Acquired angioedema with raised serum tryptase

Abstract

In a patient presenting with acute angioedema, a raised serum mast-cell tryptase level may at first suggest an allergic cause. Persistent tryptase elevation may indicate a mast cell disorder. In this case, the raised tryptase turned out to be incidental to the final diagnosis, which was acquired C1 inhibitor deficiency caused by a B-cell lymphoma. We believe this is the first report of elevated tryptase in association with B-cell lymphoid malignancy and acquired angioedema.