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Published byGervais Marsh Modified over 6 years ago
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The Fascinating World of Haemostasis and Thrombosis
Susan Louw
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Disease processes Abnormal clotting Abnormal bleeding
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Bleeding Disorders Vessel wall / Vascular Disorders Platelets
Coagulation factors
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Vascular Disorders Inherited Acquired
Hereditary Haemorrhagic Telangiectasia Connective Tissue disorders Acquired Simple benign bruising Senile purpura Scurvy Steroid purpura
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Hereditary Haemorrhagic Telangiectasia
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Platelet Disorders – Q & Q:
Quantity Failure of production (Bone Marrow failure) Increased destruction ITP Drug related Infections TTP DIC Splenomegaly
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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
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Idiopathic Thrombocytopenic Pupura (ITP)
Clinically: Mucocutaneous bleeding
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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.
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Platelet Disorders – Q & Q:
Quality Hereditary Glanzmann’s Thrombasthenia Bernard-Soulier syndrome Storage pool disease Acquired Anti-platelet drugs Uraemia Myeloproliferative and –dysplasic disorders
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Platelet Disorders – Q & Q:
Bernard-Soulier Disease:
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Bernard-Soulier Disease:
Qualitative and Quantitative abnormality of platelets Large platelets Congenital deficiency of Glycoprotein 1b on platelet surface Reduced platelet numbers
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Anti-Platelet DRUGS
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Anti-Platelet DRUGS Aspirin: cyclo-oxygenase inhibitor
Clopidogrel: blocks ADP receptors Abxicimab: GPIIb/IIIa inhibitors
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Diagnosis of Platelet Disorders
FBC and Diff BMAT Bleeding time Platelet aggregation studies
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HEREDITARY Coagulation Factor defect
Haemophilia A: defective or reduced FVIII Haemophilia B: defective or reduced FIX Von Willebrand’s disease: defective or reduced vWF
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HEREDITARY Coagulation Factor defect
Mode of inheritance: Haemophilia A: Sex linked Haemophilia B: Sex linked Von Willebrand’s disease: Autosomal dominant
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HEREDITARY Coagulation Factor defect
Treatment Factor replacement – BUT then there was….
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HEREDITARY Coagulation Factor defect
Treatment Factor replacement Viral inactivation Recombinant production DDAVP Supportive measures Gene therapy… watch this space
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Thrombotic disorders Hereditary Acquired
Deficiencies of natural anticoagulants Antithrombin / Protein C / Proten S Acquired Post surgery Post delivery Long distance travel Immobilisation
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Rudolf L.K. Virchow ( )
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Virchow’s Triad
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Thrombotic disorders
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Warfarin and its partner
Reasons for warfarin therapy Many !!!! Prevent clot formation Prothrombotic state Inherited or Acquired Life saving Side-effect: Bleeding
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The Other Partner Heparin
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