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Thrombotic Thrombocytopenic Purpura (TTP)
Deficiency of ADAMTS 13 enzyme or vWF metalloprotease

TTP is characterized by
  1. Thrombocytopenia,
  2. Microangiopathic hemolytic anemia
  3. Fever
  4. Transient neurologic deficits
  5. Renal failure.
This is due to widespread hyaline microthrombi found in arterioles and capillaries.
Causes include
  1. Pregnancy
  2. Metastatic cancer
  3. HIV infection
  4. High dose chemotherapy
  5. Mitomycin C
  6. Antiplatelet agents like ticlopidine
    a. The presence of Coomb’s negative hemolytic anemia with fragmented RBC in peripheral smear, thrombocytopenia and minimal activation of coagulation confirm the diagnosis.
    b. Increased LDH levels is a feature.
    c. Patient presents commonly in fourth decade.
TTP is treated with
  1. Corticosteroids                          
  2. Platelet aggregation inhibitors and.
  3. Plasma exchange                        
  4. Splenectomy is performed in those who show minimal improvement.
Hemolytic Uremic Syndrome (HUS) is characterized by
  1. Microangiopathic hemolytic anaemia (MAHA): intravascular hemolysis + red cell fragmentation.
  2. ARF
  3. Thrombocytopenia
  4. Fever
  1. Endothelial damage triggers thrombosis, platelet consumption and fibrin deposition, mainly in the renal microvasculature.
  2. The strands cause mechanical destruction of passing red blood cells. Thrombocytopenia and ARF occur.
  3. Cause 90% due to E coli 0157: produces a verotoxin which attacks endothelial cells.
  4. This affects young children most, often occurring in outbreaks due to consumption of undercooked contaminated meat, with abdominal pain, bloody diarrhea and ARF.
Blood film: fragmented RBC platelets, Hb. Clotting tests are normal.

Treatment: No specific treatment, dialysis for ARF. There may be hematuria, proteinuria, which after resolved spontaneously. Dialysis for ARF may be needed. Plasma exchange is used in severe persistent disease.

Prognosis: Worse in non- E. Coli cases. Mortality 3-5%.

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