成人血液病合并肺孢子菌肺炎9例临床分析

Clinical analysis of 9 cases of Pneumocystis jirovecii pneumonia in adult haematology patients

目的研究成人血液病患者发生肺孢子菌肺炎(PCP)的临床特点、高危因素、治疗方式、预后及预防措施。方法对2014年1月~2019年7月我院血液科收治的成人血液病患者中确诊为PCP者的临床表现、实验室检查、影像学特点、治疗及转归进行总结及讨论。结果9例发生PCP患者中8例为血液系统恶性肿瘤,7例为接受异基因造血干细胞移植术后患者,移植术后合并PCP的中位时间为8个月;9例患者中8例均合并巨细胞病毒血症,其中4例为多重感染;所有病例发病期间均有发热,7例伴有咳嗽咳痰;9例患者血清1-3-β-D葡聚糖(104.3~1377.1)pg/mL,中位数为293.3 pg/mL,较参考值偏高;影像学表现以弥漫性斑片影和磨玻璃影为主,严重时可出现结节及胸腔积液;给予TMP/SMZ联合伏立康唑或卡泊芬净或单用卡泊芬净治疗后8例好转,1例死亡。结论成人血液病并发PCP进展较快,常合并多重感染;发热、咳嗽是成人血液病合并PCP主要的临床表现;影像学双侧斑片样肺炎改变,血清1-3-β-D葡聚糖浓度升高对PCP诊断具有指导意义,肺泡灌洗液中查见肺孢子菌是诊断的金标准;对于高危患者应予以抗PCP预防用药,合并PCP的血液病患者早期抗PCP治疗是降低死亡率和改善预后的关键。

Objective To discuss the clinical characteristics,risk factors,treatment methods,prognosis and prevention measures of the Pneumocystis jirovecii pneumonia(PCP)in adult hematologic disorders.Methods The clinical manifestations,laboratory tests,imaging features,treatment and outcomes of adult patients with hematology diagnosed as PCP from January 2014 to July 2019 were collected.Results In all 9 patients with PCP,8 patients were hematologic malignancies and 7 patients were with allogeneic hematopoietic stem cell transplantation.The median time of diagnosed as PCP after transplantation was 8 months.8 patients were associated with cytomegalovirus,4 of which were multiple infections in all 9 cases.All cases had fever during the infection,and 7 cases were accompanied by cough and sputum production.The serum 1-3-β-D glucan was ranging from 104.3 to 1377.1 pg/mL,the median was 293.3pg/mL,higher than the reference value.Diffuse plaques or ground-glass opacity were the primary manifestation in imaging findings.Nodules or pleural effusion were occurred in severe cases.All patients were treated with TMP/SMZ combined with voriconazole or caspofungin or with caspofungin alone,8 patients were cured and one patient died.Conclusion The PCP in adult hematologic disease patients progresses rapidly,and which is usually with multiple infections.Fever and cough were the main clinical manifestation of adult hematologic disease complicated with PCP.Bilateral patchy areas in CT findings and serum 1-3-β-D glucan concentration increases both contribute to the diagnosis of PCP.Finding Pneumocystis in the bronchoalveolar lavage fluid(BALF)is the gold standard for diagnosis.Anti-PCP prophylaxis should be given for high-risk patients.Early treatment of PCP is the key to reduce death and improve the prognosis of PCP in adult hematologic disease patients.