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

Yıl 2020, Cilt: 14 Sayı: 6, 531 - 535, 30.11.2020

Mustafa Büyükavcı Olena Erkun Mehmet Fatih Orhan

https://doi.org/10.12956/tchd.725032

Öz

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.

Anahtar Kelimeler

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

Öz

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. 

Anahtar Kelimeler

Iron deficiency anemia, Cystic fibrosis, Pulmonary hemosiderosis

Kaynakça

• 1. Yajun Zhang, Fenglan Luo, Nini Wang, Yue Song at al. Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients. Abstract Journal of International Medical Research. 2019; Vol. 47(1) 293–302 2. Koker SG, Gözmen S, Oymak Y, et al. Idiopathic pulmonary hemosiderosis mimicking iron deficiency anemia: a delayed diagnosis? Hematology Reports 2017; 9:7048 3. Kabra SK, Bhargava S, Lodha R,et al. Idiopathic pulmonary hemosiderosis: clinical profile and follow up of 26 children. Indian Pediatr.2007;44:333-8 4. Marie E. Egan, Michael S. Schechter, Judith A. Voynow. Cystic Fibrosis, In: R. Kliegman, B. Stanton, J. St. Geme, N. J. Blum, S. Shah, R. Tasker, K. Wilson (eds), Nelson Textbook of Pediatrics, 21th Edition, Elsevier, Philadelphia, 2020; 2282-2297.e1 5. Bell SC, Mall MA, Gutierrez H, Macek M, Madge S, Davies JC, Burgel PR, Tullis E, Castaños C, Castellani C, Byrnes CA, Cathcart F, Chotirmall SH, Cosgriff R, Eichler I, Fajac I, Goss CH, Drevinek P, Farrell PM, Gravelle AM, Havermans T, Mayer-Hamblett N, Kashirskaya N, Kerem E, Mathew JL, McKone EF, Naehrlich L, Nasr SZ, Oates GR, O'Neill C, Pypops U, Raraigh KS, Rowe SM, Southern KW, Sivam S, Stephenson AL, Zampoli M, Ratjen F. The future of cystic fibrosis care: a global perspective. Lancet Respir Med. 2020 Jan;8(1):65-124. 6. MA Nevin. Pulmonary hemosiderosis, In: R. Kliegman, B. Stanton, J. St. Geme, N. J. Blum, S. Shah, R. Tasker, K. Wilson (eds), Nelson Textbook of Pediatrics, 21th Edition, Elsevier, Philadelphia, 2020; 2306-2308.e1 7. Castellazzi L, Patria MF, Frati G, et al. Idiopathic pulmonary haemosiderosis in paediatric patients: how to make an early diagnosis. Ital J Pediatr 2016; 42:86. 8. Minkov M, Kovacs J, Wiesbauer P, Dekan G, Gadner H. Severe anemia owing to occult pulmonary hemorrhage: a diagnostic pitfall. J Pediatr Hematol Oncol. 2006 Jul;28(7):467-70. 9. Agarwal R, Aggarwal AN, Gupta D. Lane-Hamilton syndrome: simultaneous occurrence of coeliac disease and idiopathic pulmonary haemosiderosis. Intern Med J 2007; 37:65 10. Koc AS, Sucu A, Celik U. A different clinical presentation of Heiner syndrome: The case of diffuse alveolar hemorrhage causing massive hemoptysis and hematemesis. Respir Med Case Rep. 2019 Jan 23;26:206-208.