Sinus Histiocytosis With Massive Lymphadenopathy

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Abstract
In the present issue of theArchives, Suster et al1describe a 72-year-old woman with large soft-tissue masses that spontaneously disappeared over a period of several weeks. In spite of the absence of lymphadenopathy, the authors cautiously suggest the diagnosissinus histiocytosis with massive lymphadenopathy(SHML) (Rosai-Dorfman disease), based primarily on the biopsy finding of a certain cytologic subtype of S100-positive histiocytes exhibiting lymphocytophagocytosis. It is appropriate to ask just how reliable these morphological and immunologic features are in segregating a specific disease entity. If the findings are reliable, what is our current understanding of the clinical and pathologic features of this disease, how is it related to other "histiocytoses," and what are the major directions for investigation of this disease? Histopathologic observations are currently used to subclassify many disease processes that are associated with proliferation of lymphoid and histiocytic cell lines, although immunophenotypic and immunogenotypic studies