Garatti M, Parravicini C, Meroni P L, Giulotto P, Rusconi R, Carnelli V
Pediatr Med Chir. 1983 May-Jun;5(3):95-8.
A 13-years old boy who presented a severe degree of iron-deficiency anemia and diffuse parenchimal infiltrates on the chest roentgenogram is reported. The clinical picture and the presence of hemosiderin laden macrophages in bronchial washing suggest Idiopathic Pulmonary Hemosiderosis (I.P.H.): open lung biopsy confirmed the diagnosis. Immunofluorescence studies showed no deposition of IgG, IgA, IgM and B1C in the lung. The most striking abnormality observed at electron microscopy was hemosiderin deposition in the alveolar-capillary basement membrane. One year after cyclophosphamide therapy was both in complete hematologic and pulmonary remission.
报告了一名13岁男孩，其患有严重程度的缺铁性贫血，胸部X线片显示弥漫性实质浸润。临床症状以及支气管灌洗中出现含铁血黄素的巨噬细胞提示特发性肺含铁血黄素沉着症（IPH）：开胸肺活检确诊了该诊断。免疫荧光研究显示肺中无IgG、IgA、IgM和B1C沉积。电子显微镜下观察到的最显著异常是含铁血黄素在肺泡-毛细血管基底膜中的沉积。环磷酰胺治疗一年后，血液学和肺部均完全缓解。
