Rosai-Dorfman Disease: The MD Anderson Cancer Center Experience.

Background: Rosai-Dorfman disease (RDD) is a rare disorder composed of a proliferation of histiocytes with varied clinical manifestations.

Patients And Methods: In this retrospective observational study, we obtained demographic, clinical, and outcome data from the medical records of 10 adult patients with RDD seen in the Department of Lymphoma and Myeloma at MD Anderson Cancer Center between 1995 and 2015.

Results: Patients had a median age of 56 years (range, 20-81 years) with equal gender distribution. Five patients were initially treated with systemic therapy. Five received cladribine as initial therapy or for relapse. Other therapeutic agents included clofarabine, lenalidomide, and steroids. The overall response rate was 80% in patients receiving cladribine, with a median progression-free survival of 29 months. Surgery, radiotherapy, and observation were also treatment options for localized disease. With a median follow-up of 65 months, none of the patients have died.

Conclusion: Currently, there is no standard of care for RDD. Treatment should be personalized for each patient depending on the clinical presentation, course of the disease, and prior treatment history. Purine analogues provide excellent responses; however, surgical resection and observation are also suitable for specific cases.