The Bottom Line: Rarely. Normal level of C1 esterase inhibitor proteins in association with angioedema due to lymphoproliferative disease is a rare and an uncommon finding caused by antibodies produced from the underlying disease (Gunatilake and Wimalaratna, 2014).
Acquired angioedema (AAE) that is associated with C1INH deficiency is rare, approximately 10 times more rare than the hereditary forms, which are estimated to occur in between 1/10,000 and 1/50,000 of the population (Gobert et al, 2016).
References: Gobert D, et al. A nationwide study of acquired C1-inhibitor deficiency in France. Medicine (Baltimore). 2016 Aug; 95(33):e4363. doi:10.1097/MD.0000000000004363
Gunatilake SSC and Wimalaratna H. Angioedema as the first presentation of B-cell non-Hodgkin lymphoma – an unusual case with normal C1 esterase inhibitor level: A case report. BMC Research Notes. 2014; 7:495. doi: 10.1186/1756-0500-7-495
Patel S, Patel R, Draikiwicz S, Capitle E. Peripheral T-cell lymphoma: A challenging mimicker of angioedema and urticaria. Annals of Allergy, Asthma and Immunology. 2015 Aug; 115(2):94-95. doi:10.1016/j.anai.2015.06.013
Summary: Although first described more than 130 years ago, the pathophysiology, origin, and management of several types of angioedema are not completely understood. Mimickers of angioedema further complicate the picture. It stresses the importance of always considering an underlying lymphoproliferative disorder in cases of angioedema, which could allow for more prompt treatment (Patel et al, 2015).
It is highly uncommon for angioedema to coincide with Hodgkin’s lymphoma – especially nodular sclerosis Hodgkin’s lymphoma. It is more commonly associated with non-Hodgkin’s lymphoma. This highlights a gap in the literature and thus an opportunity to add to the literature and knowledge base.