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Int J Surg Pathol. 2005 Jul;13(3):285-9.

Rosai-Dorfman disease: successful long-term results by combination chemotherapy with prednisone, 6-mercaptopurine, methotrexate, and vinblastine: a case report.

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Department of Pediatrics, Regional Hospital, Ceske Budejovice, Czech Republic.


Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease (RDD), is a rare clinicopathologic entity of unknown etiology. The majority of patients do not require treatment, demonstrating spontaneous resolution or stable disease. However, in some cases the disorder runs a progressive course and/or threatens vital structures and functions, which dictates some form of intervention. It is not clear what constitutes the best approach for those cases. We report on a girl with an extensive and progressive form of the disease who was cured with combination chemotherapy, showing no evidence of recurrence after a follow-up of 5.5 years.

[Indexed for MEDLINE]

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