Lymphoproliferative disease and acquired C1 inhibitor deficiency

• Published in: Haematologica (Roma) Vol. 92; no. 5; pp. 716 - 718
• Main Authors: Castelli, Roberto, Deliliers, Daniela Lambertenghi, Zingale, Lorenza C, Pogliani, Enrico Maria, Cicardi, Marco
• Format: Journal Article
• Language: English
• Published: Italy Ferrata Storti Foundation 01.05.2007
• Subjects: Angioedema - etiology; Angioedema - immunology; Autoantibodies - blood; Autoantibodies - immunology; Autoimmune Diseases - complications; Autoimmune Diseases - immunology; B-Lymphocytes - immunology; B-Lymphocytes - pathology; Clone Cells - immunology; Clone Cells - pathology; Complement C1 Inhibitor Protein - immunology; Disease Progression; Follow-Up Studies; Humans; Lymphoma, Non-Hodgkin - etiology; Lymphoma, Non-Hodgkin - therapy; Lymphoproliferative Disorders - complications; Lymphoproliferative Disorders - immunology; Paraproteinemias - etiology
• ISSN: 0390-6078; 1592-8721
• DOI: 10.3324/haematol.10769

* 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.