Patients with a 47,XXY karyotype (Klinefelter syndrome) appear to have
an increased risk of developing cancer, especially male breast cancer
and germ cell tumors, but rarely malignant hematologic disorders. We
report a patient with a low grade B-cell non-Hodgkin lymphoma with a 4
7,XXY karyotype in both the tumor and constitutional cells. This is on
ly the 13th reported case of malignant lymphoma in a patient with Klin
efelter syndrome. Although some authors postulate that the 47,XXY kary
otype may be a predisposing factor in the development of hematological
malignancies, the lack of reported cases suggests that such associati
ons may be no more than chance findings. (C) Elsevier Science Inc., 19
97.