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August 1965

Clinical Manifestations of Primaquine-Sensitive Anemia

Author Affiliations

From the departments of pediatrics, University of Arkansas School of Medicine (Dr. Berry), and Washington University School of Medicine (Dr. Vietti).

Am J Dis Child. 1965;110(2):166-171. doi:10.1001/archpedi.1965.02090030176013

SEVERAL HEMOLYTIC anemias are characterized by deficiency of the enzyme glucose-6-phosphate dehydrogenase (G-6-PD) in the erythrocyte. Among these are the primaquinesensitive anemias found in Negroes, a group of nonspherocytic anemias found in Caucasians, and the anemia secondary to the ingestion of fava beans in sensitive individuals. Although they share a similar defect in erythrocyte metabolism, these anemias are otherwise different.1-5

When primaquine-sensitive individuals have a severe infection or are exposed to various chemicals and drugs, a hemolytic process may ensue. The mechanism of erythrocyte destruction has not been completely established but a demonstrable and major factor is the deficiency of G-6-PD in the erythrocyte which becomes critical as the cell ages.5

The purpose of this report is to emphasize the variability of clinical manifestations and the diagnostic problems encountered in 14 Negro children and their families with primaquinesensitive anemia. Some clinical patterns of expression are delineated under the

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