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The Fascinating World of Haemostasis and Thrombosis

The Fascinating World of Haemostasis and Thrombosis. Susan Louw. Disease processes. Abnormal clotting Abnormal bleeding. Bleeding Disorders. Vessel wall / Vascular Disorders Platelets Coagulation factors. Vascular Disorders. Inherited Hereditary Haemorrhagic Telangiectasia

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The Fascinating World of Haemostasis and Thrombosis

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  1. The Fascinating World of Haemostasis and Thrombosis Susan Louw

  2. Disease processes • Abnormal clotting • Abnormal bleeding

  3. Bleeding Disorders • Vessel wall / Vascular Disorders • Platelets • Coagulation factors

  4. Vascular Disorders Inherited Hereditary Haemorrhagic Telangiectasia Connective Tissue disorders Acquired Simple benign bruising Senile purpura Scurvy Steroid purpura

  5. Hereditary Haemorrhagic Telangiectasia

  6. Platelet Disorders – Q & Q: Quantity Failure of production (Bone Marrow failure) Increased destruction ITP Drug related Infections TTP DIC Splenomegaly

  7. Idiopathic Thrombocytopenic Pupura (ITP) • Chronic (women 15-50yrs) and Acute (children) • Idiopathic or secondary (HIV!, SLE, viral infections etc.) • Platelet sensitisation with auto-antibodies with premature removal by spleen

  8. Idiopathic Thrombocytopenic Pupura (ITP) • Clinically: Mucocutaneous bleeding

  9. Idiopathic Thrombocytopenic Purpura (ITP) • Diagnosis • Plt count 5 – 50 x109 /L on FBC with increased megakaryocytes on bone marrow biopsy • Treatment • Corticosteroids • Splenectomy etc. etc.

  10. Platelet Disorders – Q & Q: Quality Hereditary Glanzmann’s Thrombasthenia Bernard-Soulier syndrome Storage pool disease Acquired Anti-platelet drugs Uraemia Myeloproliferative and –dysplasic disorders

  11. Platelet Disorders – Q & Q: • Bernard-Soulier Disease:

  12. Bernard-Soulier Disease: • Qualitative and Quantitative abnormality of platelets • Large platelets • Congenital deficiency of Glycoprotein 1b on platelet surface • Reduced platelet numbers

  13. Anti-Platelet DRUGS

  14. Anti-Platelet DRUGS • Aspirin: cyclo-oxygenase inhibitor • Clopidogrel: blocks ADP receptors • Abxicimab: GPIIb/IIIa inhibitors

  15. Diagnosis of Platelet Disorders • FBC and Diff • BMAT • Bleeding time • Platelet aggregation studies

  16. HEREDITARY Coagulation Factor defect • Haemophilia A: defective or reduced FVIII • Haemophilia B: defective or reduced FIX • Von Willebrand’s disease: defective or reduced vWF

  17. HEREDITARY Coagulation Factor defect • Mode of inheritance: • Haemophilia A: Sex linked • Haemophilia B: Sex linked • Von Willebrand’s disease: Autosomal dominant

  18. HEREDITARY Coagulation Factor defect • Treatment • Factor replacement – BUT then there was….

  19. HEREDITARY Coagulation Factor defect • Treatment • Factor replacement • Viral inactivation • Recombinant production • DDAVP • Supportive measures • Gene therapy… watch this space

  20. Thrombotic disorders • Hereditary • Deficiencies of natural anticoagulants • Antithrombin / Protein C / Proten S • Acquired • Post surgery • Post delivery • Long distance travel • Immobilisation

  21. Rudolf L.K. Virchow (1821-1902)

  22. Virchow’s Triad

  23. Thrombotic disorders

  24. Warfarin and its partner • Reasons for warfarin therapy • Many !!!! • Prevent clot formation • Prothrombotic state • Inherited or Acquired • Life saving • Side-effect: Bleeding

  25. The Other Partner • Heparin

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