Pathology, Classification, Clinical Manifestations and Prognosis of Langerhan’s Cell Histiocytosis: A Single Center Experience

Abstract

Objective: The aim of the study is to raise awareness about clinical features, histopathological and radiological analyzes and treatment details of this rare disease. Methods: A total of 55 Langerhans cell histiocytosis patients, diagnosed between the years 2006 and October 2020 in our department were included in the study. The patients were evaluated in terms of age, gender, tumor localization, risk groups, treatment modalities, recurrence, and disease outcome. Results: Twenty-three out of 55 patients were children and 32 were adults. The ages of the patients were between 7 months and 72 years. Thirty-seven of the cases were male and 18 were female. The most common clinical complaint in both groups was pain and swelling. The duration between the onset of the patient complaints and admission to the hospital varies between 7 days-12 months in children, and 10 days-23 years in adults. Forty-three of the cases had single-organ involvement and 12 had multiorgan involvement. The most frequently affected organ in both groups was bone. Forty of the 55 patients had follow-up data and the treatment modalities are as follows: Nine patients radiotherapy, 8 patients chemotherapy+steroid, 7 patients chemotherapy, 2 patients chemotherapy+radiotherapy+steroid, 1 patient steroid, and 2 patients chemotherapy+radiotherapy. Eleven patients were followed up without additional treatment after surgery. Median follow-up from the time of biopsy was 45.9 months in children and 41.9 months in adults. Conclusions: As a result, diagnosis requires a high degree of suspicion and final diagnosis is based on the histological examination of the lesions and biopsies

Keywords

• Langerhans cell histiocytosis
• adult
• pathology
• children
• prognosis

Langerhans Hücreli Histiyositozun Patolojisi, Sınıflandırılması, Klinik Belirtileri ve Prognozu: Tek Merkez Deneyimi

Abstract

Amaç: Çalışmanın amacı, nadir görülen bu hastalığın; klinik özellikler, histopatolojik, radyolojik analizler ve tedavi detayları hakkında farkındalığı arttırmaktır. Materyal method: 2006 Ocak-2020 Ekim tarihleri arasında anabilim dalımızda tanı konan 55 Langerhans hücre histiyositozu hastası çalışmaya dahil edildi. Hastalar yaş, cinsiyet, lokalizasyon, risk grupları, tıbbi tedavi, nüks ve hastalığın sonuçları açısından değerlendirildi. Sonuçlar: 55 hastanın 23’ü çocuk, 32'si yetişkindi. Hastaların yaşları 7 ay ile 72 yıl arasında değişmektedir. Olguların 37’si erkek, 18'i kadındı. Her iki gruptada en sık şikâyet ağrı ve şişlikti. Hasta şikâyeti ile hastaneye başvuru süresi çocuklarda 7 gün ile 12 ay arasında değişirken, erişkinlerde 10 gün ile 23 yıl arasında değişmektedir. Olguların 43'ünde tek organ tutulumu, 12'sinde multiorgan tutulumu vardı. Yetişkinlerde ve çocuklarda en sık etkilenen organ kemikti. Takipli hastalar tedavi açısından incelendiğinde: 9 olgu radyoterapi, 8 olgu kemoterapi + steroid, 7 olgu kemoterapi, 2 olgu kemoterapi + radyoterapi + steroid, 1 olgu sadece steroid, 2 olgu kemoterapi + radyoterapi ve onbir olgu ise cerrahi sonrası ek tedavi gerekmeksizin takip edildi. Biyopsiden sonra medyan takip süresi çocuklarda 45.9 ay ve erişkinlerde 41.2 ay idi. Sonuç olarak tanı için yüksek derecede şüphe gerektiren hastalıkta, kesin tanı lezyonların ve biyopsilerin histolojik incelemesine dayanmaktadır.

Keywords

• Langerhans hücre histiyositozu
• erişkin
• patoloji
• çocuk
• prognoz

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