Abstract
High serum cytokine levels may be responsible for various clinical features noted in virus-associated hemophagocytic syndrome (VAHS) . Prognosis of the patients varies widely and the determination of an appropriate therapy for the disease is difficult. Three types of therapy exist: immunochemotherapy which abolishes abnormal cytokine-network: plasma-exchange which reduces serum cytokine levels: and cytotoxic chemotherapy which targets abnormal lymphocyte clones. We successfully treated two of three patients with severe refractory VAHS by a combination of etoposide and cytarabine, both of them have been shown to be cell-cycle phase specific. In two of them, serum IFN-γ level was elevated and Thl dominated: in one, serum IL-4 and IL-6 levels were elevated and Th 2 dominated: and in one, Epstein-Barr virus was a causative agent. We conclude that intensified cytotoxic chemotherapeutic regimens should be considered for the patients who were diagnosed as severe VAHS.
