Rosai-Dorfman disease: a report of eight cases in a tertiary care center and a review of the literature

Abstract

Rosai-Dorfman disease (RDD) is a nonmalignant histiocytic disorder of unknown origin that is extremely rare. By immunohistochemistry, the RDD cells are characteristically S-100 positive and CD1a negative. Emperipolesis is a common histopathological finding, although not specific for RDD. Lymph node and cutaneous manifestations are most frequent, but diverse organs can be affected. The clinical course is unpredictable regardless of treatment. Here, we present a series of 8 cases presenting lymph node and/or cutaneous lesions. Lymph node involvement was seen in diverse regions, including mediastinal and retroperitoneal. The treatment response to steroids was diversified, and the chemotherapy response was disappointing. Associated autoimmune diseases (Sjögren syndrome and antiphospholipid syndrome) were observed in 2 patients. Regardless of therapy modality, these patients exhibited a favorable prognosis in a follow-up duration that ranged from 15 to 80 months.

Cytomorphology and Histology Correlation of Rosai-Dorfman Disease: A 15-Year Study from a Tertiary Referral Centre in South India

“…This study showed concordance with studies done by Maia et al [13] and Shi et al [14] in terms of age distribution, male predominance, the most commonly presenting symptoms (fever and lymphadenopathy), and extranodal involvement ( table 3 ). Emperipolesis was identified on cytology in 60% of the cases in this study, 75% in the study by Maia et al [13] and all the cases of Shi et al [14] , and was more readily identified on histopathology.…”

“…Emperipolesis was identified on cytology in 60% of the cases in this study, 75% in the study by Maia et al [13] and all the cases of Shi et al [14] , and was more readily identified on histopathology. IHC was positive for CD68 and S100 and negative for CD1a in all the cases where IHC was studied and it showed similar concordance with the Maia et al [13] study. The single discordant case turned out to be Hodgkin lymphoma in our study and, similarly, 3 discordant cases were Hodgkin lymphoma in the study by Shi et al [14] .…”

Mutually exclusive recurrent KRAS and MAP2K1 mutations in Rosai–Dorfman disease

“…In complicated cases, radical resection, radiotherapy or methotrexate combined with other chemotherapeutic agents including 6-mercaptopurine or cladribine have been used, with different response rates. Standardized therapy for Rosai-Dorfman disease is yet to be established (41, 46–48). Others have shown promising results using the RAF inhibitor, vemurafenib, as targeted therapy in patients with BRAF V600E-mutated histiocytoses (49–51), which has been followed by advances in additional targeted agents for patients with refractory histiocytoses.…”

Enfermedad de Rosai-Dorfman. A propósito de un caso

“…[1][2][3][4][5][6][7] Su etiología es desconocida; se ha considerado como de origen neoplásico, inmune o relacionada con infecciones virales (Epstein-Barr, virus del herpes 6, parvovirus B19, citomegalovirus, varicela zóster). [1][2][3][4]7,8 La ERD es de inicio insidioso, autolimitada y tiene una fase activa prolongada con remisión espontánea y subsecuentes recaídas. 3,4,9 Se presenta con linfadenopatías indoloras, en general, cervicales, aunque también pueden ser inguinales, axilares y mediastinales.…”