Home / Hematology / Thrombotic Thrombocytopenic Purpura (TTP) (HUS)

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 – 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

Download ebook Thrombotic Thrombocytopenic Purpura (TTP) (HUS) GET IT 10$

Leave a Reply

Your email address will not be published. Required fields are marked *

%d bloggers like this: