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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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The fascinating world of haemostasis and thrombosis

The Fascinating World of Haemostasis and Thrombosis

Susan Louw

Disease processes

Disease processes

  • Abnormal clotting

  • Abnormal bleeding

Bleeding disorders

Bleeding Disorders

  • Vessel wall / Vascular Disorders

  • Platelets

  • Coagulation factors

Vascular disorders

Vascular Disorders


Hereditary Haemorrhagic Telangiectasia

Connective Tissue disorders


Simple benign bruising

Senile purpura


Steroid purpura

Hereditary haemorrhagic telangiectasia

Hereditary Haemorrhagic Telangiectasia

Platelet disorders q q

Platelet Disorders – Q & Q:


Failure of production (Bone Marrow failure)

Increased destruction


Drug related





Idiopathic thrombocytopenic pupura itp

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

Idiopathic thrombocytopenic pupura itp1

Idiopathic Thrombocytopenic Pupura (ITP)

  • Clinically: Mucocutaneous bleeding

Idiopathic thrombocytopenic purpura itp

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.

Platelet disorders q q1

Platelet Disorders – Q & Q:



Glanzmann’s Thrombasthenia

Bernard-Soulier syndrome

Storage pool disease


Anti-platelet drugs


Myeloproliferative and –dysplasic disorders

Platelet disorders q q2

Platelet Disorders – Q & Q:

  • Bernard-Soulier Disease:

Bernard soulier disease

Bernard-Soulier Disease:

  • Qualitative and Quantitative abnormality of platelets

  • Large platelets

  • Congenital deficiency

    of Glycoprotein 1b

    on platelet surface

  • Reduced platelet numbers

Anti platelet drugs

Anti-Platelet DRUGS

Anti platelet drugs1

Anti-Platelet DRUGS

  • Aspirin: cyclo-oxygenase inhibitor

  • Clopidogrel: blocks ADP


  • Abxicimab: GPIIb/IIIa inhibitors

Diagnosis of platelet disorders

Diagnosis of Platelet Disorders

  • FBC and Diff

  • BMAT

  • Bleeding time

  • Platelet aggregation studies

Hereditary coagulation factor defect

HEREDITARY Coagulation Factor defect

  • Haemophilia A: defective or reduced FVIII

  • Haemophilia B: defective or reduced FIX

  • Von Willebrand’s disease: defective or reduced vWF

Hereditary coagulation factor defect1

HEREDITARY Coagulation Factor defect

  • Mode of inheritance:

    • Haemophilia A: Sex linked

    • Haemophilia B: Sex linked

    • Von Willebrand’s disease: Autosomal dominant

Hereditary coagulation factor defect2

HEREDITARY Coagulation Factor defect

  • Treatment

    • Factor replacement – BUT then there was….

Hereditary coagulation factor defect3

HEREDITARY Coagulation Factor defect

  • Treatment

    • Factor replacement

      • Viral inactivation

      • Recombinant production

    • DDAVP

    • Supportive measures

    • Gene therapy… watch this space

Thrombotic disorders

Thrombotic disorders

  • Hereditary

    • Deficiencies of natural anticoagulants

      • Antithrombin / Protein C / Proten S

  • Acquired

    • Post surgery

    • Post delivery

    • Long distance travel

    • Immobilisation

Rudolf l k virchow 1821 1902

Rudolf L.K. Virchow (1821-1902)

Virchow s triad

Virchow’s Triad

Thrombotic disorders1

Thrombotic disorders

Warfarin and its partner

Warfarin and its partner

  • Reasons for warfarin therapy

    • Many !!!!

      • Prevent clot formation

      • Prothrombotic state

        • Inherited or Acquired

      • Life saving

      • Side-effect: Bleeding

The other partner

The Other Partner

  • Heparin

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