Thrombotic Thrombocytopenic Purpura (TTP) (HUS) PDF

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 – adolescent
  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