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proLékaře.cz / Journals / Transfusion and Haematology Today / 2020 - 2

Treatment of Langerhans cell histiocytosis in adults

Czech version

Authors: Z. Král 1;  Z. Adam 1;  M. Doubková 2;  M. Krejčí 1;  T. Horvát 3;  M. Doubek 1;  L. Pour 1;  Z. Řehák 4,5;  R. Koukalová 4
Authors‘ workplace: Interní hematologická a onkologická klinika LF MU a FN Brno 1;  Klinika nemocní plicních a TBC LF MU a FN Brno 2;  Chirurgická klinika LF MU a FN Brno 3;  Oddělení nukleární medicíny, Masarykův onkologický ústav, Brno 4;  Regionální centrum aplikované molekulární onkologie (RECAMO), Masarykův onkologický ústav, Brno 5
Published in: Transfuze Hematol. dnes,26, 2020, No. 2, p. 101-112.
Category: Review/Educational Papers

Overview

Langerhans cell histiocytosis has diverse clinical manifestations and the aggressiveness of its course varies. As in the case of other malignant diseases, involvement of other organs must be determined once the diagnosis has been made.  Local treatment may suffice in the case of a single lesion (surgery, instillation of methylprednisolone within the lesion). Skin involvement responds to phototherapy (PUVA or electron beam irradiation). Remissions following the administration of thalidomide or methotrexate have been described in cases of isolated skin involvement.  In adults, 2-cholorodeoxyadenosine (cladribine) in monotherapy and less often in combination with cytosine-arabinoside is the treatment of choice in the case of multi-system involvement. Extremely aggressive forms may be treated with combined chemotherapy regimens that include etoposide as in the case of lymphomas. In resistant cases, patients may be referred to transplant centres and undergo high-dose chemotherapy with autologous or allogeneic stem cell transplantation. Patients with the BRAF V600E gene mutation have been shown to respond to vemurafenib or dabrafenib, which are registered in the Czech Republic for the treatment of disseminated melanoma. On demonstrating this mutation and documenting resistance to prior therapy, it would be possible to request reimbursement of these agents from the health insurance company. 

Keywords:

Langerhans cell histiocytosis – 2-cholorodeoxyadenosine (cladribine) – vemurafenib – dabrafenib

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