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Title: HLA Class I Deficiency Resembling Wegener's Granulomatosis: Report of Two Cases
Authors: ALMEIDA, Lais Pinto deLEITE, Olavo Henrique MunhozNICODEMO, Antonio CarlosORII, Noemia MieDIAS, Alana dos SantosMORAES-VASCONCELOS, Dewton de
Citation: JOURNAL OF CLINICAL IMMUNOLOGY, v.32, n.2, p.403-404, 2012
Abstract: HLA class I deficiency is a rare immunodeficiency characterized by recurrent respiratory infections and skin ulcers with granuloma. Herein we describe two patients with HLA class I deficiency with skin lesions and sinopulmonary disease. They were initially diagnosed as having WG and treated with immunosuppressive medication, without response. Patient 1: 25-year-old woman with previous diagnosis of TAP-1 deficiency (c2239 G>A, generating a premature stop codon). She had presented multiple ulcers on the legs, 2 episodes of severe pansinusitis, and chronic lung disease. At age 14 she was diagnosed as having WG. Patient 2: 36 year-old woman, with severe sinopulmonary disease leading to a sinuso-facial fistula and destruction of the uvula, besides chronic deep ulcers of her legs. The laboratorial features included negative c-ANCA, low CD8+ T cell counts and reduced expression of MHC class I in mononuclear cells. There are less than 30 MHC class I deficient patients described. The rarity of the syndrome turns our report interesting, in order to improve the knowledge of the disease.
Appears in Collections:

Comunicações em Eventos - FM/MDT
Departamento de Dermatologia - FM/MDT

Comunicações em Eventos - FM/MIP
Departamento de Moléstias Infecciosas e Parasitárias - FM/MIP

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

Comunicações em Eventos - LIM/46
LIM/46 - Laboratório de Parasitologia Médica

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

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