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https://observatorio.fm.usp.br/handle/OPI/3018
Title: | Angioedema Due to Acquired C1-Inhibitor Deficiency |
Authors: | BEZERRA, Thiago de Almeida; ALMEIDA, Lais Pinto de; PEREIRA, Juliana; DUARTE, Alberto Jose da Silva; MORAES-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. |
Appears in Collections: | Comunicações em Eventos - FM/MPT Comunicações em Eventos - HC/ICHC Comunicações em Eventos - LIM/03 Comunicações em Eventos - LIM/31 Comunicações em Eventos - LIM/56 |
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