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Article
June 1951

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA WITH CHRONIC HEMOLYTIC ANEMIAReport of a Case with Postmortem Observations

Author Affiliations

SAN FRANCISCO

From the Divisions of Medicine and Pathology of the University of California Medical School (San Francisco).

AMA Arch Intern Med. 1951;87(6):868-878. doi:10.1001/archinte.1951.03810060097010
Abstract

PAROXYSMAL nocturnal hemoglobinuria with chronic hemolytic anemia was first described as a clinical entity by Chauffard and Troisier in 1908.1 Subsequent reports by Marchiafava,2 later amplified by Micheli,3 resulted in the eponym Marchiafava-Micheli syndrome. A total of 73 reported cases, including 16 postmortem studies, was collected by Marks in 1949.4 Studies on the pathogenesis of the disease have been pursued in recent years by Ham,5 Dacie6 and others.7 The fundamental abnormality appears to reside within or on the surface of the red cell, thus rendering it susceptible to external influences not ordinarily reactive on the normal erythrocyte.

In the following report a case of paroxysmal nocturnal hemoglobinuria with chronic hemolytic anemia is presented. The outstanding feature was the long history of regularly recurring nausea and vomiting associated with abdominal pain. The diagnosis was made only a few weeks prior to death. Postmortem observations are included.

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