Successful treatment of virus-associated haemophagocytic syndrome in adults by cyclosporin A supported by granulocyte colony-stimulating factor.
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.
Tsuda
H
H
Division of Clinical Haematology and Immunology, Kumamoto City Hospital, Japan.
Shirono
K
K
D000328:Adult; D016572:Cyclosporine / Q000627:therapeutic use*; D003586:Cytomegalovirus Infections / Q000150:complications; D004359:Drug Therapy, Combination; D005260:Female; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D006566:Herpesviridae Infections / Q000150:complications; D015616:Histiocytosis, Non-Langerhans-Cell / Q000188:drug therapy*; D006801:Humans; D008297:Male
D016179:Granulocyte Colony-Stimulating Factor; D016572:Cyclosporine