Rosai-Dorfman disease: successful long-term results by combination chemotherapy with prednisone, 6-mercaptopurine, methotrexate, and vinblastine: a case report.
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.
Jabali
Y
Y
Department of Pediatrics, Regional Hospital, Ceske Budejovice, Czech Republic.
Smrcka
V
V
Pradna
J
J
D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage*; D000963:Antimetabolites / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D005260:Female; D015618:Histiocytosis, Sinus / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology; D015122:Mercaptopurine / Q000008:administration & dosage; D008727:Methotrexate / Q000008:administration & dosage; D011241:Prednisone / Q000008:administration & dosage; D016896:Treatment Outcome; D014747:Vinblastine / Q000008:administration & dosage
D000893:Anti-Inflammatory Agents; D000963:Antimetabolites; D014747:Vinblastine; D015122:Mercaptopurine; D011241:Prednisone; D008727:Methotrexate