Successful Treatment of Recurrent Autoimmune Cytopenias in the Context of Sinus Histiocytosis With Massive Lymphadenopathy Using Sirolimus

Stacy L. Cooper, Robert J. Arceci, Christopher J. Gamper, David T. Teachey, Eric S. Schafer

Research output: Contribution to journalArticlepeer-review

Abstract

Sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai–Dorfman disease (RDD), is a non-neoplastic, lymphoproliferative disorder that usually resolves spontaneously or with minimal conventional chemotherapy. Rarely, SHML can be associated with autoimmune findings. Such cases are often treatment resistant and have high rates of morbidity and mortality. We present a case of a patient with long-standing autoimmunity in the context of SHML, dependent on standard-treatment until he was transitioned to novel monotherapy with sirolimus. Sirolimus treatment resulted in a complete remission, now sustained after discontinuation of all treatments for over 23 months, with no observable long-term sequelae. Pediatr Blood Cancer

Original languageEnglish (US)
Pages (from-to)358-360
Number of pages3
JournalPediatric Blood and Cancer
Volume63
Issue number2
DOIs
StatePublished - Feb 1 2016

Keywords

  • Rosai–Dorfman disease
  • autoimmune cytopenia
  • sirolimus

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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