MYELODYSPLASTIC SYNDROME FOLLOWING EPIPODOPHYLLOTOXIN THERAPY IN FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS

The prognosis for patients with familial hemophagocytic lymphohistiocy tosis (FHL) is poor, but the survival of affected children has been ma rkedly prolonged by treatment with the epipodophyllotoxin derivatives etoposide and teniposide and by bone marrow transplantation. Secondary malignancies following epipodophyllotoxin therapy, including myelodys plastic syndrome (MDS) and acute myelocytic leukemia (AML), have recen tly been reported. We describe a 9-year-old boy, treated with epipodop hyllotoxins for FHL since he was 3 years old, who developed MDS. He wa s administered etoposide (cumulative doses of 6.9 g/m2 intravenously a nd 13.6 g/m2 orally) and teniposide (3.4 g/m2 intravenously), but no o ther systemic antineoplastic drugs. This is, to our knowledge, the fir st report of a child with FHL developing MDS or AML. Moreover, MDS or AML following administration of epipodophyllotoxins as the sole system ic chemotherapic drug has not been reported previously, Supportive tre atments, including the use of immunomodulating drugs, may reduce the r isk for secondary leukemia in patients with FHL.