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Report of a Case with Postmortem Observations

MATTHEW J. ELLENHORN, M.D.; LAWRENCE Z. FEIGENBAUM, M.D; CALVIN PLUMHOF, M.D.; STACY R. METTIER, M.D.

AMA Arch Intern Med. 1951;87(6):868-878.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

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. . . . [Full Text PDF of this Article]

Author Affiliations
SAN FRANCISCO

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

Footnotes
Since this report was submitted for publication, an excellent review of the historical aspects of the disease has appeared: Crosby, W. H.: Historical Review: Paroxysmal Nocturnal Hemoglobinuria: A Classic Description by Paul Strübing in 1882, and a Bibliography of the Disease. Blood 6:270-285, 1951.

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