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疑似缺铁性贫血的特发性肺含铁血黄素沉着症:诊断延误?

Idiopathic Pulmonary Hemosiderosis Mimicking Iron Deficiency Anemia: A Delayed Diagnosis?

作者信息

Koker Sultan Aydin, Gözmen Salih, Oymak Yeşim, Karapinar Tuba Hilkay, Can Demet, Genç Sinan, Vergin Raziye Canan

机构信息

Division of Pediatric Hematology, Dr. Behçet Uz Children's Hospital, İzmir, Turkey.

Division of Pediatric Allergy, Dr. Behçet Uz Children's Hospital, İzmir, Turkey.

出版信息

Hematol Rep. 2017 Jun 15;9(2):7048. doi: 10.4081/hr.2017.7048. eCollection 2017 Jun 1.

Abstract

Idiopathic pulmonary hemosiderosis (IPH) is an uncommon chronic disorder in children. It is characterized by recurrent pulmonary hemorrhage and may result in hemoptysis and pulmonary insufficiency. The most common hematologic manifestation of IPH is iron deficiency anemia. The etiology of IPH is not known and its diagnosis may be difficult due to the variable clinical courses. The most helpful signs for identifying IPH are iron deficiency anemia and recurrent or chronic cough, hemoptysis, dyspnea, wheezing. We report here 5 pediatric cases of IPH presenting with iron deficiency anemia and without pulmonary symptoms. Mean corpuscular volume was low in all patients; iron was low in 4 out of 5 cases; total iron binding capacity was high in all of them; ferritin was low in 3 patients. At follow up, none of them had responded successfully to the iron therapy. Although they didn't present with pulmonary symptoms, chest radiographs incidentally revealed diffuse reticulonoduler shadows in all of them. Computed tomography revealed diffuse ground-glass opacities, consolidation, increased density. The diagnosis was confirmed by the detection of hemosiderin-laden macrophages in bronchoalveolar lavage fluid and gastric aspirate. If patients with iron deficiency anemia don't respond to iron therapy, they should be examined for IPH. Chest radiographs should be taken even in absence of pulmonary symptoms. Early diagnosis is important for a timely management of IPH.

摘要

特发性肺含铁血黄素沉着症(IPH)是一种儿童期罕见的慢性疾病。其特征为反复的肺出血,可导致咯血和肺功能不全。IPH最常见的血液学表现是缺铁性贫血。IPH的病因不明,由于临床病程多变,其诊断可能较为困难。识别IPH最有用的体征是缺铁性贫血以及反复或慢性咳嗽、咯血、呼吸困难、喘息。我们在此报告5例以缺铁性贫血为表现且无肺部症状的儿童IPH病例。所有患者的平均红细胞体积均较低;5例中有4例铁含量低;所有患者的总铁结合力均较高;3例患者的铁蛋白较低。随访时,他们均未对铁剂治疗产生有效反应。尽管他们没有肺部症状,但胸部X线片偶然发现他们所有人都有弥漫性网状结节影。计算机断层扫描显示弥漫性磨玻璃影、实变、密度增加。通过在支气管肺泡灌洗液和胃抽吸物中检测到含铁血黄素巨噬细胞确诊。如果缺铁性贫血患者对铁剂治疗无反应,应检查是否患有IPH。即使没有肺部症状也应进行胸部X线检查。早期诊断对于IPH的及时治疗很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/644b/5477471/892933163947/hr-9-2-7048-g001.jpg

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