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
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) ,
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