Clinicopathological features, treatment approaches, and outcomes in Rosai-Dorfman disease

Gaurav Goyal, Aishwarya Ravindran, Jason R. Young, Mithun V. Shah, N. Nora Bennani, Mrinal M. Patnaik, Grzegorz S. Nowakowski, Gita Thanarajasingam, Thomas M. Habermann, Robert Vassallo, Taimur Sher, Sameer A. Parikh, Karen L. Rech, Ronald S. Go

Research output: Contribution to journalArticle

3 Scopus citations

Abstract

Rosai-Dorfman disease is a rare subtype of non-Langerhans cell histiocytosis. With the last major report published in 1990, there is a paucity of contemporary data on this disease. Our objective was to report the clinicopathological features, treatments and outcomes of patients seen at a tertiary referral center. Sixty-four patients with histopathological diagnosis of Rosai-Dorfman disease were identified from 1994 to 2017 (median age 50 years; range, 2-79). The median duration from symptom onset to diagnosis was seven months (range, 0-128), which was also reflected in the number of biopsies required to establish the diagnosis (median 2; range, 1-6). The most common presentation was subcutaneous masses (40%). Of the 64 patients, 8% had classical (nodal only) and 92% had extra-nodal disease (67% extra-nodal only). The most common organs involved were skin and subcutaneous tissue (52%), followed by lymph nodes (33%). Three patients had an overlap with Erdheim-Chester disease, which had not been described before. Two of these were found to have MAP2K1 mutations. Commonly utilized first line treatments were surgical excision (38%) and systemic corticosteroids (27%). Corticosteroids led to a response in 56% of the cases. Of those treated initially, 15 (30%) patients developed recurrent disease. The most commonly used systemic agent was cladribine (n=6), with 67% overall response rate. Our study demonstrates that Rosai- Dorfman disease has diverse clinical manifestations and outcomes. While this disease has been historically considered a benign entity, a subset of patients endures an aggressive course necessitating the use of systemic therapies.

Original languageEnglish (US)
Pages (from-to)348-357
Number of pages10
JournalHaematologica
Volume105
Issue number2
DOIs
StatePublished - Jan 31 2020

ASJC Scopus subject areas

  • Hematology

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