HEMOSTASIS & BLEEDING

1. HEMOSTASIS & BLEEDING

2. Professor Anwar Sheikha MD, FRCP, FRCPath., FCAP, FRCPA, FRCPI, FACP Senior Consultant Clinical & Lab. Hematologist Clinical Professor of Hematology University of Mississippi Medical Center, Jackson, Mississippi Professor of Hematology, University of Salahaddin, Erbil, Kurdistan, IRAQ

3. HEMOSTASIS ARREST OF BLEEDING Hemostasis is a highly integrated process involving Blood Vessels, Platelets and a number of plasma proteins that are collectively responsible for Coagulation and Fibrinolysis

4. HEMOSTASIS ARREST OF BLEEDING Hemostasis is a highly integrated process involving Blood Vessels, Platelets and a number of plasma proteins that are collectively responsible for Coagulation and Fibrinolysis DEFECT IN ANY OF THESE COMPONENTS  BLEEDING TRIGGERING OF ANY OF THESE COMPONENTS  THROMBOSIS

5. HEMOSTASIS ARREST OF BLEEDING Hemostasis is a highly integrated process involving Blood Vessels, Platelets and a number of plasma proteins that are collectively responsible for Coagulation and Fibrinolysis      

6. HEMOSTASIS Hemostasis is a highly integrated process involving Blood Vessels, Platelets and a number of plasma proteins that are collectively responsible for Coagulation and Fibrinolysis

7. HEMOSTASIS CEMENT “Clotting” BRICKS “Platelets”

8. HEMOSTASIS

9. HEMOSTASIS Blood Vessel ﮔﻪﺭﻩﻻﻮﮊﻩ Clotting Platelet

10. BLOOD VESSEL Collagen Nitric Oxide Microfibrils

12. von Willebrand Factor

13. BLEEDING PURPURIC BLEEDING DEEP SEATED BLEEDING ﻛﻪﭘﺮ ﻛﯚﺷﻚ

14. BLEEDING PURPURIC BLEEDING DEEP SEATED BLEEDING BLEEDING TIME CLOTTING SCREEN ﻛﻪﭘﺮ ﻛﯚﺷﻚ

15. How to do Bleeding Time? Simplate

16. BLEEDING DUE TO VESSEL WALL ABNORMALITIES

17. HEREDITARY HEMORRHAGIC TELENGIECTASIA

18. EHLERS- DANLOS SYNDROME

19. SENILE PURPURA

20. SCURVEY

21. MULTIPLE MYELOMA

22. HENOCH-SCHONLEIN PURPURA

23. ALLOPURINOL INDUCED PURPURA

24. SLE SYSTEMIC LUPUS ERYTHEMATOSUS

25. WISKOTT-ALDRICH SYNDROME

26. PURPURA PETICHAE BRUISES MENORRHAGIA METRORRHAGIA EPISTAXIS GI BLEEDING HEMATOMA ECCHYMOSIS

27. The old practice of Bleeding Time & Clotting Time! Do Platelet count  If Low  Do Bleeding Time  If Prolonged, give Platelets

28. Do Platelet count  If Low  Do Bleeding Time  If Prolonged, give Platelets

29. BLEEDING DUE TO PLATELET ABNORMALITIES

30. Megakaryocytes • Largest hemopoietic marrow cells (~100 um) • Multi-lobulated nuclei; no mitosis but nuclear duplication • Abundant cytoplasm with azurophilic granules • Each Produces 3000 platelets SHEIKHA

31. Megakaryocytes The committed platelet progenitor cells do not undergo classical mitosis; instead they will develop nuclear duplications & cytoplasmic expansion. The rapid increase in cytoplasm is accommodated by progressive folding, or invaginations, of megakaryocytic membrane. These demarcation membranes will eventually produce individual platelet membranes. MK pseudopodia penetrates marrow sinusoids. Blood flow breaks off large platelets that are finally fragmented to individual platelets in the pulmonary microcirculation. SHEIKHA

32. Megakaryocytes EACH MK CAN PRODUCE 3000 PALETLETS In stressed thrombopoiesis, cytoplasm matures quicker than nucleus so that low ploidy MK start to produce platelets that are larger, denser and metabolically more active SHEIKHA

33. THROMBOCYTOPENIA ↓ PRODUCTION APLASTIC ANEMIA LEUKEMIAS CHEMOTHERAPY MARROW INFILTRATION

34. THROMBOCYTOPENIA ↓ PRODUCTION APLASTIC ANEMIA LEUKEMIAS CHEMOTHERAPY MARROW INFILTRATION ↓ SURVIVAL ITP EVANS’ SLE DIC TTP/HUS SEPSIS

35. THROMBOCYTOPENIA ↓ PRODUCTION APLASTIC ANEMIA LEUKEMIAS CHEMOTHERAPY MARROW INFILTRATION ↓ SURVIVAL ITP EVANS’ SLE DIC TTP/HUS SEPSIS LOSS FROM CIRCULATION SPLENOMEGALY MASSIVE TRANSFUSION

36. ITP IMMUNE THROMBOCYTOPENIC PURPURA

38. Old View: Increased Platelet Production with High Platelet Turnover New Concept: Decreased Platelet Production!!

39. Spleen in ITP

40. ITP

41. EVANS’ SYNDROME

42. MANAGEMENT OF ITP STEROID IV IMMUNOGLOBULIN Anti-D SPLENECTOMY ?Platelet Transfusion

43. Rituximab “Anti CD20”

46. WAS

47. DRUG-INDUCED THROMBOCYTOPENIA

48. TTP/HUS THROMBOTIC THROMBOCYTOPENIC PURPURA HEMOLYTIC UREMIC SYNDROME TP MAHA RENAL FEVER CNS ICU BROKEN RBCs A PHONE CALL SAVE A LIFE

49. Mortality Rate 85%

50. Mortality Rate 85% Recovery Rate 85%