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February 1957

Paroxysmal Nocturnal Hemoglobinuria: Atypical Manifestations Suggesting an Immunologic Disease

Author Affiliations


From the Blood Research Laboratory, a unit of the Ziskind Laboratories, of the New England Center Hospital, and the Department of Medicine, Tufts University School of Medicine.

AMA Arch Intern Med. 1957;99(2):202-208. doi:10.1001/archinte.1957.00260020038006

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hemolytic disease of insidious onset and chronic course, characterized by an intracorpuscular erythrocytic defect rendering the cells unusually susceptible to hemolysis in vitro and probably in vivo upon slight acidification of the plasma. The constant feature of the disease is hemosiderinuria, rather than hemoglobinuria1; in some cases hemoglobinuria may occur rarely or not at all.2-5 Although an excellent account of this syndrome was provided by Striibing, in 1882,6 only 38 cases had been recorded by 1937.7 Crosby's classic monograph in 1953 analyzed the clinical and laboratory features of 162 collected cases.8 Since then, many typical cases have been readily recognized by their clinical features, with subsequent laboratory confirmation by the Ham acid-hemolysis test,9 and 18 additional cases have been reported.10,23

The atypical clinical and serologic features of the following three cases rendered diagnosis difficult and merit mention. Two of these cases presented with

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