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Two young adult patients with typical virus-associated haemophagocytic syndrome (VAHS) were treated with cyclosporin A and granulocyte colony-stimulating factor (G-CSF). Clinical symptoms such as high fever and malaise disappeared rapidly with concurrent haematological improvement in both patients. The serum levels of interleukin-6 (IL-6), soluble IL-2 receptor, tumour necrosis factor and macrophage-CSF were all elevated before treatment but that of G-CSF was not. The dramatic effect of cyclosporin A observed implies that it efficiently and rapidly suppresses the cytokine storm caused by dysregulated T cells in VAHS. In addition, G-CSF may promote haematological recovery without syndrome regression. We believe that the combination of cyclosporin A and G-CSF may be effective, at least in selected patients with VAHS. Further studies are required to confirm its role as first-line therapy for adult patients with VAHS.

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Between August 1992 and August 1994 we found seven adult patients with haemophagocytic syndrome associated with human parvovirus B19 (HPV) infection. Five of the patients were previously healthy but the other two had underlying immunosuppressive disease; the former five patients recovered spontaneously without any treatment. Eight cases of HPV-associated haemophagocytic syndrome have been reported; six of them were children and the remainder were adults with immunosuppressive disease or haematological disorder. Our study suggests that HPV is not a rare cause of virus-associated haemophagocytic syndrome (VAHS). Moreover, HPV-associated haemophagocytic syndrome could occur more frequently than previously thought, not only in children and adults with underlying disease but also in adults, even in good health.