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General Medicine · Haematology and oncology: recognition
Sample itemA 30-year-old woman has fever, confusion, renal dysfunction, thrombocytopenia and a microangiopathic haemolytic anaemia with schistocytes. PT and aPTT are normal and ADAMTS13 activity is below 10 percent. What is the diagnosis?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Thrombotic thrombocytopenic purpura
Severely deficient ADAMTS13 allows ultra-large von Willebrand multimers to form platelet microthrombi. Urgent plasma exchange is indicated. Normal coagulation tests and a low enzyme activity separate it from DIC and HUS.
Option by option
A. Immune thrombocytopenia
Immune thrombocytopenia has no haemolysis or schistocytes.
B. Thrombotic thrombocytopenic purpura (correct)
Correct. The pentad with ADAMTS13 deficiency is diagnostic.
C. Haemolytic uraemic syndrome
HUS is dominated by renal failure after diarrhoea and has normal ADAMTS13.
D. Disseminated intravascular coagulation
DIC prolongs clotting tests and consumes fibrinogen.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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