案例#1
一名 40 岁女性突发上腹痛和腹水。她已服用复合口服避孕药7 年。体检发现该女性肝肿大压痛。无肝颈静脉回流且无下肢水肿状况。心血管检查未显示异常。已知她患有真性红细胞增多症10年。
案例#2
一名有 12 年原发性血小板增多病史的 50 岁男性出现轻度黄疸和 RUQ 隐痛。体检显示微量腹水、肝肿大和脾肿大。
其他表现
典型的症状三联征为腹痛、腹水和肝肿大。[5]Mitchell MC, Boitnott JK, Kaufman S, et al. Budd-Chiari syndrome: etiology, diagnosis and management. Medicine (Baltimore). 1982;61:199-218.http://www.ncbi.nlm.nih.gov/pubmed/7045569?tool=bestpractice.com[6]Hemming AW, Langer B, Greig P, et al. Treatment of Budd-Chiari syndrome with portosystemic shunt or liver transplantation. Am J Surg. 1996;171:176-180.http://www.ncbi.nlm.nih.gov/pubmed/8554136?tool=bestpractice.com在 20% 的患者中观察到急性 BCS。[7]Hadengue A, Poliquin M, Vilgrain V, et al. The changing scene of hepatic vein thrombosis: recognition of asymptomatic cases. Gastroenterology. 1994;106:1042-1047.http://www.ncbi.nlm.nih.gov/pubmed/8143970?tool=bestpractice.com这些患者在数周内发生重度 RUQ 腹痛、肝肿大、黄疸和难治性腹水。在 5% 至 20% 的病例中检测到 LFT 结果轻度异常,但没有症状。[7]Hadengue A, Poliquin M, Vilgrain V, et al. The changing scene of hepatic vein thrombosis: recognition of asymptomatic cases. Gastroenterology. 1994;106:1042-1047.http://www.ncbi.nlm.nih.gov/pubmed/8143970?tool=bestpractice.com[8]Murad SD, Valla DC, de Groen PC, et al. Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology. 2004;39:500-508.http://www.ncbi.nlm.nih.gov/pubmed/14768004?tool=bestpractice.com暴发性 BCS 不常见。患者很快出现肝性脑病、肾衰竭和凝血功能障碍。肝脏增大且有压痛感。[9]Valla DC. Hepatic vein thrombosis (Budd-Chiari syndrome). Semin Liver Dis. 2002;22:5-14.http://www.ncbi.nlm.nih.gov/pubmed/11928075?tool=bestpractice.com下腔静脉 (IVC) 压迫或血栓形成的患者除肝肿大、腹水和腹痛外还会出现下肢水肿或静脉侧支循环。症状的严重性低于肝静脉血栓形成。[10]Kage M, Arakawa M, Kojioro M, et al. Histopathology of membranous obstruction of the inferior vena cava in the Budd-Chiari syndrome. Gastroenterology. 1992;102:2081-2090.http://www.ncbi.nlm.nih.gov/pubmed/1587428?tool=bestpractice.com[11]Shrestha SM, Okuda K, Uchida T, et al. Endemicity and clinical picture of liver disease due to obstruction of the hepatic portion of the inferior vena cava in Nepal. J Gastroenterol Hepatol. 1996;11:170-179.http://www.ncbi.nlm.nih.gov/pubmed/8672764?tool=bestpractice.com