阵发性睡眠性血红蛋白尿(paroxysmal nocturnal haemoglobinuria,PNH)是一种慢性获得性血管内溶血性疾病,其病因和发病机理尚未完全阐明,为了深入探讨其发病机理,不少学者曾对PNH患者进行了骨髓和外周血染色体检查,但报告不一。作者发现1例,现报告如下。患者,男,40岁,65年起反复出现阵发性酱油色尿。睡眠后尿呈酱油色,早晨色著,下午色渐减,发病与站立,运动及受寒均无关。家系成员均无类似疾病。体检:贫血貌,皮肤、巩膜轻度黄染,心尖及肺动脉瓣区 Paroxysmal nocturnal hemoglobinuria (PNH) is a chronic, acquired intravascular hemolytic disease whose etiology and pathogenesis have not yet been fully elucidated. In order to investigate its pathogenesis, many scholars have conducted PNH patients Bone marrow and peripheral blood chromosome examination, but the report is not the same. The authors found 1 case, the report is as follows. Patients, male, 40 years old, 65 years from the recurrence of paroxysmal soy sauce color urine. After sleeping, urine showed soy sauce color, morning color, afternoon color decreasing, incidence and standing, exercise and cold have nothing to do. Family members have no similar diseases. Physical examination: anemia appearance, skin, scleral mild yellow dye, apical and pulmonary valve area