Valla D, Dhumeaux D, Babany G, Hillon P, Rueff B, Rochant H, Benhamou J P
Gastroenterology. 1987 Sep;93(3):569-75.
In a series of 40 patients with Budd-Chiari syndrome, 5 (12%) were found to be afflicted with paroxysmal nocturnal hemoglobinuria. The clinico-pathological features in these 5 patients and in 26 well-documented previously reported cases could be ascribed to three groups of increasing severity: thrombosis limited to small-sized hepatic veins with no or transient ascites, partial thrombosis of large-sized hepatic veins with chronic ascites, and complete thrombosis of large-sized hepatic veins with a life-threatening course. These three groups did not differ with regard to sex, age, and duration and characteristics of paroxysmal nocturnal hemoglobinuria. In view of the relationship between prognosis and the extent of hepatic vein obstruction, we suggest that early therapy directed toward limiting the extension of thrombosis, or toward dissolving formed thrombi, should improve the prognosis of this severe complication of paroxysmal nocturnal hemoglobinuria.
在一组40例布-加综合征患者中，发现5例（12%）患有阵发性夜间血红蛋白尿。这5例患者以及之前26例有充分记录的报道病例的临床病理特征可归为三组，严重程度逐渐增加：血栓形成局限于小肝静脉，无腹水或仅有短暂腹水；大肝静脉部分血栓形成伴慢性腹水；大肝静脉完全血栓形成且病程危及生命。这三组在性别、年龄、阵发性夜间血红蛋白尿的病程和特征方面并无差异。鉴于预后与肝静脉阻塞程度之间的关系，我们建议早期治疗旨在限制血栓形成的扩展或溶解已形成的血栓，这应能改善阵发性夜间血红蛋白尿这种严重并发症的预后。
