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Nonspherocytic Hemolytic Anemia in a Caucasian Adult Associated With Neurologic Abnormalities

MIGUEL A. ESCOBAR, MD; PAUL HELLER, MD; FRANK E. TROBAUGH, JR., MD

Arch Intern Med. 1964;113(3):428-434.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

Erythrocyte glucose-6-phosphate dehydrogenase (G-6-PD) deficiency, usually designated as "primaquine sensitivity," is a common genetic disorder of wide geographic and racial distribution.^1,2 It occurs in 12% to 15% of male Negroes and also in certain Caucasian population groups, eg, Sephardic Jews, Sardinians, Iranians. Enzyme activity is usually not completely absent, and, therefore, persons with this abnormality are ordinarily not anemic unless hemolysis is provoked by the exposure to certain toxic and infectious agents.^1,2

Complete absence of G-6-PD activity in the red cell is very rare. It has been described in children,^3-5 usually of Northern European extraction, with nonspherocytic hemolytic anemia. There have been very few references ^4,6 to the existence of this variant of G-6-PD deficiency in the adult. The hemolytic process due to this disorder may not become clinically manifest until adult life, and, therefore, its occurrence in the older age groups needs to be emphasized.

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Author Affiliations
CHICAGO

Formerly Research Fellow, Section of Hematology, Department of Medicine, Presbyterian-St. Luke's Hospital (Dr. Escobar); Associate Chief of Staff for Research and Hematologist, Veterans Administration West Side Hospital, Professor of Medicine, University of Illinois College of Medicine (Dr. Heller); Director, Section of Hematology, Department of Medicine, Presbyterian-St. Luke's Hospital, Associate Professor of Medicine, University of Illinois College of Medicine (Dr. Trobaugh).

From the departments of medicine, Presbyterian-St. Luke's Hospital, Veterans Administration West Side Hospital, and the University of Illinois College of Medicine.

Footnotes
Received for publication May 27, 1963; accepted June 12.

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