Angioedema due to acquired deficiency of the CI-inhibitor is a bridging condition between autoimmunity and lymphoproliferation. We report 32 patients with acquired CI inhibitor deficiency: 23 have anti CI-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.
Lymphoproliferative disease and acquired C1 inhibitor deficiency / R. Castelli, D.M. Lambertenghi Deliliers, L.C. Zingale, E.M. Pogliani, M. Cicardi. - In: HAEMATOLOGICA. - ISSN 0390-6078. - 92:5(2007), pp. 716-718. [10.3324/haematol.10769]
Lymphoproliferative disease and acquired C1 inhibitor deficiency
R. Castelli;D.M. Lambertenghi Deliliers;L.C. Zingale;M. Cicardi
2007
Abstract
Angioedema due to acquired deficiency of the CI-inhibitor is a bridging condition between autoimmunity and lymphoproliferation. We report 32 patients with acquired CI inhibitor deficiency: 23 have anti CI-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.
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