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Approach to bleeding Disorders

Approach to bleeding Disorders. By Mohannad Ibn Homaid. A few points. Content Structure 1 st half 2 nd half. Overview. Why is it important ? Why is it so confusing ? Basic Science Clinical manifestations Laboratory tests . Basic Science Review. Blood is gold

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Approach to bleeding Disorders

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  1. Approach to bleeding Disorders By MohannadIbnHomaid

  2. A few points • Content • Structure • 1st half • 2nd half

  3. Overview • Why is it important ? • Why is it so confusing ? • Basic Science • Clinical manifestations • Laboratory tests

  4. Basic Science Review • Blood is gold • The 2 arms of Heamostasis • Platelets • Clotting Factors • Small Vessel Response To Injury

  5. Normal Response • Always Goes Through the following: • Vascular Phase • Platelet Phase • Coagulation Phase • Fibronlytic Phase • Pointless ? Or useful ?

  6. Vascular Phase Not Very important for understanding Vasoconstriction TXA2 and Aspirin

  7. Platelet Phase

  8. Platelet Phase • Unfortunately Very important • The following occurs: • Platelet Adhesion (vWF later) • Platelet Release Reaction • ADP • TXA2 • Temporary Plug < < BLEEDING STOPS HERE • Bleeding time • The Tile

  9. Coagulation Phase

  10. Coagulation Phase • VERY IMPORTANT and VERY CONFUSING • Why is it Confusing ? • Not Tangible • Coagulation Phase and 12 factors • Cofactors Ca and PF3 • Extrinsic vs intrinsic • Vitamin K factors • Anti-Thrombin 3 • And last but not least….

  11. THE ROMAN NUMBERS

  12. Coagulation Cascade • What do you need to know ? • Simple Steps : extrinsic vs intrinsic • Content of both • How to test them • Where they are made ( liver ) • Vitamin K • AT-3

  13. CONFUSING

  14. THIS SLIDE HAS BEEN INTENTIONALLY LEFT BLANK

  15. Extrinsic System: 7 Intrinsic System: 12-11-9-8 Final Common Pathway :10-5-2-1 Vitamin K : 2-7-9-10 AT-3 : 12 -11-10-9 PTT vs PT

  16. Coagulation Studies

  17. Fibrinolytic Phase

  18. Fibrinolytic Phase • Kinnnd of important but very easy • Tissue plasminogen Activator • Plasmin • Test • Fibrin Degradation Products • D-Dimer Assay

  19. Back to the clinical world • Presentation of platelet Defects • Blood leaks out of vessels • Skin and mucosal surfaces • Prolonged bleeding ( temporary plug plug ) • Presentation • Deep Tissue Bleeding • Late Rebleeding ( permanent plug Defect )

  20. Laboratory Test • Platlets • Count • Bleeding Time • Aggregation Test • Clotting Factors • PT and PTT • Factor Assay • Fibrinolysis • FDP • D-Dimer

  21. Platelet Disorders Quantitative vs Qualitative Thrombocytopenia Immune Thrombocytopenic Prupura Bernard Soulier Syndrome GlanzmannsThrombasthenia Thrombotic thrombocytopenic Purpura

  22. Thrombocytopenia Pathology :Increase Destruction or decrease Productions > > Clinical Features : depend on degree Labs: Treatment:

  23. ITP Pathology : Auto antibodies AgainsPlatlets Clinical Features: Labs: Treatment:

  24. Bernard Soulier Syndrome Pathology :GP1B receptor Defiency Clinical Features: Labs:

  25. GlanzmannThromboasthenia Pathology :GPIIb-IIIaDefiency Clinical Features: Labs: Treatment:

  26. TTP Pathology :Unkown Clinical Features: Pentad : HUS + Fever Neurological Labs: Treatment :Plasmapharesis

  27. Diorders of Coagulations Hemophilia Von Willebrand Disease

  28. Hemophilia • Pathology :Factor 8 or 9 • Clinical Features: • Acute Hemoarthrosis • Intracranial Bleeding • Hematomas • Labs: • Treatment: • Factor Replacement • DDAVP

  29. Von willebrand Disease • Function of vWF • Made in platelets and endothelium • Adhesion of platelets to exposed Collagen • Protection of Circulating Factor 8 • Pathology • Deficiency of vWF • Secondary decrease in Factor 8

  30. Von Willebrand Disease • Pathology : Mentioned • Clinical Features: • Labs: • Treatment: • DDAVP And factor concentrates

  31. DIC • Pathology :Inappropriate Activation of platelets and clotting Factors due to : • Sepsis ( 50%) • Obstetric Complications • Malignancy • Trauma • Clinical Features: • Labs: • Treatment: ICU and supportive = Treatment of underlying Cause

  32. Questions

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