SINUS ISTIOCITOSI CON MASSIVA LINFOADENOPATIA (MALATTIA DI ROSAI-DORFMAN): ETEROGENEITA DEL QUADRO CLINICO E FAVOREVOLE RISPOSTA AL TRATTAMENTO CHEMIOTERAPICO IN UN CASO PEDIATRICO

Sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai-Dorfman's disease, is a rare histiocytic proliferative disorder characterized by nodal and extranodal infiltration. We hereby report an unusual case of SHML with prevalent extranodal involvement who developed sequentially different clinical syndromes mimicking specific diseases. The final diagnosis has been delayed because of these protean clinical manifestations. The severe multi-organ involvement warranted treatment with a cytotoxic chemotherapy, protocol including prednisone, cyclophosphamide and vincristine. This case is focusing on the extreme clinical heterogeneity of SHML and provides additional evidence for the efficacy of combination cytotoxic chemoteraphy in patients with severe multi-system organ involvement.