title: Lymphoproliferative disease and acquired C1 inhibitor deficiency.
abstract: 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.
authors.forename: Roberto
authors.initials: R
authors.lastname: Castelli
authors.forename: Daniela Lambertenghi
authors.initials: DL
authors.lastname: Deliliers
authors.forename: Lorenza C
authors.initials: LC
authors.lastname: Zingale
authors.forename: Enrico Maria
authors.initials: EM
authors.lastname: Pogliani
authors.forename: Marco
authors.initials: M
authors.lastname: Cicardi
mesh_terms: D000799:Angioedema / Q000209:etiology* / Q000276:immunology; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D001327:Autoimmune Diseases / Q000150:complications / Q000276:immunology*; D001402:B-Lymphocytes / Q000276:immunology / Q000473:pathology*; D002999:Clone Cells / Q000276:immunology / Q000473:pathology*; D050718:Complement C1 Inhibitor Protein / Q000276:immunology*; D018450:Disease Progression; D005500:Follow-Up Studies; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000209:etiology / Q000628:therapy; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000276:immunology; D010265:Paraproteinemias / Q000209:etiology
chemical_list: D001323:Autoantibodies; D050718:Complement C1 Inhibitor Protein