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SLE — SCE Rheumatology MCQ

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ModerateSLE & Antiphospholipid SyndromeSLESCE Rheumatology

A 48-year-old woman with established systemic lupus erythematosus develops fatigue, jaundice and a falling haemoglobin concentration. Her reticulocyte count, unconjugated bilirubin and lactate dehydrogenase are elevated, and haptoglobin is undetectable. A blood film shows polychromasia and spherocytes but no schistocytes. The direct antiglobulin test is positive for IgG and negative for C3d. What type of haemolytic anaemia is present?

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Correct answer: C — Warm autoimmune haemolytic anaemia

This is warm autoimmune haemolytic anaemia. Reticulocytosis, raised unconjugated bilirubin and LDH, and undetectable haptoglobin establish active haemolysis. An IgG-positive direct antiglobulin test identifies IgG-coated erythrocytes, characteristic of warm AIHA, which is a recognised haematological manifestation of SLE. Spherocytes result from partial phagocytosis of antibody-coated red cells. Microangiopathic haemolysis would usually produce schistocytes and is generally direct-antiglobulin-test negative. Hereditary spherocytosis can also cause spherocytes but is non-immune and therefore has a negative direct antiglobulin test. G6PD deficiency typically causes oxidant-associated haemolysis with bite cells or blister cells. Paroxysmal nocturnal haemoglobinuria is a complement-mediated clonal disorder diagnosed by flow cytometric demonstration of deficient GPI-linked proteins, not an IgG-positive direct antiglobulin test.

Reference: Barcellini W, Fattizzo B. Autoimmune haemolytic anaemias. Nature Reviews Disease Primers. 2024; diagnostic classification and direct antiglobulin testing. https://pubmed.ncbi.nlm.nih.gov/39487134/