The Fascinating World of Haemostasis and Thrombosis

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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
• 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
• Glanzmanns 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 Willebrands 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 Willebrands 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
• 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 (1821-1902)
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Virchows 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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