Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) treated with 2-chlorodeoxyadenosine.
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.
Tasso
M
M
Division of Pediatric Oncology, Hospital General Universitario de Alicante, Alicante, Spain. tasso_mar@gva.es
Esquembre
C
C
Blanco
E
E
Moscardó
C
C
Niveiro
M
M
Payá
A
A
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
D017338:Cladribine