Risk of etoposide-related acute myeloid leukemia in the treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis

We studied the impact of etoposide on the prognosis of 81 patients (77 of whom were children <15 years old) with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH). The study group received it median cumulative dose of 1500 mg/m(2) etoposide (range, 0-14,550 mg/m(2)), with a median follow-up period of 44 months (range. 20-88 months) from the diagnosis. Only 1 patient, who received 3150 mg/m(2) etoposide, developed therapy-related acute mycloid leukemia (t-AML), at 31 months after diagnosis. Excluding 9 patients who underwent hemopoietic stem cell transplantation during the course of treatment, the prognosis was poorer for those patients who received less than it 1000 mg/m(2) cumulative dose of etoposide. Our results indicate that the risk of etoposide-related t-AML is low. An appropriate dosage of etoposide for the treatment of EBV-HLH would be in the range of 1000 to 3000 mg/m(2). However, even at these doses, care must be taken to prevent the rare risk of t-AML. Int J Heinatol. 2002:75:174-177. (C)2002 The Japanese Society of Hematology.