The fascinating world of haemostasis and thrombosis
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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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Presentation Transcript

Disease processes
Disease processes

  • Abnormal clotting

  • Abnormal bleeding


Bleeding disorders
Bleeding Disorders

  • Vessel wall / Vascular Disorders

  • Platelets

  • Coagulation factors


Vascular disorders
Vascular Disorders

Inherited

Hereditary Haemorrhagic Telangiectasia

Connective Tissue disorders

Acquired

Simple benign bruising

Senile purpura

Scurvy

Steroid purpura



Platelet disorders q q
Platelet Disorders – Q & Q:

Quantity

Failure of production (Bone Marrow failure)

Increased destruction

ITP

Drug related

Infections

TTP

DIC

Splenomegaly


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:

Quality

Hereditary

Glanzmann’s Thrombasthenia

Bernard-Soulier syndrome

Storage pool disease

Acquired

Anti-platelet drugs

Uraemia

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 drugs1
Anti-Platelet DRUGS

  • Aspirin: cyclo-oxygenase inhibitor

  • Clopidogrel: blocks ADP

    receptors

  • 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)




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