Common variable immunodeficiency (CVID) is a condition characterized by low levels of immunoglobulin (Ig) G and either IgA or IgM in the presence of recurrent infections. This disorder is associated with an increased risk of malignancy. Mucosa-associated lymphoid tissue (MALT) lymphoma is a recently recognized form of non-Hodgkin's lymphoma that is not often present in the head. MALT lymphoma in patients with CVID is rare, and until now, it has not been reported in a cranial location outside of the parotid gland. We report the cases of 2 patients who had CVID and cranial MALT lymphoma outside of the parotid gland, and we describe their successful treatment with chemotherapy.
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