Up-regulation of adhesion and MHC molecules on splenic monocyte/macrophages in adult haemophagocytic syndrome

Haemophagocytic syndrome (HPS) has been associated with the abnormal activation of mono/macrophages and increased cytokine production, However, neither the phenotype of haemophagocytic monomacrophages nor the cellular origin of cytokine production have been described. We studied splenic monomacrophages and lymphocytes from five patients with HPS (two HIV- and three HIV+) and from controls without HPS (three normal HIV- and two pathological HIV+). Using flow-cytometry, we observed a marked increase in the expression of MHC class I and UI, M-CSF-receptor and adhesion molecules LFA-1, LFA-3, ICAM-1 (P<0.05) on HPS+ splenic monomacrophages compared to HPS-, which was independent of their HIV status. A high percentage of CD8(+) lymphocytes from 4/5 HPS+ patients produced TNF alpha and IFN gamma, but no IL-6 upon in-vitro activation. In a fifth patient CD4(+) but not CD8(+) lymphocytes produced these cytokines, Although other cytokines might be involved in the pathophysiology of HPS as suggested by the high expression of M-CSF-receptor, these results suggest that TNF alpha and IFN gamma secretion by T cells might play a role in the up-regulation of adhesion and MHC molecules on monomacrophages from HPS.