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Successful Treatment of Recurrent Autoimmune Cytopenias in the Context of Sinus Histiocytosis With Massive Lymphadenopathy Using Sirolimus
Author(s) -
Cooper Stacy L.,
Arceci Robert J.,
Gamper Christopher J.,
Teachey David T.,
Schafer Eric S.
Publication year - 2016
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.25770
Subject(s) - medicine , context (archaeology) , sirolimus , autoimmune lymphoproliferative syndrome , sinus histiocytosis with massive lymphadenopathy , dermatology , discontinuation , histiocytosis , immunology , disease , paleontology , apoptosis , biochemistry , chemistry , fas receptor , programmed cell death , biology
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 © 2015 Wiley Periodicals, Inc.