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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.
AuthorsM Tasso, C Esquembre, E Blanco, C Moscardó, M Niveiro, A Payá
JournalPediatric blood & cancer (Pediatr Blood Cancer) Vol. 47 Issue 5 Pg. 612-5 (Oct 15 2006) ISSN: 1545-5009 [Print] United States
PMID16302214 (Publication Type: Case Reports, Journal Article)
CopyrightCopyright (c) 2005 Wiley-Liss, Inc.
Chemical References
  • Cladribine
Topics
  • Child
  • Cladribine (therapeutic use)
  • Follow-Up Studies
  • Histiocytosis, Sinus (diagnosis, drug therapy)
  • Humans
  • Male
  • Remission Induction
  • Treatment Outcome
  • X-Rays

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