Acquired angioedema with C1 inhibitor deficiency in a patient with low-grade B-lymphoma and the effect of underlying disease treatment on angioedema manifestations
2017
Abstract
Acquired angioedema with C1 inhibitor deficiency is a rare disease manifesting as recurrent angioedema of the skin and mucous membranes in different locations. It is usually associated with haematological malignancies or monoclonal gammopathy.
According to current findings, treatment of the underlying hematologic disease may prevent angioedema attacks in many cases. In our case report, we present a female patient suffering from acquired angioedema with C1 inhibitor deficiency and subsequently diagnosed with splenic lymphoma.
Treatment of the lymphoma resulted in the normalisation of complement parameters and complete disappearance of angioedema attacks.