Recurrent Acquired Angioedema as a Heralding Sign of Splenic Marginal Zone Lymphoma Recurrence

Acquired angioedema (AAE) is a rare disorder caused by an acquired deficiency in C1 esterase inhibitor leading to recurrent episodes of potentially life-threatening angioedema and is often associated with underlying lymphoproliferative disorders. Many cases of acquired angioedema are associated with lymphoproliferative disorders. Here, we present a case of a middle-aged woman who presented with multiple episodes of angioedema. She developed hemolytic anemia associated with angioedema, which prompted the diagnosis of an underlying splenic marginal zone lymphoma. Recurrent angioedema, particularly in middle-aged or older patients, should prompt a thorough workup for lymphoproliferative disorders, as timely treatment of the underlying condition can resolve angioedema and prevent life-threatening complications.