title: Successful Treatment of Recurrent Autoimmune Cytopenias in the Context of Sinus Histiocytosis With Massive Lymphadenopathy Using Sirolimus.
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.
authors.affiliation: Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Stacy L
authors.initials: SL
authors.lastname: Cooper
authors.affiliation: Department of Pediatrics, University of Arizona College of Medicine, Phoenix, Arizona.
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Arceci
authors.affiliation: Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Gamper
authors.affiliation: Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: David T
authors.initials: DT
authors.lastname: Teachey
authors.affiliation: Department of Pediatrics, Baylor College of Medicine, Houston, Texas.
authors.forename: Eric S
authors.initials: ES
authors.lastname: Schafer
mesh_terms: D001327:Autoimmune Diseases / Q000150:complications / Q000188:drug therapy*; D002675:Child, Preschool; D015618:Histiocytosis, Sinus / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D020123:Sirolimus / Q000627:therapeutic use*
chemical_list: D007166:Immunosuppressive Agents; D020123:Sirolimus
keywords: Rosai-Dorfman disease; autoimmune cytopenia; sirolimus