成人特发性肺含铁血黄素沉着症二例误诊分析并文献复习

Idiopathic Pulmonary Hemosiderosis in Adult:2 Misdiagnosed Cases Analysis and Literature Review

目的探讨成人特发性肺含铁血黄素沉着症(idiopathic pulmonary hemosiderosis,IPH)的临床特征、治疗策略及误诊原因、防范措施。方法对2例曾误诊的IPH的临床资料进行回顾性分析,并复习相关文献。结果 1例老年女性因咳嗽、咳痰5年,伴气促1年,加重12 d,咯血3 d入院。曾多次在外院就诊诊断为上呼吸道感染及肺部感染等,入我院后经相关检查诊断为慢性阻塞性肺疾病急性加重期合并肺部感染,予相应治疗1周病情无好转。1例因乏力伴胸闷、呼吸困难1个月,发热、头痛4 d入院。当地医院考虑肺部感染合并缺铁性贫血,予相应治疗症状稍缓解。入我院后经相关检查诊断I型呼吸衰竭、肺孢子菌肺炎,经验性抗感染治疗1周效果一般。2例均行支气管镜支气管肺泡灌洗找到大量含铁血黄素巨噬细胞,同时完善相关检查排除继发性肺出血,确诊为成人IPH,分别予糖皮质激素治疗及输红细胞悬液等对症处理后病情好转。结论临床上对于双肺浸润性病变合并贫血患者,无论有无咯血表现,均需警惕IPH,完善支气管镜检查对其诊断有重要意义。

Objective To investigate clinical features,therapeutic methods,misdiagnosed causes and preventionmeasures of idiopathic pulmonary hemosiderosis （ IPH）in adult. Methods Clinical data of 2 adult misdiagnosed patientswith IPH was retrospectively analyzed,and related literature was also reviewed. Results A elderly female patient was admitted for cough and expectoration for 5 years,short breath for 1 year,aggravation for 12 d and hemoptysis for 3 d,and was diagnosed as having upper respiratory tract infection,pulmonary infection and so on in other hospitals,and the patient was diagnosed as having chronic obstructive lung disease in acute exacerbation period combined with pulmonary infection by related examinations after admission in our hospital,but the patient ＇s condition did not improved after corresponding treatment for 1week. The other patient was admitted for fatigue for 1 month associated by dyspnea and Labored breathing,headaches and fever for 4 d,and was diagnosed as having pulmonary infection combined with irondeficiency anemia in local hospital,and thepatient＇ s condition slightly improved after corresponding treatment,and the patient was diagnosed as having type I respiratoryfailure and pneumocystis pneumonia （ PCP）by related examinations after admission in our hospital,but the curative effect waspoor by experienced antiinfection treatment. Abundant hemosiderinladen macrophage was found by bronchoalveolar lavage,and secondary pulmonary hemorrhage was disaffirmed after performing related examinations simultaneously,and then adult IPHwas confirmed in the 2 patients. Conditions of the 2 patients were improved after corresponding treatments of glucocorticoid anderythrocyte suspension transfusion respectively. Conclusion Clinicians should consider the possibility of IPH when patientshave bilateral pulmonary infiltration lesions combined with anemia with whether having hemoptysis or not,and bronchoscopy isvaluable in diagnosis.