Adult-Onset Langerhans Cell Histiocytosis And The Role of Vinblastine In The Treatment -Ankara Oncology Hospital Experience

INTRODUCTION: Langerhans cell histiocytosis is a group of diseases characterized by clonal neoplastic proliferation of atypical histiocytes in various tissues such as bone, pituitary gland, lymph nodes, lungs and central nervous system. The aim of this study was to evaluate in terms of clinical, laboratory and survival retrospectively and to examine the role of vinblastine chemotherapy in treatment.
METHODS: Eight cases included in our study between May 2011 and September 2019 and evaluated retrospectively.
RESULTS: The mean age of eight patients is 37 (youngest 19-largest 75). The male / female ratio is 7/1. The most common complaint of patients was bone pain at the rate of 50%. In clinical staging, 62.5% patients had single system involvement, while 37.5% had multi-system involvement. In 60% of patients with a single system involvement, it was assessed in high risk class like central nervous system invasion due to location of involvement. Surgical resection for diagnostic purposes was also evaluated as a treatment in 25% patients with a single system involvement. As chemotherapy, vinblastine+ methylprednisolone treatment was preferred in 37.5% of the firt line treatment and 12.5% was fort he second line treatment. Radiotherapy has been used in the treatment of 50% of patients.Induction of a patient with pituitary involvement was performed with 4 cycles of cladribine. Median follow-up is 59 months (minimum 5 months - maximum 100 months).
DISCUSSION AND CONCLUSION: The clinical course of Adult-onset Langerhans cell histiocytosis is relatively well and can be treated with resection, radiotherapy of involvement area, systemic chemotherapy. Vinblastine and steroid therapy is effective and safe in the first line of systemic therapy.

Eriþkin Baþlangýçlý Langerhans Hücreli Histiyositozis Tedavisinde Vinblastin Kemoterapisinin Yeri – Ankara Onkoloji Hastanesi Deneyimi

GÝRÝÞ ve AMAÇ: Langerhans hücreli histiyositozis atipik histiyositlerin lokal veya yaygýn olarak kemik, hipofiz bezi, lenf nodlarý,akciðerler ve merkezi sinir sistemi gibi çeþitli dokularda klonal neoplastik çoðalmasý karakterize bir hastalýk grubudur. Merkezimizde takip edilen sekiz LHH olgusu klinik, laboratuvar ve saðkalým açýsýndan retrospektif olarak deðerlendirilmesi ve tedavide vinblastin kemoterapisinin rolünün incelenmesi amaçlanmýþtýr.
YÖNTEM ve GEREÇLER: Mayýs 2011 – Eylül 2019 tarihleri arasýnda takipli 8 hastanýn verileriler retrospektif olarak deðerlendirilmiþtir.
BULGULAR: Sekiz hastanýn ortalama yaþý 37 (en küçük 19-en büyük 75) dir. Erkek / kadýn oraný 7/1’dir. Hastalarýn en sýk baþvuru þikayeti %50 oranýnda kemik aðrýsýydý. Hastalarýn klinik evrelemesinde %62.5 ‘sinin tek sistem tutulumu olduðu; %37.5’inin multi-sistem tutulumu saptandý. Tek sistem tutulumlu hastalarýn %60’ýnda tutulum yeri nedeniyle santral sinir sistemi invazyonu açýsýndan riskli bölgelere yerleþtiði görülerek yüksek risk sýnýfýnda deðerlendirildi. Taný amaçlý cerrahi rezeksiyon, tek sistem tutulu %25 hastada tedavi olarak da deðerlendirilmiþtir. Kemoterapi olarak ilk basamakta %37.5 hastada, %12.5 hastada ikinci basamakta vinblastin + metilprednizolon tedavisi tercih edilmiþtir. Radyoterapi %50 hastanýn tedavisinde kullanýlmýþtýr. Hipofizer tutulumlu bir hastamýza indüksiyonu 4 kür kladribin ile yapýlmýþtýr. Tüm hastalar tam yanýtla remisyonda takiptedir Median takip süresi 59 ay ( en az 5 ay – en çok 100 ay ) ‘dýr.
TARTIÞMA ve SONUÇ: Eriþkin baþlangýçlý LHH nispeten iyi seyretmekte olup rezeksiyon, tutulu alan radyoterapisi, sistemik kemoterapi tedavi edilebilmektedir. Vinblastin ve steroid tedavisi sistemik tedavide ilk basamakta etkin ve güvenlidir.