Diffuse large B-cell lymphoma in hyperimmunoglobulinemia E syndrome.

We report a case of diffuse large B-cell lymphoma occurring in a patient with the hyperimmunoglobulinemia E syndrome, a rare immune disorder defined by elevated immunoglobulin E levels and recurrent bacterial and fungal infections often manifesting as cold abscesses. This case further supports the notion that patients with hyperimmunoglobulinemia E have an increased risk of lymphoid malignancies and should be closely monitored. Despite a theoretic risk of severe infectious complications, chemotherapy was well tolerated and resulted in a sustained complete remission.