TY - JOUR
T1 - Clinicopathological features, treatment approaches, and outcomes in Rosai-Dorfman disease
AU - Goyal, Gaurav
AU - Ravindran, Aishwarya
AU - Young, Jason R.
AU - Shah, Mithun V.
AU - Bennani, N. Nora
AU - Patnaik, Mrinal M.
AU - Nowakowski, Grzegorz S.
AU - Thanarajasingam, Gita
AU - Habermann, Thomas M.
AU - Vassallo, Robert
AU - Sher, Taimur
AU - Parikh, Sameer A.
AU - Rech, Karen L.
AU - Go, Ronald S.
N1 - Publisher Copyright:
© 2020 Ferrata Storti Foundation. All rights reserved.
PY - 2020/1/31
Y1 - 2020/1/31
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85078815134&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85078815134&partnerID=8YFLogxK
U2 - 10.3324/haematol.2019.219626
DO - 10.3324/haematol.2019.219626
M3 - Article
C2 - 31004029
AN - SCOPUS:85078815134
SN - 0390-6078
VL - 105
SP - 348
EP - 357
JO - Haematologica
JF - Haematologica
IS - 2
ER -