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[42岁真性红细胞增多症患者的不明原因肝纤维化]

[Unclear liver fibrosis in a 42-year-old patient with polycythemia vera].

作者信息

Gundling F, Kreth F, Tröltzsch M, Tannapfel A, Bödeker H, Mössner J

机构信息

Medizinische Klinik und Poliklinik II, Universität Leipzig.

出版信息

Internist (Berl). 2004 Nov;45(11):1293-8. doi: 10.1007/s00108-004-1271-y.

Abstract

A 42-year-old patient was admitted to hospital because of ascites and polyglobulia. Laboratory tests revealed reduced liver function and a significant elevation of all three hematopoietic cell lines. Liver fibrosis and polycythemia vera were diagnosed by histologic examination. The most frequent causes for liver fibrosis were serologically excluded. Ultrasound combined with Doppler imaging revealed an obstruction of the right hepatic vein, which was indicative of Budd-Chiari syndrome. BCS can occur under fulminant and nonfulminant conditions, which can result in progressive damage of the liver. Phlebotomy and combined therapy with low-dose aspirin and anagrelide achieved permanent reduction of the elevated blood parameters. In the follow-up the patient's clinical course was stable without hepatic decompensation.

摘要

一名42岁患者因腹水和红细胞增多症入院。实验室检查显示肝功能减退,所有三种造血细胞系均显著升高。通过组织学检查诊断为肝纤维化和真性红细胞增多症。血清学排除了肝纤维化最常见的病因。超声联合多普勒成像显示右肝静脉阻塞,提示布加综合征。布加综合征可在暴发性和非暴发性情况下发生,可导致肝脏进行性损害。放血治疗以及低剂量阿司匹林和阿那格雷联合治疗使升高的血液参数永久性降低。在随访中,患者的临床病程稳定,未出现肝失代偿。

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