• JAMA · Feb 1977

    Case Reports

    Hereditary spherocytosis and glucose-6-phosphate dehydrogenase deficiency.

    • J Rubins and L E Young.
    • JAMA. 1977 Feb 21; 237 (8): 797798797-8.

    AbstractThe first reported case of hereditary spherocytosis (HS) and glucose-6-phosphate dehydrogenase deficiency in a black is presented. The recent literature is reviewed, with emphasis on the frequency of multiple inherited RBC defects in this ethnic group. Despite a coexisting hemoglobinopathy or enzyme deficiency, HS can be diagnosed in most cases by the peripheral blood smear, osmotic fragility curve, and family history. The implications of the double RBC abnormality are discussed, stressing the importance of splenectomy in relieving the hemolytic component due to spherocytosis.

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