Casciaro S, Ghio R, Balleari E, Boccaccio G
Azienda Ospedaliera Ospedale S. Martino, Genova.
Minerva Cardioangiol. 1998 Jul-Aug;46(7-8):241-53.
Budd-Chiari syndrome is a rather unusual clinical entity; among others, myeloproliferative disorders not infrequently are reported as a cause of this syndrome. In the past prognosis of Budd-Chiari syndrome was usually very poor. However, in recent years treatment with fibrinolytic agents has proved to be often successful in Budd-Chiari syndrome, as well as in other thrombotic disorders. In particular, r-TPA has appeared to be effective, due to its thrombospecificity. Three cases of Budd-Chiari's syndrome associated with myeloproliferative disorders are described, in which r-TPA administration, together with treatment of underlying disease, resulted in a complete recanalization of sovrahepatic veins. r-TPA, due to its thrombospecificity, has been shown to be more effective than other thrombolytic agents; its use is associated with a lower number of hemorrhagic events and it may be repeated in the case of uncompleted response.
布加综合征是一种相当罕见的临床病症;在其他情况中,骨髓增殖性疾病常被报道为该综合征的病因。过去,布加综合征的预后通常非常差。然而,近年来,事实证明,使用纤溶药物治疗布加综合征以及其他血栓性疾病往往是成功的。特别是,重组组织型纤溶酶原激活剂(r-TPA)因其血栓特异性似乎很有效。本文描述了3例与骨髓增殖性疾病相关的布加综合征病例,在这些病例中,给予r-TPA并治疗基础疾病后,肝上静脉完全再通。r-TPA因其血栓特异性,已被证明比其他溶栓药物更有效;其使用导致的出血事件较少,并且在反应未完成的情况下可以重复使用。