The acquired deficiency of C1-inhibitor: lymphoproliferation and angioedema.
Acquired deficiency of C1 inhibitor (C1-INH) with angioedema symptoms (acquired angioedema, AAE) is characterized by local increase in vascular permeability (angioedema) of the skin and the gastrointestinal and oro-pharyngo-laryngeal mucosa. The mediator of symptoms is bradykinin, a potent vasoactive peptide, released from high molecular weight kininogen when it is cleaved by plasma kallikrein a serine protease controlled by C1-INH. Autoantibodies inactivating C1-INH are detected in the majority of patients and account for the deficiency. Irrespectively to the presence of anti-C1-INH autoantibodies lymphoproliferative diseases, ranging from benign monoclonal gammopathies to malignant lymphoma, are frequently associated with AAE. Demonstration that monoclonal components correspond to anti-C1-INH autoantibodies and correlation between course of lymphoma and course of AAE provide strong support to consider the two diseases expression of the same pathologic process.
Cicardi
M
M
Dipartimento di Scienze Cliniche L. Sacco, Università di Milano, Ospedale Luigi Sacco, Milano, Italy. Marco.Cicardi@unimi.it
Zanichelli
A
A
D000799:Angioedema / Q000097:blood / Q000209:etiology* / Q000276:immunology; D001323:Autoantibodies / Q000097:blood; D001402:B-Lymphocytes / Q000276:immunology; D001920:Bradykinin / Q000502:physiology; D002199:Capillary Permeability; D050718:Complement C1 Inhibitor Protein / Q000276:immunology / Q000378:metabolism*; D005260:Female; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000097:blood / Q000209:etiology* / Q000276:immunology; D008297:Male; D008954:Models, Biological
D001323:Autoantibodies; D050718:Complement C1 Inhibitor Protein; D001920:Bradykinin