Abstract

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.