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Hereditary spherocytosis membrane defect — MRCPCH TAS MCQ

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HardPathologyHereditary spherocytosis membrane defectMRCPCH TAS

A 5-year-old has jaundice, splenomegaly and anaemia. Blood film shows spherocytes; direct antiglobulin test is negative and mean corpuscular haemoglobin concentration is high. What is the most likely pathology?

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Correct answer: E — Red cell membrane cytoskeleton defect

Hereditary spherocytosis is due to red cell membrane cytoskeletal defects such as spectrin or ankyrin abnormalities, causing spherocytes and extravascular haemolysis. Autoimmune haemolysis can show spherocytes but usually has a positive direct antiglobulin test. The pearl is that raised MCHC is a helpful clue to hereditary spherocytosis.

Reference: Nelson Textbook of Pediatrics; RCPCH Theory examination syllabi; Lissauer's Illustrated Textbook of Paediatrics