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: Stacy L Cooper, Robert J Arceci, Christopher J Gamper, David T Teachey, Eric S Schafer
• Journal: Pediatric blood & cancer (Pediatr Blood Cancer) Vol. 63 Issue 2 Pg. 358-60 (Feb 2016) ISSN: 1545-5017 [Electronic] United States
• PMID: 26452062 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © 2015 Wiley Periodicals, Inc.

Chemical References

• Immunosuppressive Agents
• Sirolimus

Topics

• Autoimmune Diseases (complications, drug therapy)
• Child, Preschool
• Histiocytosis, Sinus (complications, drug therapy)
• Humans
• Immunosuppressive Agents (therapeutic use)
• Male
• Sirolimus (therapeutic use)