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November 24, 1989, Vol 262, No. 20 >
ARTICLE | November 24, 1989

Hereditary Spherocytosis

Peter Agre, MD
JAMA. 1989;262(20):2887-2890. doi:10.1001/jama.1989.03430200131039.
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CASE PRESENTATION  —Wafik El-Deiry, MDA 28-YEAR-OLD woman was referred for evaluation of an abnormal peripheral blood smear. She had been cared for 1 week earlier in the emergency department because of a sore throat, and was noted to have a mild anemia. The patient knew that she had undergone splenectomy at age 6 years but was unaware of any ongoing hematologic abnormality. Her medical records indicated that she had experienced neonatal jaundice requiring exchange transfusions, and at 6 years of age she had been evaluated for severe anemia. At that time, her peripheral blood smear showed erythrocyte morphology consistent with hereditary spherocytosis. This diagnosis was confirmed by osmotic fragility testing and she underwent splenectomy. She has subsequently been asymptomatic and has had two normal children born after uncomplicated pregnancies. Both of her parents were found to be clinically normal and had normal erythrocyte fragility, but one brother died of

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