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.
|Translated title of the contribution||Sinus histocytosis with massive lymphadenopathy (Rosai-Dorfman's disease): Heterogeneity of the clinical picture and positive response to the chemotherapic treatment in a pediatric case|
|Number of pages||5|
|Journal||Rivista Italiana di Pediatria|
|Publication status||Published - 1993|
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health