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1例血液系统恶性肿瘤治疗后等待肺移植期间发生胸膜实质纤维弹性组织增生症伴胸腔漏气综合征:肺容积快速减少的影像学及病理表现

A case of thoracic air leak syndrome with pleural parenchymal fibroelastosis after treatment for hematologic malignancy while awaiting lung transplantation: Imaging and pathological findings of rapid loss in lung volume.

作者信息

Kasai Hajime, Terada Jiro, Nagata Jun, Yamamoto Keiko, Shiohira Shunya, Tomikawa Atsuko, Tamura Nao, Yamamoto Emiri, Ikehara Yuzuru, Suzuki Takuji

机构信息

Department of Respirology, Graduate School of Medicine, Chiba University, Chiba, 260-8670, Japan.

Health Professional Development Center, Chiba University Hospital, Chiba, 260-8670, Japan.

出版信息

Respir Med Case Rep. 2022 Mar 14;37:101630. doi: 10.1016/j.rmcr.2022.101630. eCollection 2022.

Abstract

We report the case of a 29-year-old man who underwent umbilical cord blood transplantation for chronic myelogenous leukemia 14 years previously. He was diagnosed with secondary pleuroparenchymal fibroelastosis (sPPFE) following treatment for hematologic malignancies (sPPFE after HM-Tx) 2.5 years ago. On computed tomography, pleural thickening in the upper lobe, lung volume loss, and recurrent bilateral pneumothorax were detected. Although he waited for cadaveric lung transplantation (LTx) for 1.5 years, his respiratory failure worsened, and he died. Pathological autopsy and clinical course indicated sPPFE. After diagnosing sPPFE after HM-Tx, the timing for deciding LTx is critical, especially when pneumothorax recurs.

摘要

我们报告了一例29岁男性患者的病例,该患者14年前因慢性粒细胞白血病接受了脐带血移植。2.5年前,他在接受血液系统恶性肿瘤治疗后(血液系统恶性肿瘤治疗后发生的继发性胸膜实质纤维弹性组织增生症,即HM-Tx后sPPFE)被诊断为继发性胸膜实质纤维弹性组织增生症。在计算机断层扫描中,发现上叶胸膜增厚、肺容积减少和双侧复发性气胸。尽管他等待尸体肺移植(LTx)达1.5年,但呼吸衰竭仍恶化,最终死亡。病理尸检和临床病程表明为HM-Tx后sPPFE。在诊断为HM-Tx后sPPFE后,决定进行LTx的时机至关重要,尤其是当气胸复发时。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5677/8933706/03afea75e3dc/gr1.jpg

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