Fujioka S, Asai T
Nihon Ketsueki Gakkai Zasshi. 1989 Dec;52(8):1386-94.
A survey on the survival of a total of 160 patients with paroxysmal nocturnal hemoglobinuria (PNH) was conducted by questionnaires to hematologists at the 86 major hospitals in Japan. Ten, 20 and 30 year-survival rates from the day of diagnosis was 71%, 57.5% and 57.5%, respectively. The survival curves by sex showed no statistical difference between males and females. The cause of death in 56 patients with PNH was investigated. Twenty cases, representing 38.5% of 52 patients excluding 4 cases of unknown cause, died of hemorrhage. Death from thrombosis occurred only in 3 cases. The incidence of hemorrhage and thrombosis as a cause of death in Japanese patients was clearly different from that in the United States and England. Various causes other than hemorrhage and thrombosis were disclosed. Patients died 4.5 years (median) from diagnosis and at a mean age of 50.4 years. Hypoplastic bone marrow was found histologically in 40.4% of 52 patients by biopsy. The findings of peripheral blood and aspirated bone marrow were nearly consistent with those of hypoplasia. A higher incidence of hemorrhagic death in Japanese patients might be related to thrombocytopenia by hypoplastic bone marrow. On initial diagnosis, 41.2% had aplastic anemia-PNH syndrome. Prevention against complications in PNH with special reference to blood transfusions are discussed.
通过向日本86家主要医院的血液科医生发放问卷，对总共160例阵发性夜间血红蛋白尿（PNH）患者的生存情况进行了调查。从诊断之日起的10年、20年和30年生存率分别为71%、57.5%和57.5%。按性别绘制的生存曲线显示，男性和女性之间无统计学差异。对56例PNH患者的死因进行了调查。在排除4例死因不明的患者后，52例患者中有20例（占38.5%）死于出血。仅有3例死于血栓形成。日本患者中出血和血栓形成作为死因的发生率与美国和英国明显不同。还发现了出血和血栓形成以外的各种死因。患者从诊断起4.5年（中位数）死亡，平均年龄为50.4岁。通过活检发现，52例患者中有40.4%组织学上存在骨髓增生低下。外周血和骨髓穿刺结果与骨髓增生低下情况基本一致。日本患者出血性死亡发生率较高可能与骨髓增生低下导致的血小板减少有关。初诊时，41.2%的患者患有再生障碍性贫血-PNH综合征。文中讨论了PNH并发症的预防，特别提及了输血方面。
