TY - JOUR T1 - The Relationship of Prognostic Factors with Regulatory T Cells in Langerhans Cell Histiocytosis TT - Langerhans Hücreli Histiositozda Prognostik Önemi Olan Faktörlerin Düzenleyici T Hücreleri ile İlişkisi AU - Çoban, Ganime AU - Coşanay Tekden, Büşra AU - Güler, Beril AU - Çakır, Fatma Betül AU - Toluk, Özlem AU - Gucın, Zuhal AU - Elagöz, Şahande PY - 2023 DA - December Y2 - 2023 DO - 10.55694/jamer.1391740 JF - Journal of Anatolian Medical Research JO - JAMER PB - Kayseri Şehir Eğitim ve Araştırma Hastanesi WT - DergiPark SN - 2587-1153 SP - 60 EP - 66 VL - 8 IS - 4 LA - en AB - Aim: Langerhans cell histiocytosis (LCH), is a clonal disorder characterized by abnormal proliferation of dendriticcells, which has an intense inflammatory microenvironment. There is limited information about contribution ofmicroenvironment to this rare disease. We aimed to compare regulatory T cells in microenvironment and BRAFV600Emutation with prognostic data.Material and Methods: Overall, 26 cases were included to the study. The number FOXP3+ regulatory T cell (Treg)and presence of BRAFV600E mutation were assessed according to age, gender, localization, unifocal or multifocal,involvement of organ at risk, and recurrence status in a histochemical manner.Results: The number of adult cases was higher than pediatric cases. Bone was the most common localization, and 81%of cases were unifocal. Risk organ involvement was observed in 3 cases, 2 of which showed recurrence. It was found thatthe number of FOXP3+ Tregs was higher in adults, those with unifocal localization, and those with bone involvement.In addition, the number of FOXP3+ Tregs was higher in the group with recurrence than those without recurrence.BRAFV600E mutation was higher in children when compared to adults (p=0.003), but, no significant correlation wasfound when compared with remaining prognostic parameters (p>0.05).Conclusion: Although BRAFV600E mutation is more common in pediatric patients, it can also be seen in adult patients.The number of FOXP3+ Tregs is proportional to CD3+ and CD4+ cells. T cells, which present at varying rates inmicroenvironment, play an essential role in the pathogenesis of LCH. KW - Langerhans KW - KW - histiocytosis KW - KW - regulatory KW - KW - FOXP3 KW - KW - BRAF KW - KW - mutation N2 - Amaç: Langerhans hücreli histiositoz (LHH), dentritik hücrelerin anormal proliferasyonu ile karakterize klonal birhastalık olup, yoğun inflamatuar bir mikroçevreye (TM) sahiptir. TM’nin, nadir görülen bu hastalığa olan katkısı hakkında bilgiler sınırlıdır. Bu çalışmada, TM deki regülatuar T hücreleri ve BRAFV600E mutasyonunu prognostik veriler ilekarşılaştırmayı amaçladık.Gereç ve Yöntemler: Çalışmaya 26 olgu dahil edildi. Olguların yaş, cinsiyet, lokalizasyon, tekli-çoklu lokalizasyon,riskli organ tutulumu ve nüks durumlarına göre immünohistokimyasal olarak FOXP3+ regülatuar T hücre (Treg) sayısıve BRAFV600E mutasyon varlığı değerlendirildi.Bulgular: Çalışmada yetişkin olgu sayısı çocuk sayısından daha fazla idi. En sık lokalizasyon kemik olup, olguların%81’i tek lokalizasyonlu idi. Riskli organ tutulumu ise 3 olguda mevcut olup, bunlardan ikisinde nüks görüldü. FOXP3+Treg hücrelerinin sayısı; yetişkinlerde, tek lokalizasyon tutulumu olanlarda ve kemik tutulumu olanlarda yüksek olarakbulundu. Ayrıca nüks olan grupta FOXP3+ hücre sayısı nüks olmayan gruba göre daha yüksekti. BRAF V600E mutasyonuçocuklarda yetişkinlere oranla daha yüksekti (p=0.003), fakat prognostik parametreler ile karşılaştırıldığında istatikselolarak anlamlı sonuç bulunamadı (p>0.05).Sonuç: BRAFV600E mutasyon varlığı çocuk hastalarda daha sık olmakla birlikte yetişkin hastalarda da görülmektedir.FOXP3+ Treg sayısı CD3+ ve CD4+ T hücre sayıları ile orantılıdır. TM de yer alan farklı oranlara sahip T hücreleriLHH patogenezinde önemli yere sahiptir CR - Mitchell J, Jenée M., Stuart P. Berzins, Kannourakis G.. A potentially important role for T cells and regulatory T cells in Langerhans cell histiocytosis. Clinical Immunology 2018; 194: 19-25. CR - Tong C, Jia X, Jia Y, He Y. 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