SINUSHISTIOCYTOSIS WITH MASSIVE LYMPHADENOPATHY (ROSAI-DORFMAN DISEASE) - RESPONSE TO METHOTREXATE AND MERCAPTOPURINE

We report a 3-year-old girl presenting with bilateral cervical lymph n ode enlargement persisting for > 3 months. Leukocytosis, elevated eryt hrocyte sedimentation rate, a marked hypergammaglobulinemia, and a mod erate hepatosplenomegaly were also found. The diagnosis of sinushistio cytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfm an disease, was established histologically by the demonstration of cha racteristic sinushistiocytosis with lymphocytophagocytosis. Treat ment was started with high dose steroids, and a decline of lymph node size and a normalization of laboratory parameters occurred. However, when steroids were tapered, lymph node size rapidly reincreased. Chemothera peutic treatment was started using etoposide, which was completely ine ffective. Therefore, treatment was changed to a combinatory low dose m ethotrexate therapy and 6-mercaptopurine for 4 months. Whereas a promp t and complete remission was reached, single 6-mercaptopurine therapy was maintained and treatment has been discontinued after a total of 2 years. The child has remained healthy for 7 years. This case would rec ommend the use of methotrexate and 6-mercaptopurine for treatment of c omplicated SHML. (C) 1996 Wiley-Liss, Inc.