Paroxysmal nocturnal haemoglobinuria. A clinicopathological study of 26 cases.
26 patients, 13 male and 13 female, with paroxysmal nocturnal haemoglobinuria (PNH) are described. The diagnosis was based on the finding of a positive Ham's test. PNH developed in 4 patients with aplastic anaemia, and 3 patients with established PNH developed marrow hypoplasia during the course of the disease. In 2 cases autoimmune haemolysis was also present; this association has not been described previously. The majority of patients presented with anaemia and dark urine, or with evidence of thrombosis. A high index of suspicion was needed to avoid missing the diagnosis. Haemolytic crises were usually precipitated by infection, and renal failure requiring dialysis sometimes resulted; a positive direct antiglobulin test was often found at times of increased haemolysis. Thromboses were the most frequent complication, and when intra-abdominal vessels were affected, pain was particularly troublesome. The disease had a widely variable course; 4 patients made a complete recovery and 10 died, 8 from thrombotic complications and 2 from infections associated with marrow hypoplasia. Survival ranged from 1 year to 30 years and the median survival in those who died was 3.5 years.
D000293:Adolescent; D000328:Adult; D000368:Aged; D000741:Anemia, Aplastic / Q000209:etiology; D000743:Anemia, Hemolytic / Q000150:complications; D001803:Blood Transfusion; D005260:Female; D005492:Folic Acid / Q000627:therapeutic use; D006457:Hemoglobinuria, Paroxysmal / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology* / Q000628:therapy; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D012744:Sex Ratio; D013927:Thrombosis / Q000209:etiology
D006493:Heparin; D005492:Folic Acid; D011239:Prednisolone