Historical Review: Paroxysmal Nocturnal Hemoglobinuria

Abstract

Abstract 1. The history of paroxysmal nocturnal hemoglobinuria is reviewed, and attention is called to four case reports published in the 19th century. A bibliography of the disease is appended. 2. The outstanding work of Paul Strübing is reviewed. Strübing identified paroxysmal nocturnal hemoglobinuria as a disease entity in 1882 but failed to give it a distinctive name. He described the disease with great accuracy, calling particular attention to the part played by sleep in precipitating the paroxysms. He cited earlier reports of nocturnal hemoglobinuria and by provocative tests differentiated the disease from the other paroxysmal hemoglobinurias. On the basis of his observations he proposed theories regarding the pathogenesis of the disease which have now been shown to be remarkably accurate.