This report describes a case of a young man with DiGeorge Syndrome, repaired Tetralogy of Fallot, relapsed metastatic Hodgkin's Lymphoma, immunodeficiency, and a history of recurrent and severe infections. A review of the literature indicates that patients with DiGeorge Syndrome are at greater risk for infection, malignancy, and cardiac events due to anatomic and immunologic complications resulting from a deletion in the 22q11.2 chromosome. As an increased number of patients with DiGeorge Syndrome are surviving into adulthood, it is important to understand the progression of the disease and the long-term implications associated with variable degrees of thymic hypoplasia and immune deficiency.
Keywords: 22q11.2 deletion syndrome; digeorge syndrome; hodgkin’s lymphoma; immunodeficiency; infectious disease; lymphopenia; t-cell deficiency.
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