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.