Abstract
Ten year old, black female originally diagnosed as having SC disease had multiple thrombotic crises resembling SS disease. Because of her unusually severe clinical course, Hb electrophoresis on citrate agar and finger printing was done and SO Arab disease was diagnosed. The clinical presentation of Hb SO Arab in this patient resembled that of Hb SD disease. Her hemoglobin varied from 9.9 to 10.5 gm/dl and reticulocyte count from 1.2 to 2.5%. The peripheral blood smear showed moderate anisopoikilocytosis, moderate polychromasia and occasional sickle forms. The RBC survival study revealed a half life of 19 days (normal 22-28 days). Hb CO Arab was found in two siblings who are asymptomatic. Numerous target cells and mild anisocytosis was seen on the peripheral blood smear. RBC survival study in one of the siblings showed a half life of 16 days with sequestration in the spleen. Oxygen dissociation curve in the patient with SO Arab showed marked decrease in oxygen affinity of P50 66.5 mm/Hg resembling the pattern seen in sickle cell anemia. In the siblings normal or slightly increased P50 of 35.4 mm/Hg was seen. This is the first report of decreased RBC survival with RBC sequestration in the spleen in CO Arab disease. We wish to emphasize the importance of citrate agar electrophoresis and finger printing for the diagnosis of SO Arab disease.
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Kini, K., Warrier, R., Maeda, K. et al. 825 HEMATOLOGICAL STUDIES IN HEMOGLOBIN SO ARAB AND CO ARAB DISEASES: CLINICAL AND LABORATORY EVALUATION. Pediatr Res 15 (Suppl 4), 580 (1981). https://doi.org/10.1203/00006450-198104001-00850
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DOI: https://doi.org/10.1203/00006450-198104001-00850