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Adenocarcinoma gástrico em doentes com imunodeficiência comum variável
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A Imunodeficiência Comum Variável (ICV) é a Imunodeficiência Primária sintomática mais frequente em adultos. Nesta existe um risco aumentado de 10-47 vezes de desenvolvimento de adenocarcinoma gástrico. Neste estudo retrospetivo, constituído por seis doentes com ICV e diagnóstico de adenocarcinoma, no período 2004-2018 no Centro Hospitalar Lisboa Norte - Portugal, tivemos como objetivo a sua caracterização clínica, imunofenotípica e estudo anatomopatológico de biópsias gástricas e peças cirúrgicas de gastrectomia. Para isso analisámos os processos clínicos, os relatórios de imunofenotipagem e anatomopatológicos dos doentes. Com uma amostra de 3 doentes do sexo masculino e 3 do sexo feminino (idade média de 43,3 anos), obtivemos como resultados um valor médio de diagnóstico de ICV aos 20,7 anos e das primeiras manifestações de ICV aos 9,3 anos. Em termos clínicos todos os doentes manifestaram patologia inserida em cinco categorias major: Infeção sem complicações; enteropatia; complicações autoimunes; complicações de infiltração linfocítica policlonal; e complicações neoplásicas. Quanto ao adenocarcinoma gástrico, este foi diagnosticado em média aos 40,5 anos. Em 5 doentes foi observada infeção por Helicobacter pylori (H. pylori), tendo as biópsias gástricas vários tipos de alterações, desde gastrite crónica, gastrite atrófica, metaplasia intestinal, displasia ou adenocarcinoma. Foram diagnosticados dois adenocarcinomas do tipo intestinal, dois do tipo difuso e dois adenocarcinomas do tipo indeterminado, sendo que em quatro casos (gastrectomia) existiam alterações morfológicas na região peritumoral e em outras áreas da mucosa gástrica, mas com menos gravidade do que na área tumoral. Fica assim patente, a enorme diversidade de subtipos de adenocarcinoma gástricos em doentes com ICV. O recurso a estudos moleculares poderá ajudar na explicação do processo de carcinogénese gástrica associada a ICV.
Common Variable Immunodeficiency (CVID) is the most frequent symptomatic primary immunodeficiency in adults. It is associated with an increased risk of 10-47 times of developing a gastric adenocarcinoma. This retrospective study, that comprises six CVID patients with a diagnosis of adenocarcinoma, between 2004 and 2018 in the Centro Hospitalar Lisboa Norte - Portugal, aims to clinically and immunophenotypically characterize these patients, and to pathologically study their gastric biopsies and gastrectomy resection specimen. Therefore, we analysed the patients’ clinical records, immunephenotyping reports and pathological reports. Our sample of 3 male and 3 female patients (mean of 43,3 years old), were diagnosed with CVID at a mean age of 20,7 years old, and had their first manifestations at 9,3 years of age. Clinically, all patients had their pathology included in five major categories: infection without complications, enteropathy, autoimmunity complications, polyclonal lymphocytic infiltration and malignancies complications. Regarding the gastric adenocarcinoma, it was diagnosed at a mean age of 40,5 years of age. Five patients had Helicobacter pylori (H. pylori) infection and their gastric biopsies showed various alterations, including chronic gastritis, atrophic gastritis, intestinal metaplasia, dysplasia and adenocarcinoma. Two intestinal, two diffuse and two undetermined adenocarcinoma types were diagnosed, and in four cases (gastrectomy) there were morphological changes in the peritumoral region and in other areas of the gastric mucosa, although less severe than in the tumoral region. It is thus clear the great diversity of gastric adenocarcinoma subtypes in CVID patients. The use of molecular studies may help explain the process of gastric carcinogenesis associated with CVID.
Common Variable Immunodeficiency (CVID) is the most frequent symptomatic primary immunodeficiency in adults. It is associated with an increased risk of 10-47 times of developing a gastric adenocarcinoma. This retrospective study, that comprises six CVID patients with a diagnosis of adenocarcinoma, between 2004 and 2018 in the Centro Hospitalar Lisboa Norte - Portugal, aims to clinically and immunophenotypically characterize these patients, and to pathologically study their gastric biopsies and gastrectomy resection specimen. Therefore, we analysed the patients’ clinical records, immunephenotyping reports and pathological reports. Our sample of 3 male and 3 female patients (mean of 43,3 years old), were diagnosed with CVID at a mean age of 20,7 years old, and had their first manifestations at 9,3 years of age. Clinically, all patients had their pathology included in five major categories: infection without complications, enteropathy, autoimmunity complications, polyclonal lymphocytic infiltration and malignancies complications. Regarding the gastric adenocarcinoma, it was diagnosed at a mean age of 40,5 years of age. Five patients had Helicobacter pylori (H. pylori) infection and their gastric biopsies showed various alterations, including chronic gastritis, atrophic gastritis, intestinal metaplasia, dysplasia and adenocarcinoma. Two intestinal, two diffuse and two undetermined adenocarcinoma types were diagnosed, and in four cases (gastrectomy) there were morphological changes in the peritumoral region and in other areas of the gastric mucosa, although less severe than in the tumoral region. It is thus clear the great diversity of gastric adenocarcinoma subtypes in CVID patients. The use of molecular studies may help explain the process of gastric carcinogenesis associated with CVID.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2018
Palavras-chave
Imunodeficiência comum variável Adenocarcinoma gástrico Helicobacter pylori Imunologia