合并血液系统疾病的弥漫性实质性肺疾病46例临床分析

Clinical analysis of 46 cases of diffuse parenchymal lung disease with hematological diseases

目的总结合并血液系统疾病的弥漫性实质性肺疾病(diffuse parenchymal lung disease,DPLD)患者的临床特征,以提高临床医生对该类疾病的认识。方法收集2010年1月至2020年10月入住南京鼓楼医院呼吸与危重症医学科的46例合并血液系统疾病的DPLD患者的临床资料。回顾性分析患者的临床特征、实验室检查、影像学表现、诊断方法、治疗及预后等。结果46例患者中,男26例,女20例,年龄(60±13)岁。患者主要症状为咳嗽、咳痰、呼吸困难、发热、胸闷等;实验室检查可见部分患者三系/两系减低,乳酸脱氢酶、C反应蛋白、血沉、β2微球蛋白等升高;胸部高分辨率CT主要表现为双肺磨玻璃影、实变影、结节影、网状影、牵拉性支气管扩张等。13例患者经病理确诊,其中机化性肺炎5例,肺泡蛋白沉积症4例,急性纤维素性机化性肺炎2例,弥漫性肺泡出血1例,双肺淀粉样变性1例。余33例患者为临床诊断,包括3例药物性间质性肺疾病和1例外源性过敏性肺泡炎。以弥漫性肺部病变为首发表现,继而诊断出血液病的患者有30例(65.2%),其中有2例患者同时合并2种血液病。其余16例患者均为血液病诊断先于DPLD。46例患者中,26例患者经治疗后好转,其中18例予糖皮质激素抗炎,8例予N-乙酰半胱氨酸、吡非尼酮治疗肺纤维化,4例予吸入重组人粒细胞–巨噬细胞集落刺激因子和(或)肺泡灌洗,2例予克拉霉素免疫调节等;15例患者明确血液病诊断后因各种因素拒绝治疗转回当地医院后失访;5例患者死亡,2例死于呼吸衰竭,3例因血液病进展死亡。结论DPLD病种繁多,常常病因不明,临床表现缺乏特异性,诊断相当困难。血液系统疾病本身可以是DPLD的病因,而血液病的相关治疗以及治疗后的免疫抑制等状况也都可以引起DPLD,临床上需要仔细甄别并系统鉴别,以便针对不同的病因进行对因治疗,控制病情,改善预后。

Objective To summarize the clinical characteristics of patients with diffuse parenchymal lung disease(DPLD)combined with hematological diseases in order to improve the clinicians’knowledge of these diseases.Methods The clinical data of 46 patients was collected,who were hospitalized in Nanjing Drum Tower Hospital from January 2010 to October 2020 for DPLD combined with hematological diseases.Their clinical manifestations,laboratory tests,imaging features,diagnostic methods,treatment and prognosis were analyzed retrospectively.Results Among the 46 patients,there were 26 males and 20 females,with an average age of 60±13 years old.The main symptoms were cough and sputum,dyspnea,fever,chest tightness,and so on.Laboratory tests showed that some patients had pancytopenia or two-line cytopenia,and increase in lactate dehydrogenase,C-reactive protein,erythrocyte sedimentation rate andβ2-microglobulin.Bilateral ground glass opacity,consolidations,big or small nodules,reticular shadows,and traction bronchiectasis were showed on chest high-resolution computed tomography.Among the 13 patients who were diagnosed clearly by pathology,they had 5 cases of organizing pneumonia,4 cases of pulmonary alveolar proteinosis,2 cases of acute fibrinous and organizing pneumonia,1 case of diffuse alveolar hemorrhage,and 1 case of lung amyloidosis.Thirty-three patients were clinically diagnosed,including 3-case drug-induced interstitial lung disease,and 1-case exogenous allergic alveolitis.The patients with diffuse pulmonary lesions as the first manifestation and subsequently diagnosed with hematological diseases accounted for 65.2%(30/46).Among these patients,2 of them had two kinds of hematological diseases at the same time.In the rest of the 16 cases,hematological diseases were diagnosed before DPLD.Among the 46cases,26 patients improved after treatment,18 of them were treated with glucocorticoid,8 with N-acetylcysteine and pirfenidone,4 with granulocyte-macrophage colony stimulating factor inhaling and/or whole lung alveolar lavage,and 2with clarithromycin for immune regulation,etc.Fifteen patients refused treatment and transferred back to local hospital after the diagnosis of hematological diseases.Five patients died,2 of them died of respiratory failure and 3 of them died of diseases progression.Conclusions DPLD includes many kinds of diseases,with known or unknown etiology and lack of specificity in clinical manifestations.Therefore,diagnosis for them is quite difficult.Hematological diseases themselves can be the causes of DPLD.At the same time,the treatment for hematological diseases and the related immunosuppression after treatment can also cause DPLD.In the clinical practice,careful screening and systematic differentiation are urgently needed in order to treat different causes precisely,control the conditions and improve the prognosis.