Esra YAZAR, Selim KAHRAMAN, Akif ÖZGÜL, Nur BÜYÜKPINARBAŞI, Veysel YILMAZ

Pulmoner Langerhans Hücreli Histiyositozisli İki Olgu ve Literatür Derlemesi

Pulmoner langerhans hücreli histiyositozis PLHH etiyolojisi ve sıklığı tam olarak bilinmeyen nadir görülen bir akciğer parankim hastalığıdır. Biz de kliniğimizde tanı koyduğumuz biri 19 1. olgu diğeri 22 2. olgu yaşlarında iki erkek hastayı literatürler eşliğinde inceledik. Etiyolojide suçlanan sigara içiciliği her iki hastada da mevcuttu sırasıyla; 8 ve 30 paket/yıl . Birinci olguda nefes darlığı ön planda iken diğer olguda konstitüsyonel semptomlar ve hemoptizi ön plandaydı. Her iki olguda da DLCO düşüktü, ilk hastada restriktif tipte solunum fonksiyon bozukluğu varken diğerinde obstrüktif tipteydi ve akciğer volümlerinde belirgin artışla birlikteydi. Her iki hastanın da toraks bilgisayarlı tomografisi PLHH radyolojisi ile uyumluydu. İlk hastaya transbronşiyal biyopsi örneklerinin immünhistokimyasal boyaması ile tanı konulurken diğer hastada bronko-alveoler lavajda CD-1a pozitif langerhans hücre oranının %5’in üzerinde olması ile tanı konuldu. Her iki hastada da yaşları ve radyolojilerinin tipik olması nedeniyle daha az invaziv işlemler tercih edilerek tanı konuldu. Sonuç olarak PLHH’nin tipik klinik ve radyolojik bulguları olan hastalarda tanıda açık akciğer biyopsisine gerek duyulmamaktadır

Two Cases With Pulmonary Langerhans' Cell Histiocytosis and Review of The Literature

The etiology and frequency of pulmonary langerhans’cell histiocytosis PLCH is not exactly known and it is a rare pulmonary parenchymal disease. We investigated two cases first case aged 19 yr male, second case aged 22 yr male which we have diagnosed in our clinic. Both of the patients were smoker as evidence implicating tabacco smoke in the etiology 8p/year and 30p/year, respectively . The presentation symptom in the first case was dyspnea and in the other case was constitutional symptoms and hemoptysis. The DLCO was low in both cases. Whereas in the first case a restrictive pattern of pulmonary function disorder was observed in the second case there was an obstructive pattern with incrased lung volumes. Appearence of the thorax computed tomography of both patients was consistent with PLCH. The diagnosis was made by immunohistochemical staining of transbronchial biopsy and by high CD-1a positive langerhans’ cell ratio 5% in bronchoalveolar lavage in the first and second case, respectively. The diagnosis of both patients was made with less invasive procedure due to the typically radiologic findings and age. An open lung biopsy is not necessary in patients with typically clinic and radiologic findings

Keywords:

pulmonary langerhans’cell histiocytosis, radiologic manifestation, diagnosis,

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