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Evans Syndrome in SLE — RACP Adult Medicine MCQ

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ModerateHaematologyEvans Syndrome in SLERACP Adult Medicine

A 50-year-old woman with SLE presents with fatigue, Hb 78 g/L, reticulocyte count 12%, LDH elevated, haptoglobin undetectable, DAT positive (IgG + C3d). She also has thrombocytopenia (platelets 45 × 10⁹/L). What is the most likely haematological complication?

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Correct answer: AEvans syndrome (AIHA + ITP)

Concurrent warm AIHA (positive DAT, elevated LDH, low haptoglobin, reticulocytosis) and immune thrombocytopenia (low platelets) in SLE is Evans syndrome. It results from autoantibody-mediated destruction of both red blood cells and platelets. Treatment is prednisolone; rituximab or mycophenolate for refractory cases. Splenectomy is a last resort.

Reference: BSH – 2024 – AIHA/Evans Syndrome; EULAR 2024 SLE