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
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
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