Tekrarlayan Akciğer Enfeksiyonu ve Dirençli Demir Eksikliği Anemisi Ayırıcı Tanısında Nadir Bir Antite: İdiyopatik Pulmoner Hemosiderozis

Abstract

İdiyopatik pulmoner hemosiderozis (İPH), demir eksikliği anemisi (DEA), tekrarlayan alveoler hemoraji atakları ve hemoptizi ile seyreden ve etyolojisi tam bilinmeyen nadir bir hastalıktır. Olguların büyük çoğunluğu çocukluk döneminde görülür. Klinik olarak hemoptizi, alveoler hemoraji atakları sırasında gelişen solunum sıkıntısı, akciğer grafisinde parankimal infiltratlar ve sekonder demir eksikliği anemisi gibi bulgularla prezente olur. Bu nedenle de tekrarlayan solunum sıkıntısı ve demir eksikliği anemisine neden olan diğer hastalıklarla ayırıcı tanısı yapılmalıdır. Biz burada tekrarlayan alt solunum yolu infeksiyonu ve kistik fibrozis ön tanısıyla izlenen ve İPH tanısı alan iki yaşındaki bir olguyu sunuyoruz. Olgunun tanısı bronkoalveoler lavaj sıvısında hemosiderin yüklü makrofajlar görülerek doğrulanmış ve steroid tedavisi ile remisyon sağlanmıştır.

Keywords

• demir eksikliği anemi
• kistik fibrozis
• pulmoner hemosiderozis

A Rare Entity in the Differential Diagnosis of Recurrent Pulmonary Infection and Refractory Iron Deficiency Anemia: Idiopathic Pulmonary Hemosiderosis

Abstract

Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder of unknown etiology and characterized with iron deficiency anemia (IDA), recurrent attacks of alveolar hemorrhage and hemoptysis. The vast majority of cases occur during childhood. Clinically, it is presented with symptoms such as hemoptysis, respiratory distress during attacks of alveolar hemorrhage, parenchymal infiltrates on chest x-ray and secondary iron deficiency anemia. Therefore, differential diagnosis should be done to rule out other diseases causing recurrent respiratory distress and iron deficiency anemia. We present a 2-year-old patient with a history of recurrent lower respiratory tract infection and cystic fibrosis who was diagnosed with IPH. The diagnosis of the case was confirmed by seeing hemosiderin-laden macrophages in bronchoalveolar lavage fluid. Remission was achieved with steroid treatment. 

Keywords

• Iron deficiency anemia
• Cystic fibrosis
• Pulmonary hemosiderosis

References

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