Léčba tří pacientů s Erdheimovou-Chesterovou chorobou kladribinem, případně kombinací kladribinu a cyklofosfamidu a přehled léčby této nemoci
[Therapy of 3 patients with Erdhiem-Chester disease with cladribin or cladribin in combination with cyclophosphamide. Case report and review of the therapy]
Language Czech Country Czech Republic Media print
Document type Case Reports, Journal Article
PubMed
34171955
PII: 127307
- Keywords
- Erdheim-Chester disease, anakinra, cladribine, kladribin,
- MeSH
- Interleukin 1 Receptor Antagonist Protein MeSH
- Cyclophosphamide MeSH
- Adult MeSH
- Erdheim-Chester Disease * MeSH
- Remission Induction MeSH
- Cladribine * MeSH
- Humans MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
- Names of Substances
- Interleukin 1 Receptor Antagonist Protein MeSH
- Cyclophosphamide MeSH
- Cladribine * MeSH
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.