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

Clinicopathological features, treatment approaches, and outcomes in Rosai-Dorfman disease. Haematologica. 2019 Apr 19;: Authors: Goyal G, Ravindran A, Young JR, Shah MV, Bennani NN, Patnaik MM, Nowakowski GS, Thanarajasingam G, Habermann TM, Vassallo R, Sher T, Parikh SA, Rech KL, Go RS, Mayo Clinic Histiocytosis Working Group 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 7 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 has not been described before. Two of these were found to have MAP2K1 mutations. Commonly utilized first line treatments were su...
Source: Haematologica - Category: Hematology Authors: Tags: Haematologica Source Type: research