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

Authors: Zdeněk Král 1;  Zdeněk Řehák 2;  Marta Krejčí 1;  Renata Koukalová 2;  Sabina Ševčíková 3;  Luděk Pour 1;  Martin Krejčí 1;  Martin Štork 1;  Viera Sandecká 1;  Zdeněk Adam 1
Authors‘ workplace: Interní hematologická a onkologická klinika, LF MU a FN Brno 1;  Oddělení nukleární medicíny, Masarykův onkologický ústav Brno 2;  Ústav patologické fyziologie, LF MU a FN Brno 3
Published in: Vnitř Lék 2021; 67(3): 157-164


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.


Erdheim-Chester disease – kladribin – anakinra


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