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4 out of 6

All of the following feature may be seen in thrombotic thrombocytopenic purpura, except: (AIIMS May 2010)

A Fever
B Hemolysis

C Hypertension
D Low platelets count

Ans. C


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

Extra Edge.

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. 3. Platelet aggregation inhibitors and.

3. Plasma exchange

4. 4. Splenectomy is performed in those who show minimal improvement.