慢性粒单核细胞白血病相关的肺泡蛋白沉积症：一例报告并文献复习

Chronic myelomonocytic leukemia-associated pulmonary alveolar proteinosis: A case report and review of literature.

作者信息

Chen Can, Huang Xi-Lian, Gao Da-Quan, Li Yi-Wei, Qian Shen-Xian

机构信息

Department of Hematology, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China.

Department of Intensive Care Unit, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China.

出版信息

World J Clin Cases. 2021 Feb 16;9(5):1156-1167. doi: 10.12998/wjcc.v9.i5.1156.

DOI:10.12998/wjcc.v9.i5.1156

PMID:33644180

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7896663/

Abstract

BACKGROUND

Pulmonary alveolar proteinosis (PAP) is a rare condition that can cause progressive symptoms including dyspnea, cough and respiratory insufficiency. Secondary PAP is generally associated with hematological malignancies including chronic myelomonocytic leukemia (CMML). To the best of our knowledge, this is the first reported case of PAP occurring secondary to CMML.

CASE SUMMARY

We report the case of a 63-year-old male who presented with a recurrent cough and gradually progressive dyspnea in the absence of fever. Based upon clinical symptoms, computed tomography findings, bone marrow aspiration, flow cytometry studies and cytogenetic analyses, the patient was diagnosed with PAP secondary to CMML. He underwent whole lung lavage in March 2016 to alleviate his dyspnea, after which he began combined chemotherapeutic treatment with decitabine and cytarabine. The patient died in January 2020 as a consequence of severe pulmonary infection.

CONCLUSION

This case offers insight regarding the mechanistic basis for PAP secondary to CMML and highlights potential risk factors.

摘要

背景

肺泡蛋白沉积症（PAP）是一种罕见疾病，可导致进行性症状，包括呼吸困难、咳嗽和呼吸功能不全。继发性PAP通常与血液系统恶性肿瘤相关，包括慢性粒单核细胞白血病（CMML）。据我们所知，这是首例报道的继发于CMML的PAP病例。

病例摘要

我们报告了一名63岁男性病例，该患者反复咳嗽，在无发热情况下逐渐出现进行性呼吸困难。根据临床症状、计算机断层扫描结果、骨髓穿刺、流式细胞术研究和细胞遗传学分析，该患者被诊断为继发于CMML的PAP。他于2016年3月接受了全肺灌洗以缓解呼吸困难，之后开始接受地西他滨和阿糖胞苷联合化疗。该患者于2020年1月因严重肺部感染死亡。