Eldem İrem, İleri Talia, İnce Elif, Asarcikli Fikret, Pekpak Esra, Çakmakli Hasan F, Ceyhan Koray, Uysal Zümrüt
Departments of *Pediatrics †Pediatric Hematology ‡Pathology, Ankara University School of Medicine, Dikimevi, Ankara, Turkey.
J Pediatr Hematol Oncol. 2015 Oct;37(7):e435-7. doi: 10.1097/MPH.0000000000000412.
Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder with unknown pathogenesis that usually presents in the first decade of life. As a result of diffuse alveolar hemorrhage, respiratory symptoms such as cough attacks, hemoptysis, dyspnea, and recurrent and refractory iron-deficiency anemia (IDA) are observed. We present an 8-year-old girl who was followed up with recurrent IDA and allergic asthma and later diagnosed with IPH. IPH was confirmed by the presence of hemosiderin-laden macrophages in bronchoalveolar lavage obtained by bronchoscopy and exclusion of the secondary causes of pulmonary hemosiderosis. Glucocorticoids and iron supplementation were started. Clinical and laboratory improvement was observed with therapy. Our case illustrates that refractory/recurrent IDA with any pulmonary symptoms may be the only presenting feature of IPH.
特发性肺含铁血黄素沉着症(IPH)是一种发病机制不明的罕见疾病,通常在生命的第一个十年出现。由于弥漫性肺泡出血,可观察到咳嗽发作、咯血、呼吸困难和反复难治性缺铁性贫血(IDA)等呼吸道症状。我们报告一名8岁女孩,她因反复IDA和过敏性哮喘接受随访,后来被诊断为IPH。通过支气管镜检查获得的支气管肺泡灌洗中存在含铁血黄素巨噬细胞,并排除了肺含铁血黄素沉着症的继发原因,从而确诊为IPH。开始使用糖皮质激素和补充铁剂。治疗后观察到临床和实验室指标有所改善。我们的病例表明,伴有任何肺部症状的难治性/复发性IDA可能是IPH的唯一表现特征。