Abstract

Summary1. A patient with paroxysmal nocturnal hemoglobinuria is presented to emphasize the importance of an adequate history, physical examination and appropriate laboratory studies in the diagnosis of anemia, especially of this type.2. Paroxyasmal nocturnal hemoglobinuria produces a chronic normocytic anemia, of unknown etiology, though it is thought to represent an intracorpuscular defect, although extracorpuscular alterations are necessary before hemolysis develops, such as sleep and/or change in the pH of the plasma.3. The laboratory findings of significance will be revealed in the complete blood count, bone marrow examination, urinalysis, particularly for bile, iron, free hemoglobin and red cells, reticulocyte count and the Ham test. Other hematologic tests which are usually normal are, the fragility test, Coomb's test, plasma and hemoglobin electrophoresis. Radioactive chromium tagged RBC. and ferrokynetics, while not diagnostic in themselves, will provide useful information in the study of patients with hemolytic anemaias.4. Treatment of paroxysmal nocturnal hemoglobinuria is predominantly replacement of iron, transfusions of washed red blood cells when necessary, and avoidance of splenectomy At times it may be necessary to use anticoagulants (coumarin derivatives) if hemolytic crises are accompanied by thromboses, but not heparin. The prognosis is generally fair, especially in patients who do not manifest the major complications of thrombosis or severe hemolytic crises.