Colorectal cancer in patients with X-linked agammaglobulinaemia.

Primary immunodeficiency disorders can predispose to certain malignancies but hitherto no such relation has been established for X-linked agammaglobulinaemia (XLA). We have diagnosed rapidly progressive colorectal cancer in 3 unrelated young adults with XLA. We could find no explanation for the tumours. Since the calculated incidence of rectosigmoid cancer is increased 30-fold in patients with XLA, we advise the screening of these individuals, and perhaps people with other agammaglobulinaemias, for colorectal cancer.