Özet:

Aim: Langerhans cell histiocytosis (LCH) is a rare disease that can affect all organs and systems and cause a wide variety of clinical findings. Treatment and prognosis vary according to site and risk of organ involvement. In this study, we aimed to evaluate the patients who were followed up with diagnosis of LCH in our clinic. Materials and methods: In the study, data of 24 patients diagnosed with LCH between January 2000 and December 2019 were retrospectively analyzed. The patients were evaluated in terms of age at diagnosis, gender, initial symptoms, organ involvement, treatments, follow-up period, and outcome. Results: The mean age at diagnosis was 4.7±4.6 (0.6-16.6) years, and the mean follow-up time was 91.6±67.8 (12.1-240) months. While the most common symptom at the time of diagnosis was skin lesions, the most common organ involvements were bone and skin. This was followed by the pituitary, liver, lymph node, lung, spleen, periodontal region, thyroid, orbital region, mastoid, pancreas, and bone marrow, respectively. Patients were treated in accordance with the LCH-III study protocol according to risk groups. Cladribine treatment was applied to 5 of 6 patients who progressed, and hematopoietic stem cell transplantation (HSCT) was applied to 1 patient, and complete response was obtained in all of them. Conclusion: Depending on the organs and systems involved in LCH and the extent of the disease; chemotherapy, radiotherapy and surgical treatment options are applied in different combinations. In cases of unresponsiveness or progression, cladribine and HSCT treatments are successfully applied, as in our series.

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