RESUMO
We describe a 49-year-old patient with lip biopsy proven Sjögren's syndrome (SS) and keratoconjunctivitis sicca, who had dental caries, xerostomia, recurrent upper respiratory tract infections, arthritis in her hands, elbows and knees, and recurrent parotid inflammation. She developed bilateral breast nodules in 1988. Right breast nodules were excised in 1993 and 1995, but reappeared in 1996, requiring 2 more excisions. Breast tissue samples showed remarkable intralobular and perilobular mononuclear cell infiltrates that were predominantly CD4+ T cells and expressed bcl-2. A few cells stained CD20+ and CD8+. SS breast glandular epithelial cells stained more intensely for Fas compared to normal cells. CD4+ T cells and Fas mediated cell death may be involved in the mammary gland lesions in SS.
Assuntos
Doenças Mamárias/patologia , Linfócitos T CD4-Positivos/patologia , Glicoproteínas de Membrana/metabolismo , Síndrome de Sjogren/patologia , Receptor fas/metabolismo , Mama/metabolismo , Mama/patologia , Doenças Mamárias/metabolismo , Linfócitos T CD4-Positivos/metabolismo , Epitélio/metabolismo , Epitélio/patologia , Proteína Ligante Fas , Feminino , Humanos , Técnicas Imunoenzimáticas , Ceratoconjuntivite Seca/patologia , Pessoa de Meia-Idade , Proteínas Proto-Oncogênicas c-bcl-2/metabolismo , Síndrome de Sjogren/metabolismo , Xerostomia/patologiaRESUMO
This study was undertaken to summarize the current status of the clinicopathologic factors related to non-Hodgkin's lymphoma (NHL) development in patients with Sjögren's syndrome (SS). Through a MEDLINE search, pertinent articles on SS, monoclonality, and NHL were found and reviewed. Malignant lymphoma description was based on the International Lymphoma Study Group classification. Patients with SS are known to have an increased risk for developing B-cell NHL (B-NHL). However, such a complication occurs in less than 10% of patients, being reported mainly in those with primary SS. Extranodal low-grade B-NHL are observed most frequently. Persistent enlargement of parotid glands, adenopathy, monoclonal gammopathy, and cross-reactive idiotypes are all signs suggesting possible lymphoma evolution. Although monoclonality does not mean malignancy unequivocally, it is considered to be a precursor for NHL development in SS. Factors implicated into lymphomagenesis in SS include dysregulation in the mechanisms leading to apoptosis, hyperstimulation of B-1 cells, and an infectious agent. Polyclonal lymphoproliferation characterizing SS might in some instances transform into monoclonal, and then to malignancy. Further studies on the mechanism whereby NHL develops in SS are warranted.
Assuntos
Linfoma não Hodgkin/complicações , Síndrome de Sjogren/complicações , Idoso , Doenças Autoimunes/complicações , Doenças Autoimunes/imunologia , Humanos , Linfoma não Hodgkin/imunologia , Pessoa de Meia-Idade , Síndrome de Sjogren/imunologiaRESUMO
We live in a time of great biologic discovery, and this is especially true in the field of immunology. The rapidity with which creative thinking is translated into scientific observation in immunology has made it difficult even for workers in the field to keep abreast.