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Hereditary Spherocytosis — RACP Paediatrics MCQ

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HardHaematology & OncologyHereditary SpherocytosisRACP Paediatrics

A 9-year-old has compensated haemolysis, splenomegaly and spherocytes. The direct antiglobulin test is negative, but iron deficiency makes osmotic-fragility testing difficult to interpret. Which test best supports hereditary spherocytosis?

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Correct answer: E — Request eosin-5-maleimide binding by flow cytometry to assess membrane-protein loss

Explanation lettering: D = shown as A · E = shown as B · A = shown as C · B = shown as D · C = shown as E

C is correct because reduced eosin-5-maleimide binding is a useful confirmatory test for hereditary spherocytosis when the film and DAT support a non-immune membrane disorder. D treats an unproven immune process. E detects haemoglobin variants, not membrane defects. A confirms haemolysis but not its cause. B bypasses confirmation, severity assessment, vaccination planning and consideration of age-related operative risk.

Reference: Royal Children’s Hospital Melbourne, Anaemia: https://www.rch.org.au/clinicalguide/guideline_index/Anaemia_Guideline/