Successful treatment of virus‐associated haemophagocytic syndrome in adults by cyclosporin A supported by granulocyte colony‐stimulating factor

Abstract

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.

Hemophagocytic syndrome associated with hepatitis A: case report and literature review

“…Recent reports support the safety of Granulocyte-Colony Stimulating Factor (G-CSF) in patients with VAHS and severe neutropenia 3,23,44,45 . M-CSF is described as a trigger to the macrophage activation, increasing hemophagocytosis and worsening cytopenia 11,12,35 .…”

Correspondence

“…Haemophagocytic syndrome seems to be related to T-cell deregulation and a broad spectrum of inflammatory cytokines has been reported to be increased in patient sera, including M-CSF (Tsuda & Shirono, 1995). Tsuda & Shirono (1996) emphasized in their report that serum G-CSF levels were rarely elevated in haemophagocytic syndrome. Although G-CSF does not appear to be involved in initiation of this syndrome, systemic administration of supraphysiological doses of this cytokine could maintain or even increase macrophage activation.…”

“…In the report of Tsuda & Shirono (1996), G-CSF administration was discontinued after WBC recovery which occurred promptly after the onset of treatment. However, G-CSF doses were relatively low (100 mg/d for a 21-year-old patient).…”

“…The patient was given fibrinogen, platelets, red cell concentrates and broad-spectrum antibiotics. Furthermore, on day 0, based on the results of Tsuda & Shirono (1996), intravenous cyclosporin A (3 mg/d) and subcutaneous low-dose G-CSF (1 mg/kg) were started (Fig 1). Methylprednisolone (1 mg/ kg) was simultaneously added.…”

“…Tsuda & Shirono (1996) have described two cases of virusassociated haemophagocytic syndrome successfully treated by cyclosporin A and G-CSF. G-CSF was administered for 3 or 4 d and led to rapid recovery of white blood cell counts.…”