Hereditary Non-spherocytic Hemolytic Anemia of the Pyruvate-kinase Deficient Type
Authors: HERBERT S. BOWMAN
Journal: Annals of Internal Medicine (1963)

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. Nextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byAnemia at the Extremes of Life: Congenital Hemolytic AnemiaEnzymatic Methods Used for DiagnosisRed cell pyruvate kinase deficiency: from genetics to clinical manifestationsSurgical aspects of splenic disease and lymphomaFailure of partial splenectomy to ameliorate the anemia of pyruvate kinase deficiencyHereditary red cell enzymopathiesCongenital haemolytic anaemia associated with adenylate kinase deficiencyPyruvate Kinase Deficiency and Other Enzymopathies of the Embden—Meyerhof PathwayMembrane Abnormalities of Pyruvate Kinase Deficient Red CellsCongenital Non-spherocytic Haemolytic Anaemia Variants with Primary and Secondary Pyruvate Kinase Deficiency.Pyruvate Kinase DeficiencyEnzymdefekte in Glykolyse und Nukleotidstoffwechsel roter Blutzellen bei nichtsph�rocyt�ren h�molytischen An�mienHereditary Disorders of the ErythrocyteHereditary Hemolytic Anemia Due to Enzyme Defects of GlycolysisHaemolysis due to Pyruvate Kinase Deficiency and Other Glycolytic EnzymopathiesSkeletal changes in the anemiasA new colorimetric method for the determination of glutathione in erythrocytesHereditäre Enzymdefekte des Erythrocyten: Expressivität und molekulare Heterogenität anomaler EnzymproteinePyruvate kinase deficiencyOn the molecular basis pyruvate kinase deficiencyPyruvate kinase in cultivated amniotic fluid cellsMechanisms of Hemolysis — The Minimal Red-Cell DefectPyruvate Kinase Deficiency Hemolytic Anemia:Splenic Macrophage Interaction with Red Cells in Pyruvate Kinase Deficiency and Hereditary SpherocytosisEnzyme Multiplicity in the Glycolytic Pathway and the Pentose—Phosphate CycleThe Metabolism and Function of Red Cells with Inherited DefectsEnzyme Multiplicity in the Glycolytic Pathway and the Pentose—Phosphate CycleDie Milz bei hämolytischen AnämienAnämien des KindesDie hämolytischen AnämienAcute Pancreatitis with Biliary Disease in Erythrocyte Pyruvate-Kinase DeficiencyA Low K m Phosphoenolpyruvate Mutant in the Amish with Red Cell Pyruvate Kinase DeficiencyErythrocytenelimination und Erythrocytenumsatz bei verschiedenen hereditären hämolytischen AnämienLiver pyruvate kinase (PK) isozymes in a PK-deficient patientThe Autohaemolysis Test: Appraisal of the Method for the Diagnosis of Pyruvate Kinase Deficiency and the Effect of pH and AdditivesLife-Span and Organ Sequestration of the Red Cells in Pyruvate Kinase DeficiencyNONSPHEROCYTIC HAEMOLYTIC ANAEMIA AND SEVERE JAUNDICE IN A NEWBORN WITH PARTIAL PYRUVATE KINASE DEFICIENCYChristmas disease, color-blindness and blood group XgaA Case of Haemolytic Anaemia Due to Pyruvate Kinase DeficiencyQualitative Befunde zur Beurteilung der ErythropoesePurification of human erythrocyte pyruvate kinasePyruvate kinase deficiency and related disorders of red cell glycolysisControl of Glycolysis in the Human Red Blood CellHereditäre nichtsphärozytäre hämolytische AnämienDie Erythrocyten-Pyruvat-Kinase bei Gesunden und bei nichtsphärocytärer, hämolytischer Pyruvat-Kinase-Mangel-AnämieThe Metabolism of Erythrocytes, and Its Relation to Hemolytic Anemias in the NewbornSplenectomyRed-Cell Enzymes and Co-Enzymes in Non-Spherocytic Congenital Haemolytic AnaemiasMedical genetics 1963Hereditary Non-Spherocytic Haemolytic Anaemia.Extreme Hemolysis and Red-Cell Distortion in Erythrocyte Pyruvate Kinase DeficiencyDie enzymopenischen ErythropathienNonspherocytes 1 April 1963Volume 58, Issue 4Page: 567-591KeywordsDehydrogenasesGenetic diseasesHematology and oncologyHemoglobinHemolytic anemiaHospital medicineInfantsPathogenesisPediatricsPyruvate ePublished: 1 December 2008 Issue Published: 1 April 1963 Copyright & PermissionsCopyright ©, 1963, by The American College of PhysiciansPDF downloadLoading ...