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Lymphoproliferative disease and acquired C1 inhibitor deficiency

^* Division of Internal Medicine, Department of Internal Medicine and Medical Specialities IRCCS Fondazione Ospedale Maggiore Policlinico of Milan, Milan, Italy
^° Clinica Ematologia, TMO Università degli Studi di Milano, Bicocca Ospedale San Gerardo di Monza; II Division of Internal Medicine, Sacco Hospital, University of Milan, Italy

Correspondence: Roberto Castelli, MD, Department of Internal Medicine and Medical Specialities, IRCCS Fondazione Ospedale Maggiore Policlinico of Milan, via Pace 9, 20122 Milan, Italy. Phone: international +39.02.55035320. Fax: international +39.02.55034722. E-mail: castelli39{at}interfree.it

Angioedema due to acquired deficiency of the C1-inhibitor is a bridging condition between autoimmunity and lymphoproliferation. We report 32 patients with acquired C1 inhibitor deficiency: 23 have anti C1-inhibitor autoantibodies; 13 have monoclonal gammopathies of unknown significance and 9 have non-Hodgkin’s lymphoma. Our series suggest that different forms of B cell disorders coexist and/or evolve into each other in acquired angioedema.

Key words: C1 inhibitor, deficiency, acquired, lymphoproliferative, autoantibody.