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

Thrombotic Thrombocytopenic Purpura (TTP)Deficiency of ADAMTS 13 enzyme or vWFmetalloproteaseTTP 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 inarterioles 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

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hemolytic anemia with fragmented RBCin peripheral smear, thrombocytopeniaand minimal activation of coagulationconfirm the diagnosis.

b. Increased LDH levels is a feature.

c. Patient presents commonly in fourthdecade.

TTP is treated with

1. Corticosteroids

2. Platelet aggregation inhibitors and.

3. Plasma exchange

4. Splenectomy is performed in those who showminimal 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, plateletconsumption and fibrin deposition, mainly in therenal microvasculature.

4. The strands cause mechanical destruction ofpassing red blood cells. Thrombocytopenia andARF occur.

5. Cause 90% due to E coli 0157: produces averotoxin which attacks endothelial cells.

6. This affects young children most, often occurring

Comment

in outbreaks due to consumption of undercookedcontaminated meat, with abdominal pain, bloodydiarrhea and ARF.

Tests:

Blood film: fragmented RBC platelets, Hb. Clotting testsare normal.

Treatment: No specific treatment, dialysis for ARF. Theremay be hematuria, proteinuria, which after resolvedspontaneously. Dialysis for ARF may be needed. Plasmaexchange is used in severe persistent disease.

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

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