Lymphoproliferative disease and acquired C1 inhibitor deficiency

R. Castelli, D. L. Deliliers, L. C. Zingale, E. M. Pogliani, M. Cicardi
2007 Haematologica  
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. Haematologica 2007; 92:5: 716-718 Angioedema due to the
more » ... gioedema due to the acquired deficiency of the inhibitor of the first component of human complement (CI-INH) is a rare syndrome usually identified as acquired angioedema (AAE). One hundred and thirtysix cases are described literature. 1-3 The clinical features of C1-INH deficiency, which can also be of genetic origin (hereditary angioedema, HAE), includes subcutaneous, non-pruritic swelling without accompanying urticaria, involvement of the upper respiratory tract, and partial obstruction of the gastrointestinal tract presenting as abdominal pain. Unlike HAE, AAE has no family history of angioedema, but is characterized by a Letters to the Editor | 716 | haematologica/the hematology journal | 2007; 92(05) Letters to the Editor haematologica/the hematology journal | 2007; 92(05) | 717 |
doi:10.3324/haematol.10769 pmid:17488706 fatcat:kcnml7odmbhhphytpmxxfsulke