Syndrome de destombes-rosai-dorfman [a propos d'un cas]

ABSTRACT

Destombes-RosaiDorfman syndrome or "sinusal histiocytosis with massive lymphadenopathy" is characterized by massive and painless adenopathies, initially cervical, with a biological inflammatory syndrome and a characteristic anatomopathological aspect. This disease, though rare, poses problems of etiological diagnosis as well as differential diagnosis, chiefly with malignant hemopathies. We report an observation of a fifteen-months infant, presenting left latero-cervical left isolated adenopathies, which appeared 3 months earlier with conservation of the general state and a histological exam of a ganglionary biopsy in favour of a hemophagocytar lymphadenitis, type Destombes RosaiDorfman. On the graund of this clinical observation, histological and evolutive aspects of this disease will be studied