Knowledge Check

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

Fever

MAHA

Thrombocytopenia

Neurological findings

Low vWF levels

ADAMTS13 < 10%

Schistocytes on blood smear

Skin rash

Renal dysfunction

Part of pentad

Not part of pentad

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MAHA, macroangiopathic hemolytic anemia

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

Which two components of the pentad are now considered acceptable to make a diagnosis of TTP?

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1. MAHA
2. Thrombocytopenia

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

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

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

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