H. Tsuda et K. Shirono, SUCCESSFUL TREATMENT OF VIRUS-ASSOCIATED HEMOPHAGOCYTIC SYNDROME IN ADULTS BY CYCLOSPORINE-A SUPPORTED BY GRANULOCYTE-COLONY-STIMULATING FACTOR, British Journal of Haematology, 93(3), 1996, pp. 572-575
Two young adult patients with typical virus-associated haemophagocytic
syndrome (VAHS) were treated with cyclosporin A and granulocyte colon
y-stimulating factor (G-CSF). Clinical symptoms such as high fever and
malaise disappeared rapidly with concurrent haematological improvemen
t in both patients. The serum levels of interleukin-6 (IL-6), soluble
IL-2 receptor, tumour necrosis factor and macrophage-CSF were all elev
ated before treatment but that of G-CSF was not. The dramatic effect o
f cyclosporin A observed implies that it efficiently and rapidly suppr
esses the cytokine storm caused by dysregulated T cells in VAHS. In ad
dition, G-CSF may promote haematological recovery without syndrome reg
ression. We believe that the combination of cyclosporin A and G-CSF ma
y be effective, at least in selected patients with VAHS. Further studi
es are required to confirm its role as first-line therapy for adult pa
tients with VAHS.