Malignant histiocytosis in adults: report of 7 patients from Northeast Italy.

The clinical records and histologic material of seven adult male patients with malignant histiocytosis (MH), observed consecutively at our center during a four-year period, have been reviewed; four of these cases were seen in a year. All patients were born and lived within a radius of 30 miles from Pordenone, a town in northeast Italy. The diagnosis of MH was made in all cases on lymph node biopsy on the basis of the established histologic criteria [1]. At presentation, abnormal histiomonocytic cells were found in the bone marrow and peripheral blood of four patients, two of whom also showed cerebrospinal fluid involvement with atypical histiocytes at a relatively early stage of the disease. Staging procedures documented extensive disease in lymphatic, as well as in extralymphatic, sites in four patients; in three patients, the disease was confined to the lymphatic system. Combination chemotherapy was the first treatment employed in all cases. Four patients, three of whom had limited disease, achieved complete remission with quadruple combination chemotherapy (ABVD, CHOP, or MOPP); three patients with extensive disease achieved only partial remission of short duration with HOP. These results further support the view that adult MH is not obligatorily and rapidly fatal. Good responses to treatment with combination chemotherapy are possible, particularly in patients with disease limited to only lymphatic sites.