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

Article1 April 1963Hereditary Non-spherocytic Hemolytic Anemia of the Pyruvate-kinase Deficient TypeHERBERT S. BOWMAN, M.D., F.A.C.P., FRANK PROCOPIO, M.D.HERBERT S. BOWMAN, M.D., F.A.C.P.Search for more papers by this author, FRANK PROCOPIO, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-58-4-567 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThe 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]...References1. DACIEMOLLISONRICHARDSONSELWYNSHAPRIO JVPLNJGL: Atypical congenital hemolytic anemia. Quart. J. Med. 22: 79, 1953. MedlineGoogle Scholar2. DACIE JV: The Haemolytic Anemias, Congenital and Acquired. Part 1. The Congenital Anemias, 2d ed., J. & A. Churchill Ltd., London, 1960, p. 171. Google Scholar3. DE GRUCHYSANTAMARIAPARSONSCRAWFORD GCJNICH: Nonspherocytic congenital hemolytic anemia. Blood 16: 1371, 1960. CrossrefMedlineGoogle Scholar4. SELWYNDACIE JGJV: Autohemolysis and other changes resulting from the incubation in vitro of red cells from patients with congenital hemolytic anemia. 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Bowman, M.D., Division of Hematology, Laboratory of Pathology, Harrisburg Hospital, Harrisburg, Pennsylvania. 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