Acquired angioedema (AAE) is a result of an acquired deficiency or inactivity of the C1 esterase inhibitor (C1-INH). There is a well-known link between AAE and lymphoplasmacytic disorders.

A 65-year-old woman who was diagnosed with chronic lymphocytic leukemia (CLL), presented with recurrent episodes of angioedema.

Although no association between the CLL and angioedema was initially recognized, further workup showed her to have low C1-INH levels.

Chemotherapy helped prevent subsequent episodes, but three years later she redeveloped angioedema. She was then placed on ofatumumab maintenance and has since remained free of angioedema.

Knowledge of this rare disease and anticipation of the link between CLL and AAE can prevent further attacks and associated morbidity.