HEMOPHAGOCYTIC SYNDROME-ASSOCIATED WITH HEMATOLOGICAL NEOPLASIAS

Hemophagocytic syndrome (HPS) is a reactive process that complicates s everal diseases including hematological neoplasias (HN). It has been s uggested that HPS may be a negative prognosis factor for neoplastic di seases. In this retrospective analysis, 13 cases with HPS associated t o HN were compared with two age, sex, diagnosis, disease stage and tre atment matched controls in order to determine the impact of this syndr ome on the survival. Cases with HPS were adult patients with a male:fe male ratio of 1:1 and their clinical picture was characterized by feve r, lymphadenopathy, hepatosplenomegaly, and pancytopenia, Median survi val since HN diagnosis was 7 and 48 months for the HPS and control gro ups, respectively (P = 0.0001). In ten patients who died, median survi val after HPS presentation was 1 month. These results suggest that the presence of HPS is a negative prognosis factor in patients with HN. D ue to its high mortality rate, an individualized, early, and intensive chemotherapeutic regimen may be required for HN complicated with this syndrome. (C) 1998 Elsevier Science Ltd. All rights reserved.