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Bleeding and

Bleeding and. Thrombotic Disorders. Kristine Krafts, M.D. Bleeding and Thrombotic Disorders. Bleeding disorders von Willebrand disease Hemophilia A and B DIC TTP/HUS ITP Thrombotic disorders Factor V Leiden. Platelet bleeding Superficial (skin) Petechiae Spontaneous.

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Bleeding and

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  1. Bleeding and Thrombotic Disorders Kristine Krafts, M.D.

  2. Bleeding and Thrombotic Disorders Bleeding disorders • von Willebrand disease • Hemophilia A and B • DIC • TTP/HUS • ITP Thrombotic disorders • Factor V Leiden

  3. Platelet bleeding • Superficial (skin) • Petechiae • Spontaneous • Factor bleeding • Deep (joints) • Big bleeds • Trauma * * Includes prolonged bleeding after dental work

  4. Petechiae

  5. Palatal petechiae

  6. Palatal ecchymosis

  7. Purpura

  8. Bleeding after buttock injection in patient with hemophilia

  9. Bleeding and Thrombotic Disorders Bleeding disorders • von Willebrand disease

  10. Von Willebrand Disease Things you must know • Most common hereditary bleeding disorder • Autosomal dominant • vW factor decreased (or abnormal) • Variable severity

  11. What’s von Willebrand Factor? • Huge multimeric protein • Made by megs and endothelial cells • Glues platelets to endothelium • Carries factor VIII • Decreased or abnormal in vW disease

  12. Extrinsic Intrinsic TF VII IX VIII X V thrombin fibrin clot

  13. Symptoms of Von Willebrand Disease • Mucosal bleeding in most patients • Deep joint bleeding in severe cases

  14. Lab Tests in Von Willebrand Disease • Bleeding time: prolonged • PTT: prolonged (“corrects” with mixing study) • PT: normal

  15. Treatment of Von Willebrand Disease • DDAVP (raises VIII and vWF levels) • Cryoprecipitate (contains vWF and VIII) • Factor VIII

  16. SINtrinsic Intrinsic Extrinsic thrombin exposed TF XI XIa TF VIIa IX IXa VII VIIIa VIII X Xa Va V prothrombin thrombin busy clot fibrinogen fibrin distracting sinful

  17. Bleeding and Thrombotic Disorders Bleeding disorders • von Willebrand disease • Hemophilia A and B

  18. Hemophilia A • Most common factor deficiency • X-linked recessive in most cases (30% are spontaneous mutations) • Factor VIII level decreased • Variable amount of “factor” bleeding Things you must know

  19. Extrinsic Intrinsic TF VII IX VIII X V thrombin fibrin clot

  20. Deep joint bleeding in patient with hemophilia

  21. Normal knee Knee of patient with hemophilia Hemophilic arthropathy of knee

  22. Joint Deformity in Hemophilia

  23. Hemophilia A Lab tests • PTT prolonged • Factor VIII level low • DNA studies abnormal Treatment • DDAVP • Factor VIII

  24. Hemophilia B • Factor IX level decreased • Much less common than hemophilia A • Same inheritance pattern • Same clinical and laboratory findings Things you must know

  25. Extrinsic Intrinsic TF VII IX VIII X V thrombin fibrin clot

  26. Bleeding and Thrombotic Disorders Bleeding disorders • von Willebrand disease • Hemophilia A and B • DIC

  27. Thrombosis Hemorrhage

  28. Remember these for sure: • Malignancy • OB complications • Sepsis • Trauma

  29. Bleeding and Thrombotic Disorders Bleeding disorders • von Willebrand disease • Hemophilia A and B • DIC • TTP/HUS

  30. Thrombotic Thrombocytopenic Purpura Things you must know • Pentad: MAHA, thrombocytopenia, fever, neurologic defects, renal failure • Deficiency of ADAMTS13 • Big vWF multimers trap platelets • Plasmapheresis or plasma infusions

  31. Nasty creatures Rodent of unusual size (ROUS) -The Princess Bride, 1987 Von Willebrand multimer of unusual size (MOUS) - NEJM, 1982

  32. Thrombotic Thrombocytopenic Purpura Clinical pentad • Hematuria/jaundice (MAHA) • Bleeding/bruising (thrombocytopenia) • Fever • Bizarre behavior (thrombi in CNS) • Renal failure (thrombi in kidney) Treatment • Plasmapheresis (in acquired TTP) • Plasma infusions (in hereditary TTP)

  33. Hemolytic Uremic Syndrome Things you must know • MAHA and thrombocytopenia • Most are related to E. coli infection • Toxin damages endothelium • Treat supportively

  34. Bleeding and Thrombotic Disorders Bleeding disorders • von Willebrand disease • Hemophilia A and B • DIC • TTP/HUS • ITP

  35. Idiopathic Thrombocytopenic Purpura Things you must know • Antiplatelet antibodies coat platelets • Splenic macrophages eat platelets • Diagnosis of exclusion • Steroids or splenectomy

  36. Bruising after minor trauma in ITP

  37. Bleeding and Thrombotic Disorders Bleeding disorders • von Willebrand disease • Hemophilia A and B • DIC • TTP/HUS • ITP Thrombotic disorders • Factor V Leiden

  38. Blood clot sequelae

  39. Deep venous thrombosis

  40. Deep venous thrombosis

  41. Pulmonary embolus

  42. Thrombosis Risk Factors Endothelial damage • Atherosclerosis Stasis • Immobilization • Varicose veins • Cardiac dysfunction Hypercoagulability • Surgery • Carcinoma • Estrogen/postpartum • Thrombotic disoders

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