Graham M L, Rosse W F, Halperin E C, Miller C R, Ware R E
Department of pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA.
Br J Haematol. 1996 Mar;92(3):707-10. doi: 10.1046/j.1365-2141.1996.365901.x.
Thrombosis of the hepatic veins (Budd-Chiari syndrome) is a life-threatening thrombotic complication which can occur in patients with paroxysmal nocturnal haemoglobinuria (PNH). Despite aggressive medical and surgical therapy, mortality from Budd-Chiari syndrome remains high. We report a boy with PNH who developed Budd-Chiari syndrome and underwent syngeneic bone marrow transplantation (BMT). Now, 3 years following BMT, he has had dramatic clinical and radiographic evidence of resolution of the thrombosis. We suggest that BMT for PNH can successfully correct life-threatening thrombosis in patients with PNH.
肝静脉血栓形成（布加综合征）是一种危及生命的血栓性并发症，可发生于阵发性夜间血红蛋白尿（PNH）患者。尽管采取了积极的药物和手术治疗，布加综合征的死亡率仍然很高。我们报告一名患有PNH的男孩，他发生了布加综合征并接受了同基因骨髓移植（BMT）。现在，BMT后3年，他有显著的临床和影像学证据表明血栓已消退。我们认为，PNH患者的BMT可成功纠正危及生命的血栓形成。
