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Hereditary Non-spherocytic Hemolytic Anemia of the Pyruvate-kinase Deficient Type

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Copyright ©, 1963, by The American College of PhysiciansThe American College of Physicians

Ann Intern Med. 1963;58(4):567-591. doi:10.7326/0003-4819-58-4-567
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The non-spherocytic hemolytic anemias comprise a seemingly diverse group of diseases intrinsic to the red cell when they are contrasted with the better known congenital hemolytic anemias, such as hereditary spherocytosis. Knowledge of their inheritance, course, and pathogenesis is incomplete. Since initially defined by Dacie (1) in 1953 as "atypical congenital hemolytic anemia'' the general characteristics of this syndrome have been outlined (2, 3): [1] although hereditary, the anemia affects siblings rather than parents within a family; [2] the erythrocytes tend to be macrocytic, and spherocytes are absent; [3] the osmotic fragility of fresh native blood is not increased; [4]


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