HUS & TTP

HUS & TTP (Ref. Hari. 18th ed., Pg- 970)

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 Coombs 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
Pathophysiology
3. Endothelial damage triggers thrombosis, platelet
consumption and fibrin deposition, mainly in the
renal microvasculature.
4. The strands cause mechanical destruction of
passing red blood cells. Thrombocytopenia and
ARF occur.
5. Cause 90% due to E coli 0157: produces a
verotoxin which attacks endothelial cells.
6. This affects young children most, often occurring

in outbreaks due to consumption of undercooked

contaminated meat, with abdominal pain, bloody
diarrhea and ARF.
Tests:

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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Immune Thrombocytopenic
Purpura (ITP)

Hemophilia

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