Langerhans Cell Histiocytosis Successfully Treated with Combination of Thalidomide and Corticosteroid.

Langerhans cell histiocytosis (LCH) is rare disease characterised by clonal proliferation of Langerhans cells. The effectiveness of the current standard of treatment for LCH has not been proved in prospective trials, and 50% of patients treated with the current standard of treatment were unresponsive. Here we describe a case of a patient with LCH that was unresponsive after treatment with vinblastine and prednisolone but showed improvement after treatment with thalidomide and methylprednisolone. A 51-year-old woman was admitted to the haematology and medical oncology outpatient clinic due to an ulcerative skin lesion on the left auricular, axilla, inguinal and perineum regions. She was diagnosed with multi-organ LCH and was previously treated with vinblastine and prednisolone, but with a non-complete response. Her PET/ CT F-18 FDG body scan compared with previous one showed no resolution in abdominal nodes. There was interval progression in hypermetabolic skin thickening in the left external auditory canal, bilateral axilla and perineum, and mild interval increase in metabolic activity within the L4 vertebral lesion. She was given ultraviolet light phototherapy and continued with a chemotherapy regimen consisting of etoposide and methylprednisolone. After that she was treated with thalidomide 1 × 100 mg and methylprednisolone, which improved her skin condition. LEARNING POINTS: There is no gold standard of treatment for Langerhans cell histiocytosis (LCH) in adults.Around 50% of patients with LCH were unresponsive to the standard therapy and no standard regimen was recommended for patients with refractory LCH.A combination of thalidomide and corticosteroid may be a therapeutic option in patients with LCH.