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October 1, 1967

Paroxysmal Nocturnal Hemoglobinuria: Report of a Case With Refractory Megaloblastic-Hypoplastic Bone Marrow, Bizarre Erythrocyte Morphology, and a Postive Coombs' Test

Author Affiliations

Rochester, NY

From the Hematology Unit, Department of Medicine, University of Rochester (NY) School of Medicine and Dentistry. Dr. Bart was a fellow in hematology at the University of Rochester School of Medicine and Dentistry and a clinical fellow of the National Cancer Society; he is now at the USAF Hospital, Sheppard AFB, Wichita Falls, Tex.

Arch Intern Med. 1967;120(4):487-493. doi:10.1001/archinte.1967.04410010101015

PAROXYSMAL nocturnal hemoglobinuria (PNH) is a rare hemolytic disease due to an apparent acquired intracorpuscular defect of the erythrocyte.1,2 Although the exact nature of the cellular defect has not been elucidated, the erythrocytes are quite sensitive, in vitro, to agglutination and complement induced hemolysis.3

The present report describes a patient with paroxysmal nocturnal hemoglobinuria whose illness was characterized by such unusual features as megaloblastic-hypoplastic anemia, bizarre red blood cell (RBC) morphology, and a positive direct Coombs' test.

Report of a Case  The patient is a 32-year-old white male engineer who was first seen in consultation in April 1963 for evaluation of previously diagnosed PNH. In 1946 at the age of 13, he noted red urine in the morning on one occasion. He was well, however, until 1956 (age 23) when he complained of excessive fatigue while playing football. Anemia was found but a specific diagnosis was not established. Two units

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