Which statement regarding hereditary spherocytosis is correct?

• A It is usually inherited as autosomal recessive due to spectrin deficiency
• B Gallstones occur only after splenectomy
• C Splenectomy completely abolishes the membrane defect
• D The eosin-5-maleimide binding test shows reduced fluorescence and is more sensitive than osmotic fragility ✓

Explanation

Hereditary spherocytosis is usually autosomal dominant, involving defects in vertical membrane proteins such as ankyrin, band 3, spectrin or band 4.2. The EMA binding test measures reduced dye binding to band 3 and has higher sensitivity and specificity than the classic osmotic fragility test. Splenectomy removes the site of destruction and improves counts but does not correct the intrinsic membrane defect. Pigment gallstones result from chronic haemolysis and commonly precede any surgery.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.