[Sinus histiocytosis with massive lymphadenopathy or the Destombes-Rosai-Dorfman disease in the Ivory Coast].

Abstract

Sinusal histiocytosis with massive lymphadenopathy is a rare pathological entity since, as of 1983, only 200 cases have been published. Following the seminal description in 1965 by Destombes in Blacks, histological features of this disease were specified by Rosai and Dorfman in 1969 and 1972. We report three cases in young Ivorian subjects. The presenting finding is always chronically enlarged lymph nodes, but extranodal lesions are possible, most commonly involving the eye, salivary glands, upper respiratory tract, skin, bone, testis, and nervous system. Diagnosis can be ascertained only upon histologic examination of lymph node biopsy specimens which shows the three cardinal criteria, i.e. massive sinusal histiocytosis, lymphophagocytosis, and mature plasmocytosis. Although a few fatal cases have been reported, the disease usually runs a benign course, with exacerbations of variable duration. Great caution should therefore be taken in deciding upon management, particularly as regards corticosteroid therapy, radiotherapy or anticancer chemotherapy. Etiopathogeny of the disease is unsettled; there is general agreement as to the existence of a cellular immune dysfunction resulting in the lympho-histiocytic proliferation.