title: Dramatic clinical efficacy of cladribine in Rosai-Dorfman disease and evolution of the cytokine profile: towards a new therapeutic approach.
abstract: 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.
authors.affiliation: Department of Adult Hematology, Hôpital Necker Enfants-Malades, Paris V University, Assistance Publique-Hôpitaux de Paris (AP-HP), 49- 161 rue de Sèvres, 75743 Paris Cedex 15, France.
authors.forename: Achille
authors.initials: A
authors.lastname: Aouba
authors.forename: Benjamin
authors.initials: B
authors.lastname: Terrier
authors.forename: Viorel
authors.initials: V
authors.lastname: Vasiliu
authors.forename: Sophie
authors.initials: S
authors.lastname: Candon
authors.forename: Nicole
authors.initials: N
authors.lastname: Brousse
authors.forename: Bruno
authors.initials: B
authors.lastname: Varet
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
mesh_terms: D000963:Antimetabolites / Q000627:therapeutic use*; D017338:Cladribine / Q000627:therapeutic use*; D016207:Cytokines / Q000097:blood*; D005260:Female; D015618:Histiocytosis, Sinus / Q000097:blood / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D015850:Interleukin-6 / Q000097:blood; D008875:Middle Aged; D012074:Remission Induction; D014409:Tumor Necrosis Factor-alpha / Q000032:analysis
chemical_list: D000963:Antimetabolites; D016207:Cytokines; D015850:Interleukin-6; D014409:Tumor Necrosis Factor-alpha; D017338:Cladribine