKHA Hereditary Spherocytosis

38. HEMOLYTIC ANEMIAS SHEIKHA

39. HEMOLYTIC ANEMIAS SHEIKHA

40. HEMOLYTIC ANEMIAS SHEIKHA

41. HEMOLYTIC ANEMIAS SHEIKHA

42. HEMOLYTIC ANEMIAS SHEIKHA

43. HEMOLYTIC ANEMIAS SHEIKHA

44. HEMOLYTIC ANEMIAS SHEIKHA Hereditary Elliptocytosis

45. MANAGEMENT OF HS SPLENECTOMY OPSI PNEUMOVAX Hib Folate

46. HEMOLYTIC ANEMIAS SHEIKHA

47. HEMOLYTIC ANEMIAS SHEIKHA Glucose 6 Phosphate Dehydrogenase Deficiency

48. HEMOLYTIC ANEMIAS SHEIKHA Glucose 6 Phosphate Dehydrogenase Deficiency Sex-linked 200 million Middle East Mediterranean Africa Saudis13% Kurds40% Suly Study 6% Malaria protection

49. SHEIKHA G-6-P F-6-P F 1,6 DP DHAP Ga3P 1,3 DPG 3 PG 2 PG PEP LactatePyruvate Glucose Glycolysis Embden-Meyerhof Pathway

50. SHEIKHA G-6-P F-6-P F 1,6 DP DHAP Ga3P 1,3 DPG 3 PG 2 PG PEP LactatePyruvate Glucose Glycolysis ATP ADP ATP ADP OXIDATIVE DAMAGE

51. SHEIKHA G-6-P F-6-P F 1,6 DP DHAP Ga3P 1,3 DPG 3 PG 2 PG PEP LactatePyruvate 6PGRu5P NADP NADP H * Glucose NADPNADPH * OXIDATIVE DAMAGE G6PDH PPP

52. SHEIKHA G-6-P F-6-P F 1,6 DP DHAP Ga3P 1,3 DPG 3 PG 2 PG PEP LactatePyruvate 6PGRu5P NADP NADP H * Glucose NADPNADPH * G6PDH A- Mediterranean 10% of Blacks 10% Activity

53. HEMOLYTIC ANEMIAS SHEIKHA Glucose 6 Phosphate Dehydrogenase Deficiency Favism Highly Palatable Easily Cultivated Staple Food in G6PDH areas High Protein High Nutritional Value

54. HEMOLYTIC ANEMIAS SHEIKHA Glucose 6 Phosphate Dehydrogenase Deficiency Favism Specific to Fava beans Peas & others are safe Erratic Only in 25% of G6PDH Deficient Patients Same Patient “Only Occasionally” Dosage “Children” Bean Maturity Food Processing

55. HEMOLYTIC ANEMIAS SHEIKHA Acute drug-induced hemolysis Favism Chronic hereditary non- Spherocytic Anemia Neonatal jaundice Glucose 6 Phosphate Dehydrogenase Deficiency

56. HEMOLYTIC ANEMIAS SHEIKHA

57. HEMOLYTIC ANEMIAS SHEIKHA

58. G-6-P F-6-P F 1,6 DP DHAP Ga3P 1,3 DPG 3 PG 2 PG PEP LactatePyruvate Glucose 2,3 DPG PK PK Deficiency

59. SHEIKHA Drugs and Chemicals Associated with Hemolysis in G6PDH-Deficient Subjects DrugsDefinite AssociationPossible AssociationDoubtful Association AntimalarialsPrimaquineChloroquineQuinacrine Pamaquine--Quinine SulfonamidesSulfanilamideSulfadimidineSulfoxone SulfacetamideSulfasalazineSulfadiazine SulfapyridineGlyburideSulfisoxalone Sulfamethoxazole-- SulfonesDapsone-- NitrofuransNitrofurantoin-- Antipyretic/analgesicAcetanilidAspirinAcetaminophen -- Phenacetin

60. SHEIKHA DrugsDefinite Association Possible Association Doubtful Association Other drugsNalidixic acidCiprofloxacinPAS NiridazoleChloramphenicolDoxorubicin Methylene blueVitamin K analogsProbenecid PhenazopyridineAscorbic acidDimercaprol SeptrinPAS-- Other chemicalsNaphthaleneAcalypha indica-- Trinitrotoluene-- Drugs and Chemicals Associated with Hemolysis in G6PDH-Deficient Subjects

61. HEMOLYTIC ANEMIAS SHEIKHA

62. HEMOLYTIC ANEMIAS SHEIKHA

63. HEMOLYTIC ANEMIAS SHEIKHA

64. HEMOLYTIC ANEMIAS SHEIKHA

65. HEMOLYTIC ANEMIAS SHEIKHA

66. HEMOLYTIC ANEMIAS SHEIKHA

67. HEMOLYTIC ANEMIAS SHEIKHA

68. HEMOLYTIC ANEMIAS SHEIKHA

69. HEMOLYTIC ANEMIAS SHEIKHA

70. HEMOLYTIC ANEMIAS SHEIKHA

71. HEMOLYTIC ANEMIAS SHEIKHA