An overview of the treatment of Langerhans cell histiocytosis in adult patients


Authors: Z. Adam;  M. Krejčí;  L. Pour;  P. Szturz;  R. Hájek
Authors‘ workplace: Interní hematoonkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Mayer, CSc.
Published in: Vnitř Lék 2010; 56(Supplementum 2): 131-140
Category: Langerhans cell histiocytosis and some other Hematology rare diseases

Overview

Treatment of Langerhans cell histiocytosis (LCH) is determined by the stage of the disease. The treatment of choice in a localized form of the disease is a surgery or local application of glucocorticoids. If skin only is affected: local glukocorticoids or tacrolimus, and, should this treatment be unsuccessful, systemic treatment with methotrexate or thalidomide or short-term PUVA therapy. A combined treatment with vinblastin and mercaptopurin with prednisone represents the first line treatment in patients with generalized disease; in case of the lack of efficacy of treatment involving vinblastin or in the case of initial treatment of aggressive forms, cladribine might be used as the first line treatment. Other cytostatics or, alternatively, high-dose chemotherapy with autologous or allogeneic transplantation may be used if this treatment is unsuccessful. Anecdotal evidence also suggests efficacy of other treatment modalities (anti-TNF, retinoids, cyclooxygenase, inhibitors, interferon α, imatinib) although this has not been proven in clinical studies. Bisphosphonates have been shown to be effective in patients with bone involvement.

Key words:
Langerhans cell histiocytosis – vinblastine – thalidomide – cladribine – bisphosphonate


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