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一名62岁女性,因髓外造血导致双侧胸腔积液和肺部浸润。

A 62-year-old woman with bilateral pleural effusions and pulmonary infiltrates caused by extramedullary hematopoiesis.

作者信息

Schwarz C, Bittner R, Kirsch A, Loddenkemper C, Mairinger T, Schonfeld N, Serke M, Loddenkemper R

机构信息

Lungenklinik Heckeshorn, HELIOS Klinikum Emil von Behring, Berlin, Deutschland.

出版信息

Respiration. 2009;78(1):110-3. doi: 10.1159/000150913. Epub 2008 Aug 4.

Abstract

A 62-year-old female presented with a 1-month history of irritating cough and increasing dyspnea. A chronic idiopathic myelofibrosis had been diagnosed 5 years ago. CT of the chest and abdomen showed bilateral pleural effusions with a thickened pleura, nodular infiltrations in both lungs, enlarged intraabdominal lymph nodes and splenomegaly. Pleuroscopy (medical thoracoscopy) on the left side revealed dense tumorous nodules mainly on the posterior chest wall pleura, but also on the diaphragm and the lung. Biopsies taken from the chest wall pleura revealed extramedullary hematopoiesis (EMH) with abnormal megakaryocytes as well as myeloid and erythroid precursors. After unsuccessful tetracycline pleurodesis, talcum slurry was instilled via the chest tube without recurrence of the pleural effusion. Furthermore, treatment with hydroxyurea was started, and the disease regressed and then remained stable over the next 24 months. In conclusion, the pleuropulmonary findings were caused by EMH due to chronic idiopathic myelofibrosis. The definite diagnosis was established by pleuroscopy followed by successful pleurodesis with talc slurry, after tetracycline pleurodesis had failed.

摘要

一名62岁女性,有1个月刺激性咳嗽和进行性呼吸困难病史。5年前诊断为慢性特发性骨髓纤维化。胸部和腹部CT显示双侧胸腔积液伴胸膜增厚、双肺结节状浸润、腹腔淋巴结肿大及脾肿大。左侧胸腔镜检查(内科胸腔镜)显示密集的肿瘤样结节,主要位于后胸壁胸膜,但也见于膈肌和肺。取自胸壁胸膜的活检显示髓外造血(EMH),伴有异常巨核细胞以及髓系和红系前体细胞。四环素胸膜固定术失败后,经胸管注入滑石粉浆,胸腔积液未复发。此外,开始使用羟基脲治疗,疾病在接下来的24个月内消退并保持稳定。总之,胸膜肺部表现是由慢性特发性骨髓纤维化引起的EMH所致。在四环素胸膜固定术失败后,通过胸腔镜检查并成功使用滑石粉浆进行胸膜固定术,确立了明确诊断。

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