Pınar ERGÜN, Funda DEMİRAĞ, Çiğdem BİBER, Ülkü Y. TURAY, İ. Atilla KEYF, Yurdanur ERDOĞAN

Idiopathic pulmonary haemosiderosis

Yirmi yedi yaşında kadın hasta dispne, şiddetli hemoptizi ve demir eksikliği anemisi ile başvurdu. Olgunun göğüs radyografisinde bilateral retikülonodüler infiltrasyonlar ve sağ kostodiyafragmatik sinüsü kapatan homojen infiltrasyon mevcuttu. Hasta tüm alveoler hemoraji sendromları yönünden araştırıldı ve açık akciğer biyopsisi ile idiyopatik pulmoner hemosiderozis tanısı koyuldu. idiyopatik pulmoner hemosiderozis genellikle infantlarda veya birinci dekadda görülen bir tablodur ue etyolojisi bilinmemektedir. Erişkinlerde nadir olup 1-17 yaş arasında sıktır. Klinik prezentasyon; anemi, öksürük, dispneyi içeren sinsi bir başlangıçtan, tekrarlayan akut hemoptizi ile karakterize fulminan bir tabloya kadar değişebilmektedir. Hastalığın kesin tanısı için genellikle açık akciğer biyopsisi gerekmektedir.

İdiyopatik pulmoner hemosiderozis

Twentyseuen years old woman was admitted to our hospital with dyspnea, severe hemoptysis and iron deficiency anemia. The chest X-ray showed bilateral interstitial markings with homogenous infiltration at right costodiafragmatic sinus. The patient was investigated for all alveolar hemorrhagic syndromes. The diagnosis of Idiopathic pulmonary haemosiderosis (IPH) was made by open lung biopsy. IPH usually presents in infancy or within the first decade of life and is unknown aetiology. It is most common between ages 1-17 and exceedingly rare in adults. Clinical presentation of IPH varies from an in-sidious onset with anemia, cough, dyspnea to a fulminant onset with recurrent acute hemoptysis. Histological confirmation with open lung biopsy is often necessary for definite diagnosis.

PDF

___

1. loachimescu OC, Sieber S, Kotch A. Idiopathic pulmo- nary haemosiderosis revisited. Eur Respir J 2004; 24: 162-70.
2. Akyar S, Ozbek SS. Computed tomography findings in idi- opathic pulmonary hemosiderosis. Respiration 1993; 60: 63-4.
3. Saeed MM, Woo SM, Mac Laughlin EF, et al. Prognosis in pediatric idiopathic pulmonary hemosiderosis. Chest 1999; 116: 721-5.
4. Frankel LR, Smith DW, Pearl RG, Leviston NJ. Nitro- glycerine responsive pulmonary hypertension in idi- opathic pulmonary hemosiderosis. Am Rev Respir Dis 1986; 133: 170-2.
5. Ramirez T, Prados C, Gomez de Terroros Caro J, et al. İdi- opathic pulmonary hemosiderosis in a patient of advan- ced age. Ach Bronchopneumol 1999; 35: 507-9.
6. Michaeli J, Kornberg A, Menashe M, et al. Exacerbation of idiopathic pulmonary hemosiderosis in pregnancy. Eur J Obstet Reprod Biol 1987; 24: 153-8.
7. Louie S, Russell LA, Richeson RB, Cross CE. Circulating immune complexes with pulmonary haemorrhage during pregnancy in idiopathic pulmonary haemosiderosis. Chest 1993; 104: 1907-9.
8. Sargel KH, Sommers SC. İdiopathic pulmonary haemosiderosis and related syndromes. Am J Med 1962; 32:499-511.
9. Leatherman JW. Immune alveolar hemorrhage. Chest 1987; 91: 891-7.
10. Albelda SM, Gefter WB, Ebstein DM, Miller WT. Diffuse pulmonary hemorrhage: a review and classification. Radiology 1985; 154:289-97.
11. Leatherman JW, Davies SF, Hoidal JR. Alveolar hemorhage syndromes: microvascular lung hemorrhage in immune and idiopathic disorders. Medicine 1984; 63: 343-61.
12. Airaghi L, Ciceri L, Giannini S, et al. İdiopathic pulmonary hemosiderosis in adult. Favourable response to azathioprine. Monaldi Arch Chest Dis 2001; 56:211-3.