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Article
April 1941

CHRONIC HEMOLYTIC ANEMIA WITH PAROXYSMAL NOCTURNAL HEMOGLOBINEMIA: REPORT OF A CASE WITH ONLY OCCASIONAL HEMOGLOBINURIA AND WITH COMPLETE AUTOPSY

Author Affiliations

Assistant Instructor in Pathology, University of Pennsylvania School of Medicine PHILADELPHIA

From the Medical Clinic of the Hospital of the University of Pennsylvania.

Arch Intern Med (Chic). 1941;67(4):735-745. doi:10.1001/archinte.1941.00200040028002
Abstract

A clinical syndrome characterized by chronic hemolytic anemia associated with paroxysmal nocturnal hemoglobinuria was independently described by Marchiafava1 in 1928 and Micheli2 in 1931. Since then several investigators3 have reported on the clinical manifestations of the disease, but its mechanism remained unknown until 1937, when T. H. Ham4 demonstrated the nature of the factors responsible for the production of the hemolysis.

In a recent article in this journal T. H. Ham5 made the first comprehensive report on these factors. He demonstrated that the fundamental abnormality in this disease resides in the red blood cells, and not in the serum. He showed that the red blood cells of patients with the disease were abnormally sensitive to slight changes in pH. Lowering the pH, even within the physiologic range, always caused increased hemolysis, and raising the pH reduced or eliminated the hemolysis.

Ham also demonstrated the presence of

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