Haematologica, Vol 91, Issue 12_Suppl, ECR52-ECR52
Copyright © 2006 by Ferrata Storti Foundation
Dramatic clinical efficacy of cladribine in Rosai-Dorfman disease and evolution of the cytokine profile: towards a new therapeutic approach
A Aouba,
B Terrier,
V Vasiliu,
S Candon,
N Brousse,
B Varet,
and
O Hermine
Department of Adult Hematology, Hopital Necker Enfants-Malades, Paris V University, Assistance Publique-Hopitaux de Paris (AP-HP), 49- 161 rue de Sevres, 75743 Paris Cedex 15, France.
Rosai-Dorfman disease (RDD) is a rare disorder, often benign but with possible life-threatening prognosis. In most cases, specific treatment is not necessary; when required, the management of RDD is not codified to date, and various chemotherapies have been shown to be ineffective. Here, we report a patient with RDD who presented a dramatic and sustained response with cladribine. Analysis of the cytokine profile evolution shows a clear correlation between serum levels of TNF-alpha and IL-6 and disease activity. Our findings show the promising efficacy of cladribine and suggest that therapies targeting specifically cytokines might be useful in some cases of active RDD.
