NON-HODGKINS-LYMPHOMA AND KLINEFELTER SYNDROME

Patients with a 47, XXY karyotype (Klinefelter syndrome) appear to hav e an increased risk of developing a malignancy in adulthood, usually c ancer of the breast, extragonadal germ cell tumor, and acute nonlympho blastic leukemia. There is growing evidence to show that these patient s also have an increased risk of developing a malignancy in childhood. There are reports describing the development of acute lymphoblastic l eukemia, retinoblastoma, and rhabdomyosarcoma in children with a 47, X XY or mosaic 47, XXY/46, XY karyotype. We report a child with a bone m etastasizing, B-cell lineage, non-Hodgkin's lymphoma (NHL) who was fou nd to have a 47, XXY karyotype in both the tumor and constitutional ce lls.