Bragagni Gianpaolo, Brogna Raffaele, Dazzani Francesca, Franceschetti Paola, Zoli Giorgio
Unità Operativa di Medicina Interna, Ospedale di Cento, Azienda USL di Ferrara.
Ann Ital Med Int. 2004 Jul-Sep;19(3):184-8.
We report the case of a 41-year-old woman, affected by Vaquez syndrome, admitted to our hospital for a severe pain in the right hypochondrium, suddenly followed by hepatomegaly and ascites. The clinical and laboratory data were suggestive of hepatic insufficiency and abdominal ultrasonography, integrated by color Doppler and computed tomography, revealed an interrupted hepatic venous outflow. In addition a spontaneous prolonged partial thromboplastin time was present and the patient was found to be positive for lupus anticoagulant. A transient clinical improvement, with a partial reperfusion of suprahepatic veins, was achieved with medical treatment by using anticoagulants, diuretics and paracentesis. However, the patient showed a subsequence of suprahepatic venous thrombosis, although two transjugular intrahepatic portosystemic shunts with stent placement and local thrombolysis were performed. The polycythemia vera is a disease mainly associated with Budd-Chiari syndrome but, in our patient, the thrombotic event occurred in spite of normal values of hematocrit and platelet count. Certainly in this case the lupus anticoagulant positivity represents an additional thrombogenic factor. Nowadays the antiphospholipid antibody syndrome is a recognized and not unusual cause of Budd-Chiari syndrome but, to our knowledge, this is the first case characterized by the presence of polycythemia vera and antiphospholipid antibody syndrome to be reported.
我们报告了一例41岁患有瓦凯兹综合征的女性病例，该患者因右季肋部剧痛入院，随后突然出现肝肿大和腹水。临床及实验室检查结果提示肝功能不全，腹部超声检查（结合彩色多普勒和计算机断层扫描）显示肝静脉流出道中断。此外，患者出现自发的部分凝血活酶时间延长，且狼疮抗凝物检测呈阳性。通过使用抗凝剂、利尿剂及腹腔穿刺术进行药物治疗，患者获得了短暂的临床改善，肝上静脉部分再通。然而，尽管进行了两次经颈静脉肝内门体分流术并置入支架及局部溶栓治疗，患者仍出现了肝上静脉血栓形成。真性红细胞增多症是一种主要与布加综合征相关的疾病，但在我们的患者中，尽管血细胞比容和血小板计数正常，仍发生了血栓事件。在这种情况下，狼疮抗凝物阳性无疑是一个额外的血栓形成因素。如今，抗磷脂抗体综合征是布加综合征公认且并不罕见的病因，但据我们所知，这是首例同时存在真性红细胞增多症和抗磷脂抗体综合征的病例报告。
