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    Subjects/Pathology/Hemolytic Anemias
    Hemolytic Anemias
    medium
    microscope Pathology

    Which feature best distinguishes hereditary spherocytosis from hereditary elliptocytosis?

    A. Osmotic fragility test shows increased fragility in both conditions
    B. Both present with neonatal jaundice and splenomegaly
    C. Membrane protein defect involves spectrin in both conditions equally
    D. Spherocytes on blood smear with positive osmotic fragility; elliptocytes with normal osmotic fragility

    Explanation

    Distinguishing Hereditary Spherocytosis from Hereditary Elliptocytosis

    Key Morphological and Functional Differences
    Table
    FeatureHereditary SpherocytosisHereditary Elliptocytosis
    RBC ShapeSpherical, denseElliptical / rod-shaped
    Osmotic FragilityIncreased (fragile in hypotonic saline)Normal
    Incubated Osmotic FragilityMarkedly increasedNormal
    Primary DefectSpectrin, ankyrin, band 3, protein 4.2Spectrin, protein 4.1, glycophorins
    Hemolysis SeverityModerate to severeMild (often asymptomatic)
    Splenectomy BenefitExcellent responseMinimal benefit
    Key Point

    Osmotic fragility testing is the gold standard discriminator. Hereditary spherocytosis RBCs lyse at higher saline concentrations (increased fragility) because their spherical shape with reduced surface-area-to-volume ratio cannot accommodate water influx. Hereditary elliptocytes remain intact in the same conditions due to their elongated shape and normal osmotic tolerance.

    High-YieldNEET PG

    Incubated osmotic fragility (RBCs incubated at 37°C for 24 hours before testing) is even more discriminatory — spherocytes show marked increase in fragility, while elliptocytes remain normal. This test is rarely done in routine practice but is a classic NEET PG question stem.

    Clinical Pearl

    Hereditary elliptocytosis patients are often asymptomatic and discovered incidentally on blood smear. Hereditary spherocytosis typically presents with jaundice, anemia, and splenomegaly requiring intervention.

    Robbins 10e Ch 13

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