BLEEDING DISORDERS

1. BLEEDING DISORDERS AN OVERVIEW WITH EMPHASIS ON EMERGENCIES

2. CLASSIFICATION • PLATELET DISORDERS AND VASCULAR COMPONENTS • COAGULATION ABNORMALITIES • OTHER

3. VASCULAR AND PLATELET ABNORMALITIES • Vascular - hereditary • Hereditary hemorrhagic telangiectasia • Ehlers-Danlos syndrome • - acquired • “senile purpura” • Henoch-Schonlein syndrome • Scurvy - Amyloid • Steroid purpura

6. Acquired Coagulation Disorders • Vitamin K deficiency • Warfarin therapy • Liver disease • DIC • Antibodies against factor VIII (or other factors) • Massive transfusion

7. Thrombocytopenia • False reading • Clumping of platelets • Platelet satelitism • Margination in congestive splenomegaly • True thrombocytopenia • Failure to produce platelets • Acquired - drugs - alcohol • Inherited – TAR, May-Hegglin. Wiscott-Aldrich • Platelet destruction • Immune • TTP • DIC

8. Disorders of platelet function • Inherited • Von Willebrand’s disease • Glanzmann’s • Bernard-Soulier • Storage pool disease – gray platelets • Acquired • Drugs • MDS • Multiple myeloma – hyperglobulinemia • Uremia

9. COAGULATION DISORDERS • Hemophilias A & B • Others • Von Willebrand’s disease

10. Inherited Coagulation Disorders • Factor VIII deficiency may be severe <1%, moderate 1-5%, or mild 5-20% activity • After repeated doses of Factor VIII patients may develop and antibody against the factor • Treatment then requires bypassing the factor to effect hemostasis with FEIBA or Factor VIIa • Rarely the low concentration of antibody can be taken up by larger amounts of factor VIII

11. Other inherited factor deficiencies • Factor XI – mostly in Ashkenazi Jews • Bleeding is usually mild except after surgery or significant injury • XI concentrate may be available or give plasma • Factor XIII deficiency is rare and is similar to XI deficiency in clinical presentation • Factor VII and X deficiencies are seen and are variable in severity from case to case

12. True Emergencies • Hemophilia A or B with bleeding • TTP • DIC • ITP with bleeding • Factor inhibitor with bleeding

13. True Emergencies • Remember that bleeding can occur because of injury with no bleeding diathesis! • Hemophilia A or B with bleeding • TTP • DIC • ITP with bleeding • Factor inhibitor with bleeding

14. Management of the Emergency • Be sure you have a proper diagnosis. • Remember the things you must not do, if there are any. • Be sure that the institution has the capacity to deal with the problem. • Available medications • Available procedures and equipment

15. Injured or Postoperative Patients • With no history of bleeding in the past (or no history at all) • Do the diagnostic things – PT/PTT, Plt and PFT • If above are normal think of an open vessel.

16. Hemophilia • Diagnosis is based on the Factor level and the history • Replacement of factor is the main thing • Remember antibodies

17. Thrombotic Thrombocytopenic Purpura • Diagnosis is made by finding 3 of 5 features. • Microangiopathic hemolytic anemia • Thrombocytopenic purpura • Fever • Neurologic abnormalities • Renal failure

18. TTP • Things to avoid. • Don’t waste time. This is a serious, rapidly progressive and fatal illness. • Transfusion of blood cells, especially platelets, can accelerate the disease.

19. TTP Treatment • Plasma infusion in large amounts. This almost always means plasmapheresis, using plasma to replace volume for volume. • Other treatment with pheresis includes: • ASA and persantine • Prednisone

20. DIC • Diagnsis is ever so important

21. TTP – Warning! • Do not try to take care of TTP in a hospital that doesn’t have pheresis and a blood bank able to handle the demands. • Immediate transfer to a hospital that has these capacities is in order. • Before transfer, you might consider giving 2 – 3 units of FFP