[Intravenously administered gamma-globulins in reactive hemaphagocytic syndrome. Multicenter study to assess their importance, by the immunoglobulins group of experts of CEDIT of the AP-HP].

Abstract

Reactive hemophagocytic syndrome is characterized by systemic proliferation and activation of benign hemophagocytic cells of the monocyte-macrophage lineage. Treatment should be directed to the etiology, but successful treatment with high-dose gamma-globulin has been reported, especially in viral-associated hemophagocytic syndrome. We report 17 patients, of which 9 had infection-associated hemophagocytic syndrome, all treated with high-dose gamma-globulin. High-dose gamma-globulins appear to be more effective in infection-associated hemophagocytic syndrome, with a mean dose of 1.6gm/kg for one or two cycles. A multicentric randomized study is required to evaluate high-dose gamma-globulin in the treatment of reactive hemophagocytic syndrome.