title: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) treated with 2-chlorodeoxyadenosine.
abstract: Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease (RDD) is a rare but well-defined histiocytic proliferative disorder of unknown etiology that usually presents with cervical lymphadenopathy, fever, leukocytosis, and hypergammaglobulinemia in an otherwise healthy child. Although many patients undergo spontaneous remission, a subset of patients with systemic disease has a more serious course. For those patients with a poor outcome, steroids and chemotherapeutic agents such as etoposide or 6-mercaptopurine plus low dose methotrexate have been used. We present a child with a massive cervical lymphadenopathy treated with 2-chlorodeoxyadenosine (2-CdA, cladribine) after other approaches failed.
authors.affiliation: Division of Pediatric Oncology, Hospital General Universitario de Alicante, Alicante, Spain. tasso_mar@gva.es
authors.forename: M
authors.initials: M
authors.lastname: Tasso
authors.forename: C
authors.initials: C
authors.lastname: Esquembre
authors.forename: E
authors.initials: E
authors.lastname: Blanco
authors.forename: C
authors.initials: C
authors.lastname: Moscardó
authors.forename: M
authors.initials: M
authors.lastname: Niveiro
authors.forename: A
authors.initials: A
authors.lastname: Payá
mesh_terms: D002648:Child; D017338:Cladribine / Q000627:therapeutic use*; D005500:Follow-Up Studies; D015618:Histiocytosis, Sinus / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D008297:Male; D012074:Remission Induction; D016896:Treatment Outcome; D014965:X-Rays
chemical_list: D017338:Cladribine