Klinefelter's syndrome and malignant lymphoma

The high risk for malignancy for carriers of several congenital chromosomal abnormalities is discussed. We report herein a patient with Klinefelter's syndrome associated with a high grade malignant non-Hodgkin lymphoma. Chromosome analysis of PHA-stimulated lymphocytes showed a mosaic of 46,XXY (90%) and normal 46,XY (10%) metaphases. To our knowledge, this is the third reported case of a malignant non-Hodgkin lymphoma in a patient with Klinefelter's syndrome in the literature.