Try it
Pathology · Haematology: Evans syndrome
Sample itemA 30-year-old woman has jaundice and petechiae. Haemoglobin is 7 g/dL, reticulocytes are raised, platelets are 20,000 per microlitre and the smear shows spherocytes without schistocytes. The direct antiglobulin test is positive, and PT, aPTT and fibrinogen are normal. What is the diagnosis?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
D. Evans syndrome
Evans syndrome is autoimmune haemolytic anaemia together with immune thrombocytopenia, so the direct antiglobulin test is positive, spherocytes are present and coagulation tests are normal. Thrombotic microangiopathies and DIC cause schistocytes and a negative antiglobulin test, with DIC also prolonging clotting times.
Option by option
A. Haemolytic uraemic syndrome
HUS has schistocytes with renal failure and a negative DAT.
B. Disseminated intravascular coagulation
DIC prolongs PT and aPTT with low fibrinogen, which are normal here.
C. Thrombotic thrombocytopenic purpura
TTP has schistocytes and a negative DAT, caused by ADAMTS13 deficiency.
D. Evans syndrome (correct)
Correct. A positive DAT with haemolysis and thrombocytopenia, and normal coagulation, indicates Evans syndrome.
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.
More Pathology questions
- HaematopathologyBinucleate 'owl-eye' cells that stain for CD15 and CD30 are characteristic of which cond…
- NeuropathologyEosinophilic intracytoplasmic inclusions in the pigmented neurons of the substantia nigr…
- HaemodynamicsWhich of the following is NOT a component of Virchow's triad for thrombosis?
- Granulomatous diseaseNon-caseating granulomas with Schaumann bodies and asteroid bodies are typical of: