Stock Heather, Kadry Zakiyah, Smith Jill P
Department of Internal Medicine, Harrisburg Hospital, Harriburg, PA, USA.
J Hepatol. 2009 Apr;50(4):831-5. doi: 10.1016/j.jhep.2008.10.035. Epub 2009 Feb 12.
BACKGROUND/AIMS: Bleeding esophageal varices are a common complication of portal hypertension in patients with underlying liver disease. Often patients with hepatic cirrhosis have hypersplenism with thrombocytopenia and leukopenia. Felty's syndrome is a disorder where patients with rheumatoid arthritis develop splenomegaly, neutropenia, and on rare occasions, portal hypertension without underlying cirrhosis.
We present a case of a patient with portal hypertension secondary to Felty's syndrome and discuss the importance of recognizing this condition since the treatment of choice is surgical management with splenectomy. A review of the literature and underlying liver histologic features are discussed.
Medical and surgical management of patients with Felty's syndrome is different from those with portal hypertension due to cirrhosis.
Splenectomy is the treatment of choice for complications of portal hypertension in patients with Felty's Syndrome.
背景/目的：食管静脉曲张破裂出血是患有基础肝病的门静脉高压患者的常见并发症。肝硬化患者常伴有脾功能亢进，出现血小板减少和白细胞减少。费尔蒂综合征是一种类风湿关节炎患者出现脾肿大、中性粒细胞减少，且极少数情况下出现无基础肝硬化的门静脉高压的疾病。
我们报告一例继发于费尔蒂综合征的门静脉高压患者，并讨论认识这种疾病的重要性，因为首选治疗方法是脾切除术。本文还讨论了文献回顾及潜在的肝脏组织学特征。
费尔蒂综合征患者的内科和外科治疗与肝硬化所致门静脉高压患者不同。
脾切除术是费尔蒂综合征患者门静脉高压并发症的首选治疗方法。
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