特发性肺含铁血黄素沉着症误诊21例

Twenty-One Cases of Misdiagnosed Idiopathic Pulmonary Hemosiderosis

目的探讨儿童特发性肺含铁血黄素沉着症（IPH）的误诊情况及其临床特点。方法回顾性分析本院1993年6月-2007年5月收治的曾误诊的21例IPH的临床资料。男9例，女12例；发病年龄1～14岁；病程1～6个月。所有患儿均通过胸部X线片、胸部cT、骨髓穿刺检查、痰、胃液或支气管肺泡灌洗液检查发现含铁血黄素巨噬细胞而确诊，分析IPH患儿的治疗情况及预后。结果21例患儿有程度不同的贫血，咳嗽11例（52．38％），发热9例（42．86％），纳差、乏力6例（28．50％），咯血4例（19．05％）。X线胸片及cT呈点网状、斑片状改变。行骨髓常规检查18例，均显示增生性骨髓像。其中4例（22．2％）伴缺铁性贫血改变；痰、胃液或支气管灌洗液检查找到含铁血黄素巨噬细胞19例（90．50％）。21例均被误诊，误诊为支气管肺炎并轻度贫血8例，肺结核并贫血5例，缺铁性贫血4例，溶血性贫血3例，骨髓增生异常综合征1例。确诊IPH后行肾上腺皮质激素治疗，均有效。其中4例联合使用大剂量人血丙种球蛋白治疗，3例联合使用长春新碱治疗。随访18例，其中3例痊愈并停止治疗2a以上，11例患儿临床持续缓解，4例病情反复有恶化趋势。结论儿童IPH易误诊，早期诊断、长期正规治疗对尽早控制急性发作、减少复发次数、改善预后有重要的作用。

Objective To explore the clinical characteristics, diagnosis, treatment and misdiagnosis matter of children with idiopathic pulmonary hemosiderosis （IPH）. Methods The data of clinical characteristics, laboratory examination, treatment and follow - up of 21 chil- dren admitted from Jun. 1993 to May 2007 were retrospectively analyzed, included 9 males, 12 females, aged 1 -14 years old, course of di- sease were 1 - 6 months. Twenty - one patients were diagnosed as IPH by chest X - ray radiography, CT scan, bone marrow biopsy, hemosi- derin - laden macrophages in either sputum or gastric juice or bronchoalveolar lavage fluid. Then, therapy and prognosis of IPH were analyzed. Results All patients had varied degrees of anemia, 11 （52.38%） children had cough,9 （42.86%） children had fever,6 （28.50%） cases had shortage of orexia,4 （ 19.05% ） children had hemoptysis. Chest X- ray radiography and CT scan demonstrated diffuse patchy, nodular, reticulate pattern. Eighteen children received bone marrow biopsy and presented hyperplastic erythropoiesis,4 （22.2%） cases were accompanied iron deficiency anemia. Nineteen （90.80%） cases shown the presence of hemosiderin - laden macrophages in either sputum or gastric juice or bronchoalveolar lavage fluid. Twenty - one misdiagnosed patients consisted of bronchopneumonia combined anemia （8 cases） , lung tuberculosis combined anemia （ 5 cases） , nulli - iron anemia （4 cases） , hemolytic anemia （ 3 cases ） , myelodysplastic syndrome （ 1 case ） and received corticosteroid therapy. Four cases of all patients were associatated with large - dose human - 31 - globulin and 3 cases were associatated with vincfistine therapy. The therapeutic effect was significant. Eighteen patients were followed - up, 3 patients were of which cured and had stopped treatment for over 2 years,11 patients presented clinically persistent remission,4 patients were recurred and aggravated. Conclusions Early diagnosis and long - term therapy of cortieosteroid are very important for controlling acute onset, lessening the frequency of IPH recurrence and improving prognosis of the disease.