Knowledge Check

Drag and drop the cards with clinical parameters on the top to the appropriate bucket (TTP pentad or not) on the bottom.

MAHA
Schistocytes on blood smear
Fever
Skin rash
ADAMTS13 < 10%
Thrombocytopenia
Low vWF levels
Neurological findings
Renal dysfunction
Part of pentad
Not part of pentad

MAHA, macroangiopathic hemolytic anemia

The pentad is present in only 5% of patients with thrombotic thrombocytopenic purpura.

True of false: the direct antiglobulin test (DAT, also called Coombs test) is typically positive in patients with thrombotic thrombocytopenic purpura (TTP).

a
True
b
False

True of false: congenital thrombotic thrombocytopenic purpura (TTP) is more common than the acquired form.

a
True
b
False

True of false: red cell transfusion is relatively contraindicated in patients with thrombotic thrombocytopenic purpura (TTP).

a
True
b
False
Platelet transfusions are relatively contraindicated in patients with TTP, not red cell transfusions.

Which of the following should be started in all patients with a first episode of suspected thrombotic thrombocytopenic purpura (TTP):

a
Corticosteroids
b
Plasma transfusion/infusion
Used only if plasma exchange is delayed or in cases of congenital TTP.
c
Plasma exchange
Correct. All patients should receive prompt therapy with plasma exchange.
d
Rituximab
Only in some patients with TTP.
e
Recombinant ADAMTS13
Not available/approved yet.
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