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Haematologica. 2007 May;92(5):716-8.

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.

PMID:
17488706
DOI:
10.3324/haematol.10769
[Indexed for MEDLINE]
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