Abstract
A 58-year-old woman presented to emergency departments on several occasions with episodic angioedema. Lymphoplasmacytic lymphoma with an IgM paraprotein (Waldenstrom's macroglobulinemia) was eventually diagnosed 14 months later in association with acquired C1 esterase inhibitor deficiency. Resolution of the angioedema and C1 esterase inhibitor deficiency was achieved with danazol and treatment of the underlying lymphoma.
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