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proLékaře.cz / Journals / Transfusion and Haematology Today / 2020 - 4

Necrobiotic xanthogranuloma associated with monoclonal gammopathy: case report and overview of therapeutic options

Czech version

Authors: Z. Král 1;  M. Krejčí 1;  J. Kynclová 2;  Z. Řehák 3;  R. Koukalová 3;  Š. Skorkovská 4;  L. Pour 1;  Z. Adam 1
Authors‘ workplace: Interní hematologická a onkologická klinika LF MU a FN Brno 1;  Patologický ústav LF MU a FN BRNO 2;  Oddělení nukleární medicíny, MOU Brno 3;  Oční klinika NeoVize Brno 4
Published in: Transfuze Hematol. dnes,26, 2020, No. 4, p. 310-319.
Category: Case Report

Overview

Necrobiotic xanthogranuloma is an uncommon non-Langerhans cell histiocytosis involving skin and extra cutaneous tissues. Necrobiotic xanthogranuloma is most frequently associated with MGUS, myeloma and may also occur in other lymphoproliferative disorders. Its pathogenesis remains unclear. Necrobiotic xanthogranuloma is usually a chronic and slowly progressive disease. Several treatments have been proposed and have yielded variable results. The aim of this case report and systematic review is to summarize all reported treatments of necrobiotic xanthogranuloma of the skin, with or without underlying malignant conditions. Treatment options include corticosteroids, thalidomide, high-dose intravenous immunoglobulins (IVIG), chlorambucil, cyclophosphamide, fludarabine, rituximab, melphalan, infliximab, cladribine, laser therapy, radiotherapy and other possibilities. The treatment options are at most only partially effective; the best response is achieved with IVIG therapy. In our case, treatment with cyclophosphamide, dexamethasone and bortesomib did not lead to any significant response. We then used IVIG and electron beam irradiation achieving a partial response and stabilization of this rare disease..

Keywords:

necrobiotic xanthogranuloma

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Dopisy redakci
Dopisy redakci
Za pani profesorkou MUDr. Adrienou Sakalovou, DrSc.
Zemřela MUDr. Zdeňka Vorlová, CSc.
Jubileum prof. MUDr. Vlastimila Ščudly, CSc.
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