Management and outcomes of sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman Disease)

Abstract

Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman Disease [RDD]), is a rare, benign but clinically heterogeneous histiocytic disorder. Our aims were to analyze the clinical characteristics of the disease and explore the outcomes of patients with RDD followed at our institution. Between January 2000 and February 2019, there were 15 patients with a pathologically confirmed diagnosis of RDD. Median age at diagnosis was 48 years old (range 26–78). The majority (87%, n = 13) of the patients had extranodal disease. Frontline approaches included surgical intervention/complete excision (n = 5, 33%), rituximab monotherapy (n = 5, 33%), observation (n = 3, 20%), and radiation (n = 2, 13%). Two of the five patients underwent surgical excision and were subsequently treated with rituximab. Of the 7 patients who were given rituximab, 64% remained progression free 24 months after the initial rituximab administration. Our review parallels previous reports and highlights rituximab as a favorable option for therapy if ineligible for surgery or radiation.

Keywords:

• Antibody-based immunotherapy
• immunotherapeutic approaches
• radiation

Acknowledgments

M.E.H receives research funding from Acerta Pharma and HOPA, and is on an advisory committee at AstraZeneca, Sanofi/Genzyme, and Janssen. J.P.P has a patent pending with Novartis. D.J.L receives research funding from Triphase, Takeda, Curis, is on an advisory committee for Celgene and Curis, consults for Curis, and is on speaker’s bureau for Seattle Genetics. A.M. receives research funding from Merck. S.D.N receives research funding from 47 (Forty Seven), Roche, Rafael, Millenium/Takeda, Debiopharm, Aileron, ATARA, Pharmacyclics, honoraria from Celgene, and is on the data safety monitoring committee for Merck.

Disclosure statement

No potential conflict of interest was reported by the authors.