Lymphoproliferative disease and acquired C1 inhibitor deficiency.
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.
Castelli
Roberto
R
Deliliers
Daniela Lambertenghi
DL
Zingale
Lorenza C
LC
Pogliani
Enrico Maria
EM
Cicardi
Marco
M
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
D001323:Autoantibodies; D050718:Complement C1 Inhibitor Protein