Pleuroparenchymal fibroelastosis secondary to autologous hematopoietic stem cell transplantation: A case report
Pleuroparenchymal fibroelastosis secondary to autologous hematopoietic stem cell transplantation: A case report
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自体造血干细胞移植继发的胸膜实质弹力纤维增生症：一例报告
DOI：
10.3892/etm.2019.7275
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发表时间：
2019-04-01
影响因子：
2.7
通讯作者：
Zhai, Zhenguo
中科院分区：
文献类型：
--
作者：
Zhang, Shuai;Xie, Wanmu;Zhai, Zhenguo
摘要胸膜实质纤维弹性增生症是一种罕见的肺间质性疾病。虽然最近报道了越来越多的PPFE病例，但这种情况的特征尚未得到很好的描述。本研究报告的情况下，34岁的男性患者谁提出单侧肺异常。患者因9年进行性咳嗽和用力呼吸困难病史以及接受自体造血干细胞移植（HSCT）治疗的霍奇金淋巴瘤病史而入院。患者最初被诊断为结核病，并接受了18个月的常规抗结核治疗；然而，症状继续恶化。连续胸部计算机断层扫描显示逐渐恶化的弥漫性胸膜增厚，胸膜下致密混浊和体积减少，伴有右肺纤维化的证据。体格检查患者为病质，体重指数为18.5 kg/m(2)，胸廓扁平。动脉血气分析显示缺氧。肺功能检查显示限制性通气功能障碍和弥散能力下降。微生物学和细胞学检查均为阴性。肺活检显示胸膜增厚，由大量胶原蛋白和弹性纤维组成，同时存在胸膜下肺泡内纤维化和肺泡间隔弹性增生，未见炎症浸润。患者被诊断为继发于HSCT的PPFE，最终在等待肺移植时死于呼吸衰竭和感染。医生应该了解这种罕见疾病的典型和非典型特征，因为其临床和放射学特征可能导致误诊，特别是慢性感染。没有有效的长期治疗，预后仍然很差。
Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease. Although an increased number of PPFE cases have been reported recently, the characteristics of this condition have not been well described. The present study reports on the case of a 34-year-old male patient who presented with unilateral lung abnormalities. The patient was admitted due to a 9-year history of progressive cough and exertional dyspnea, as well as a history of Hodgkin's lymphoma treated by autologous hematopoietic stem cell transplantation (HSCT). The patient had been initially diagnosed with tuberculosis and received regular anti-tuberculosis therapy for 18 months; however, the symptoms progressed. Serial chest computed tomography scans indicated a gradually worsening diffuse pleural thickening, dense subpleural opacification and volume loss, associated with evidence of fibrosis in the right lung. On physical examination the patient was cachectic, with a body mass index of 18.5 kg/m(2), and he had a flattened thoracic cage. Arterial blood gas analysis revealed hypoxia. Pulmonary function tests revealed restrictive ventilation dysfunction and decreased diffusion capacity. The microbiological and cytological examinations were negative. Lung biopsy revealed a thickened pleura consisting of large amounts of collagen and elastic fibers, coexisting with subpleural intra-alveolar fibrosis with alveolar septal elastosis, without inflammatory infiltrates. The patient was diagnosed with PPFE secondary to HSCT and eventually succumbed to respiratory failure and infection while waiting for a lung transplant. Physicians should be aware of the typical and atypical characteristics of this rare disease, as its clinical and radiological characteristics may lead to misdiagnosis, particularly as chronic infections. The prognosis remains poor without effective long-term treatment.