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• W2042945301 abstract "The case histories and investigations are presented of four patients with paroxysmal nocturnal hemoglobinuria who satisfy the diagnostic criteria of abnormal erythrocyte acid hemolysis, positive intravascular hemolysis and low erythrocyte acetylcholinesterase. The clinical presentations of these patients ranged from a young man with a classic history, through a woman with macroglobulinemia and a Coombs' positive hemolytic anemia, and a man with apparent drug-induced hypoplastic anemia, to an elderly woman with a mild but refractory anemia. This clinical variation was also supported by the laboratory investigations performed. Ferrokinetic studies revealed increased plasma iron turnover in all four patients; in two it had reached the theoretical maximum of which the marrow was capable. Erythrocyte incorporation of iron showed a frankly hemolytic picture in two patients, a hypofunctional marrow pattern in one, and one was normal. Significant splenic sequestration was demonstrated in two patients, and this was confirmed with chromium51 studies in one of them. Reasons for this apparent difference are put forward, as well as further evidence obtained for the dual erythrocyte population theory. The management of this disease is discussed with particular reference to the place of splenectomy (which was performed in one patient), of phenindione, and of prednisone, which was used with apparent benefit in two patients. The concept is put forward that paroxysmal nocturnal hemoglobinuria may represent a syndrome, characterised by the specific pathologic tests listed, which, it is suggested, may result from an abnormal cell line of erythrocytes." @default.
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• W2042945301 date "1964-12-01" @default.
• W2042945301 modified "2023-09-25" @default.
• W2042945301 title "The syndrome of paroxysmal nocturnal hemoglobinuria" @default.
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• W2042945301 doi "https://doi.org/10.1016/0002-9343(64)90132-9" @default.
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