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  • Volume 7
  •  Issue 6
  • Publication Date: May 2007


Diffuse Large B-Cell Lymphoma in Hyperimmunoglobulinemia E Syndrome


Nadège Wallet, David Ghez, Richard Delarue, Felipe Suarez, Marie-Thérèse Rubio, Alain Fischer, Danielle Canioni, Bruno Varet, Olivier Hermine

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.

Key words: Antigenic stimulation, Immunodeficiency, Job syndrome, Lymphomagenesis



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