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Title: Angioedema Due to Acquired C1-Inhibitor Deficiency
Authors: BEZERRA, Thiago de AlmeidaALMEIDA, Lais Pinto dePEREIRA, JulianaDUARTE, Alberto Jose da SilvaMORAES-VASCONCELOS, Dewton de
Citation: JOURNAL OF CLINICAL IMMUNOLOGY, v.32, n.2, p.395-395, 2012
Abstract: Angioedema due to acquired C1-inhibitor deficiency is a rare, life-threatening disease with poorly defined etiology, therapy, and prognosis. It is characterized by increase in vascular permeability (angioedema) of the skin, the gastrointestinal and oro-pharyngo-laryngeal mucosa. The mediator of symptoms is bradykinin, released from HMW kininogen, cleaved by plasma kallikrein, a serine protease controlled by C1-INH. We report one 58 years old female patient with acquired C1-INH deficiency. The age at onset of angioedema was 58 years (May, 2011). C1-INH function and C4 antigen quantitation were below 50% of normal. C1q was also reduced. Associated disease was a B cell follicular non-Hodgkin lymphoma. Autoantibodies inactivating C1-INH are detected in the majority of patients and account for the deficiency. Lymphoproliferative diseases, ranging from benign monoclonal gammopathies to malignant lymphoma, are frequently associated with AAE. Antifibrinolytic agents are more effective than attenuated androgens in long-term prophylaxis. Patients with acquired C1-INH deficiency may be resistant to replacement therapy with C1-INH plasma concentrate.
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Comunicações em Eventos - FM/MPT
Departamento de Patologia - FM/MPT

Comunicações em Eventos - HC/ICHC
Instituto Central - HC/ICHC

Comunicações em Eventos - LIM/03
LIM/03 - Laboratório de Medicina Laboratorial

Comunicações em Eventos - LIM/31
LIM/31 - Laboratório de Genética e Hematologia Molecular

Comunicações em Eventos - LIM/56
LIM/56 - Laboratório de Investigação em Dermatologia e Imunodeficiências

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