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Thrombotic Thrombocytopenic Purpura (TTP) (HUS)


Thrombotic Thrombocytopenic Purpura (TTP) (HUS)

  • Aetiology :  Autoimmune disorder, Abs. against ADAMTS enzyms .
    (This enz. normally clears VWF multimers to produce normal functional units and
    its deficiency results in large VWF multimers which cross – link platelets and leads
    to microvascular occlusion by plat. Thrombi e.g. brain, kidney).
    Idiopathic, associated with drugs (Ticlopidine, Ciclosporine), HIV, Malignancy.
  • Clinically : Sporadic, Fluctuating, Familial: reported, F > M , young – adolescentthrombotic thrombocytopenic purpura
    1. Fever
    2. Anemia : Microangiopathic haemolytic (Fragmented RBCs) ,
    ↑ Retculocyte count, Jaundice, ↑ LDH, ‐ve coomb’s test.
    3. Thrombocytopenia – Purpura, count usually < 20 X 109 /L
    4. Neurological: Convulsion, Coma, Paralysis ( Transient or prolonged) ,
    Psychological .
    5. Renal : intermittent proteinuria, Renal Failure (more in HUS)
  • Lab. ↑ WBC, ↓ plat.
    PT, PTT ‐‐‐‐‐‐‐ Normal
    B.M. ‐‐‐‐‐‐‐ Normal or increase cellularity
    Gingival Biosy – Helpful
  • DD : ITP


  • Prognosis :
  • 10% Recurrent.
  • Untreated – 90% Mortality in 10 days
  • Treated – Mortality 20‐30% at 6 months
  • Sudden death due to cardiac Microthrombi
  • Treatment :
  • Plasmapharesis with FFP 12 bags/day for weeks – Months
  • Corticosteroids, Rituximab have a role.
  • Pregnant – May needs termination if no response

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