Paroxysmal nocturnal hemoglobinuria. Report of a case with refractory megaloblastic-hypoplastic bone marrow, bizarre erythrocyte morphology, and a positive Coombs' test.

Abstract

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