Good综合征合并肺孢子菌肺炎一例并文献复习

Good syndrome associated with pneumocystis carinii pneumonia:a case report and review of the literature

目的 提高对Good综合征并发肺孢子菌肺炎 (PCP)的认识和诊断水平。方法 报道1例Good综合征并发PCP临床表现、诊断、治疗经过以及转归。以 “Good综合征”且 “肺孢子菌”为检 索 词 检 索 万 方 数 据 库 和 中 国 知 网;以 “Goodsyndrome” 或 “Good'ssyndrome” 且“pneumocystis”为检索词,检索PubMed数据库,检索时间截至2018年3月。结果 患者女,46岁,胸腺瘤切除术后3年出现咳嗽、咳痰、气喘。血免疫球蛋白定量分析显示免疫球蛋白下降,符合Good综合征诊断。经皮肺穿刺病理明确肺部病变为PCP。给予复方磺胺甲恶唑联合注射免疫球蛋白治疗后,患者肺部病变较前轻度好转后出院,于出院1月余死亡。PubMed数据库、万方数据库、中国知网检索到7篇Good综合征合并PCP相关文献,共6例患者。其中男4例,女2例;年龄范围为43~74岁;临床症状以咳嗽、气喘、发热为主。所有患者免疫球蛋白G均低于参考值。除1例死亡外,其余患者随访症状均好转。结论 不明原因免疫球蛋白缺陷患者应该考虑Good综合征可能。若Good综合征患者肺部出现感染病灶,要警惕并发PCP。

Objective To enhance the acknowledgement and diagnosis of Good syndrome with pneumocystis pneumonia (PCP).Methods The clinical data includmg manifestations,diagnosis, treatments,and outcomes of a Good syndrome patient with PCP was reported.The electronic databases, namely PubMed,Wangfang,CNKI,were searched by using the following search terms:Good syndrome or Good's syndrome,and pneumocystis before March 2018.Results A 46 years old female presented with cough,sputum and dyspnea three years after thymectomy for a thymoma.A quantitative analysis of immunoglobulin revealed hypogammaglobulinemia,and therefore Good syndrome was considered.The concomitant diagnosis of PCP was established by lung biopsy.Sulfamethoxazole and intravenous immunoglobulin infusions were prescribed.The patient then discharged due to mild improvement of pulmonary lesions.However,the patient died after more than one month of discharge.A total of seven articles that involved six patients with four male and two female were included.Age ranged from 43 to 74 years.The main symptoms were cough,dyspnea and fever.Serum immunoglobulin G concentrations of all patients were lower than reference value.Five patients had improvement of symptoms during follow-up, and only one died.Conclusions Awareness of Good syndrome is significantly important,especially in patients with unexplained immunoglobulin deficiency'.PCP should be considered once a Good syndrome patient suffers from pneumonia.