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Hereditary Nonspherocytic Hemolytic Anemia with Pyruvate Kinase Deficiency.

K. R. Tanaka, M.D. (Associate); W. N. Valentine, M.D., F.A.C.P.; and A. S. Schneider, M.D.
Ann Intern Med. 1963;58(4):732. doi:10.7326/0003-4819-58-4-732_1
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In recent years, biochemical studies have enabled a better delineation of the hereditary nonspherocytic hemolytic anemias. Nine patients (5 males and 4 females, aged 1 to 39 years), corresponding in all major respects to those classified as Type II by Selwyn and Dacie, have been studied. These patients have a Coombs-negative, nonspherocytic, hemolytic anemia of variable degree. Their red cells exhibit increased autohemolysis, not correctible (or only partially correctible) by administration of glucose, but correctible by administration of adenosine triphosphate (ATP). The age of apparent onset, clinical severity, and transfusion requirements vary considerably. Splenectomy in 4 patients has not


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