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, R., Deliliers, D., Zingale, L., Pogliani, E., Cicardi, M. (2007). Lymphoproliferative disease and acquired C1 inhibitor deficiency. HAEMATOLOGICA, 92(5), 716-718 [10.3324/haematol.10769].
Lymphoproliferative disease and acquired C1 inhibitor deficiency
POGLIANI, ENRICO MARIA;
2007
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.
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