Simultaneous Occurrence of Rosai–Dorfman Disease and Nodal Marginal Zone Lymphoma in a Patient with Sjögren’s Syndrome

Abstract

We present an exceptionally rare case of co-occurrence of Rosai–Dorfman disease (RDD) and nodal marginal zone lymphoma (NMZL) in a 60-year-old Caucasian female with a 20-year course of Sjögren's syndrome (SS). In response to treatment for lymphoma, the patient presented a short positive response, followed by a rapid progression of the disease accompanied by the development of the peripheral facial nerve palsy. We failed to detect Epstein–Barr virus (EBV) in the NMZL/RDD sample by EBV-encoded RNA (EBER) in situ hybridization but identified genomic DNA of EBV by polymerase chain reaction. A second biopsy revealed EBV-positive diffuse large B-cell lymphoma (DLBCL), not otherwise specified. The identical clonal immunoglobulin heavy chain gene rearrangements in the NMZL and DLBCL pointed to their clonal relationship. Though the role of EBV in the pathogenesis of some lymphomas is well-known, there have been only few cases of EBV-induced transformation of low-grade B-cell lymphoma into high-grade lymphoma and no cases of a patient with an NMZL background. To our knowledge, this is the first report of a concomitant occurrence of RDD and NMZL in a SS patient.

Rosai-Dorfman disease: an overview

“…4 Sporadic RDD is the most common form and includes the classic nodal form, extranodal RDD, neoplasia-associated RDD and immune disease-associated RDD. 4 Neoplasms associated with RDD include lymphomas, [25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42] leukaemias, [43][44][45] malignant histiocytoses, 46 47 LCH [48][49][50][51] and ECD 52 (see table 2). Systemic lupus erythematosus, idiopathic juvenile arthritis, autoimmune haemolytic anaemia and HIV are among the immune disorders that are associated with RDD.…”

Clinicopathological and Gene Mutation Analysis of 27 Cases with Extranodal Rosai–Dorfman Disease

“…Olmedo–Reneaum et al 6 reported a case of a person with HIV, RDD, and simultaneous infection with Salmonella and Mycobacterium avium who eventually died. Gorodetskiy et al 7 reported a case of a person with Sjögren’s syndrome accompanied by RDD and lymph node marginal region lymphoma in which the Epstein–Barr virus was detected in both the RDD and lymphoma tissues. It is suggested that viral infection may create an immune environment that ultimately leads to activation of the histiocyte–macrophage system or tumor.…”

Ocular Adnexal MALT Lymphoma in Association With Rosai–Dorfman Disease in a Child

“…Literature search disclosed 4 reported cases of RDD in association with MALT. 2,5,6,12 None of the 4 cases occurred in a pediatric patient or involved the orbit. While the 2 conditions can occur concurrently, each can also be located at different anatomical sites and develop at different time intervals.…”