G. Di Leonardo, C. Ligas, M. Stati, L. Ginaldi, D. Quaglino

Clinica Medica II, Università degli Studi de L’Aquila 

There are few disease entities so confusing as those related to the histiomonocytic cells and their malignant proliferations, defined either as Malignant Histiocytosis or histiocytic lymphoma. Both terms have been considered synonymous and employed indifferently by most Authors, although certain differences in the clinical manifestations have sometimes been outlined. The term “malignant-histiocytosis” was applied to a hematological condition first described by Scott and Robb-Smith [1] as histiocytic medullary reticulosis on the basis of its clinical features. Rappaport [2] introduced the term “malignant histiocytosis” in 1966 and reviewed the pathological features of this condition. Malignant histiocytosis has been considered a most aggressive disease. It was characterized by the systemic proliferation of malignant cells [3] and presented with fever, wasting, pancytopenia, hepatosplenomegaly and adenopathy.