Castelli Roberto, Deliliers Daniela Lambertenghi, Zingale Lorenza C, Pogliani Enrico Maria, Cicardi Marco
Haematologica. 2007 May;92(5):716-8. doi: 10.3324/haematol.10769.
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.
由于获得性C1抑制物缺乏引起的血管性水肿是自身免疫和淋巴增殖之间的一种过渡状态。我们报告了32例获得性C1抑制物缺乏患者：23例有抗C1抑制物自身抗体；13例有无意义单克隆丙种球蛋白病，9例有非霍奇金淋巴瘤。我们的系列研究提示，在获得性血管性水肿中，不同形式的B细胞疾病共存和/或相互演变。
