Haematologica (Feb 2020)

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

DOI
https://doi.org/10.3324/haematol.2019.219626
Journal volume & issue
Vol. 105, no. 2

Abstract

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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.