Immunodeficiency in patients with hemophilia: an underlying deficiency and lack of correlation with factor replacement therapy or exposure to human immunodeficiency virus.

Nineteen patients with hemophilia, five of whom were classified as untreated, were immunologically evaluated by the measurement of cell surface markers, natural killer cell activity, mitogenic responses, polyclonal immunoglobulin production, and serologic evidence of human immunodeficiency virus infection. No correlations were found between human immunodeficiency virus infection and immune abnormalities, or between patients who did or did not receive factor therapy, although the abnormalities were more profound in those who received treatment. An intrinsic B cell abnormality in patients with hemophilia is suggested.