Acquired C1-Esterase Inhibitor Deficiency Associated with Burkitt Lymphoma

Abstract

C1-esterase inhibitor deficiency results in recurrent episodes of angioedema. Acquired deficiency has been associated with B-cell lymphoproliferative disorders, rheumatologic diseases or, in a small proportion of patients, the cause remains unknown. Of the malignancies associated with acquired C1-esterase deficiency, indolent lymphomas and plasma cell disorders are the most common. Aggressive lymphomas are rarely reported in such cases. We report the association of Burkitt lymphoma, a highly aggressive B-cell malignancy, which evolved seven months after the initial symptoms of acquired angioedema in a fifty-five year old man. Following treatment of the lymphoma, episodes of angioedema ceased. An association of acquired angioedema with Burkitt lymphoma has not previously been reported.