Ersin GÜNAY, Göktürk FINDIK, Yetkin AĞAÇKIRAN, Sadi KAYA, Koray AYDOĞDU, İrfan TAŞTAPE, Nurettin KARAOĞLANOĞLU, Sibel GÜNAY

Pulmonary Langerhans cell histiocytosis; characteristics of 11 cases

Giriş: Pulmoner Langerhans hücreli histiyositozis (PLHH) genç popülasyonun nadir görülen bir hastalığıdır. Hastaların hemen hemen hepsi sigara içicisidir. Bu çalışmada, PLHH tanılı 11 olgunun özelliklerini, tanısını, tedavisini ve prognozunu değerlendirmeyi amaçladık.Materyal ve Metod: Patolojik olarak PLHH tanısı konulan 11 olgu serisi tarandı. Olguların ortanca yaşı 35 (19-51) ve erkek/kadın oranı 5/6 idi. Hastaların hepsi semptomatikti. En sık semptomlar dispne (%81.8) ve kuru vasıflı öksürük (%72.7) idi. Ortalama semptom süresi 10.8 ay idi. İki olgu dışında tüm olgular (%81.8) aktif sigara kullanıcısıydı. Tüm olguların pasif tütün maruziyeti mevcuttu.Bulgular: Bilateral kistik görünüm (n= 9, %81.8), interstisyel bulgular [septal ve peribronkovasküler kalınlaşma (%72.7) ve nodüler patern (%54.5)] en sık radyolojik bulgulardı. Spontan pnömotoraks iki olguda saptandı. Hastalara cerrahi biyopsi (%90.9) veya transbronşiyal biyopsi (%9.1) ile tanı konuldu. Sigara bırakma ve immünsüpresif tedavi (metilprednizolon) hastalara önerilen tedavilerdi. Ortalama takip süresi 5.40 ± 1.78 yıl idi. Semptomlar sigara bırakma veya metilprednizolon tedavisiyle geriledi. Olgulardan sadece bir tanesi takipte tekrarlayan pnömotoraks nedeniyle hastaneye başvurdu.Sonuç: PLHH gelişimden aktif sigara kullanımı yanında pasif içicilik de sorumludur. PLHH tedavisi için birkaç öneri dışında tam bir fikir birliği mevcut değildir. Bu nadir hastalığın patogenezinin anlaşılmasıyla yeni tedavi yöntemleri gelişebilecektir.

Pulmoner Langerhans hücreli histiyositozis; 11 olgunun özellikleri

Introduction: Pulmonary Langerhans cell histiocytosis (PLCH) is a rarely seen disease of younger population. Almost all of the patients were smoker. In this study we aimed to evaluate the characteristics, diagnosis, treatment modalities and prognosis of 11 cases with PLCH.Materials and Methods: We retrospectively reviewed our case series of eleven patients who were pathologically diagnosed as PLCH. The median age was 35 years (19-51) and male to female ratio (M/F) was 5/6. All of the patients were symptomatic. The most common symptoms were dyspnea (81.8%) and dry cough (72.7%). Mean duration of the symptoms was 10.8 months. All patients except two of them were smoker (81.8%). All patients were also passive smokers.Results: Bilateral cystic appearance (n= 9, 81.8%), interstitial findings [septal and peribronchovascular thickening (72.7%) and nodular pattern (54.5%)] were common radiological findings. Spontaneous pneumothorax was present in two cases. All patients were diagnosed with surgical biopsies (90.9%) or transbronchial parenchymal biopsy (9.1%). Smoking cessation (81.8%) and immunosupression therapy (methylprednisolone) were the treatment modalities. Mean follow-up period was 5.40 ± 1.78 years. Generally, symptoms were improved with smoking cessation or methylprednisolone therapy. One patient was readmitted to our clinic with recurrent pneumothorax. In conclusion, it should be kept in mind that passive smoking is also responsible in the pathogenesis of PLCH.Conclusion: Exact consensus for PLCH treatment was not present except a few recommendations. In the future, with the understanding of the pathogenesis of the disease, new therapeutic agents will be discovered for this rare condition.

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