Pulmonary Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue Associated with Granulomatous Inflammation in a Child with Chromosome 22q11.2 Deletion Syndrome (DiGeorge Syndrome)

Patients with immunodeficiency disorders have an increased incidence of lymphoproliferative disorders; however, only 4 such patients with DiGeorge/chromosome 22q11.2 deletion syndrome have been reported. We report a case of a pulmonary Epstein-Barr virus-negative extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in a child with this syndrome.