Abstract

Immunodeficiency is associated with increased susceptibility to infections and B-cell lymphoproliferative disorders, which range from polyclonal proliferations to monoclonal lymphomas. In primary immunodeficiencies, the true incidence of lymphoproliferative disorders has been estimated between 1.4% and 24%. Recurrent or chronic infections resulting in continuous antigen exposure result in abnormal B-cell proliferation and ultimately lymphoma. Immunosuppression leads to increase in likelihood of Epstein-Barr virus-associated malignancies. We report a case of the child with multifocal extranodal non-Hodgkin lymphoma involving both the lungs and brain in a case of primary immunodeficiency.

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