Paroxysmal nocturnal hemoglobinuria: analysis of 40 cases.
Forty cases of paroxysmal nocturnal hemoglobinuria (PNH) were studied from 1978 to 1988. Thirty patients were male and 10 were female. Their ages ranged from 15 to 58 years with a median of 32 years. Symptoms related to anemia (85%) and dark colored urine (45%) were the most frequent clinical manifestations. Seven patients (17.5%) had a previous history of aplastic anemia. The interval between the diagnosis of aplastic anemia and PNH ranged from 11 months to 26 years. All the patients had anemia with varying combinations of cytopenia. In 36 patients, bone marrow examinations were performed, and 32 were hypercellular, one normocellular and 3 hypocellular. Documented thrombosis was noted in 3 patients, involving the intra-abdominal, cerebral and renal veins, respectively. The patient with intra-abdominal venous thrombosis subsequently died of E. coli septicemia. The remaining two patients achieved complete recovery. All 40 patients were treated with corticosteroids and/or anabolic agents, 32 (80%) patients improved and 4 (10%) achieved normal hemoglobin levels. Our studies demonstrate that there is a male predominance in Chinese PNH patients and the incidence of thrombotic complications is much lower than that reported by Western countries.
Department of Internal Medicine, Chang Gung Memorial Hospital, Taipei, Taiwan, R.O.C.
P
P
Dunn
L Y
LY
Shih
S J
SJ
Liaw
D000293:Adolescent; D000328:Adult; D000741:Anemia, Aplastic / Q000150:complications; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications* / Q000401:mortality / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D015996:Survival Rate