Vnitr Lek 2021, 67(3):157-164 | DOI: 10.36290/vnl.2021.037

Therapy of 3 patients with Erdhiem-Chester disease with cladribin or cladribin in combination with cyclophosphamide. Case report and review of the therapy

Zdeněk Král1, Zdeněk Řehák2, Marta Krejčí1, Renata Koukalová2, Sabina Ševčíková3, Luděk Pour1, Martin Krejčí1, Martin Štork1, Viera Sandecká1, Zdeněk Adam1
1 Interní hematologická a onkologická klinika, LF MU a FN Brno
2 Oddělení nukleární medicíny, Masarykův onkologický ústav Brno
3 Ústav patologické fyziologie, LF MU a FN Brno

Three adult patients with confirmed Erdheim-Chester disease (ECD) are followed at our department. Cladribine in monotherapy or in combination with cyclophosphamide were used for first line therapy. The median number of cycles of cladribine or cladribine and cyclophosphamide was 7 (range 6-8). In two cases complete response was achieved, in one case this therapy achieved no response. The duration of response is in one case 11 years, in second case the follow up is too short for evaluation of response duration. In case of no-response to cladribine and cyclophosphamide stabilisation of disease was achieved with anakinra. The tolerance was good without any toxicity grade II and higher. Cladribin and cyclophosphamide is one option for treatment of Erdheim-Chester disease.

Keywords: Erdheim-Chester disease, kladribin, anakinra.

Published: May 26, 2021  Show citation

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Král Z, Řehák Z, Krejčí M, Koukalová R, Ševčíková S, Pour L, et al.. Therapy of 3 patients with Erdhiem-Chester disease with cladribin or cladribin in combination with cyclophosphamide. Case report and review of the therapy. Vnitr Lek. 2021;67(3):157-164. doi: 10.36290/vnl.2021.037.
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