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Linfoma de células do manto : relação com hiperplasia nodular linfóide : a propósito de um caso clínico
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Introdução: Linfoma de Células do Manto (LCM) é um linfoma não Hodgkin de células B, cujo diagnóstico é feito geralmente em estadios avançados, em indivíduos do sexo masculino e com idade média de 60 anos. A Hiperplasia Nodular Linfóide (HNL), que a maioria das vezes aparece como um achado endoscópico, tem surgido como um possível fator de risco para o desenvolvimento de linfoma do trato gastrointestinal.
Caso Clínico: Doente de 48 anos, com história de diarreia crónica e emagrecimento, realizou uma colonoscopia que evidenciou lesões polipoides no íleon terminal e cujas biópsias sugeriram o diagnóstico de HNL. Cerca de três anos mais tarde, o doente recorreu ao serviço de urgência com dor abdominal e hematoquézias, detetando-se após investigação intussuscepção ileo-cólica por LCM.
Discussão e Conclusão: O trato gastrointestinal está descrito como podendo ser o local mais comum de envolvimento extraganglionar do LCM. Embora geralmente se manifeste como dor abdominal, diarreia ou perda de peso, a intussuscepção é uma apresentação possível sobretudo quando o linfoma se apresenta como polipose linfomatosa múltipla (PLM).
Nos adultos a HNL associa-se muitas vezes a outras patologias, desde imunodeficiências a infeções por Giardia lamblia ou Helicobacter pylori. Além disso, também tem sido sugerido que a HNL pode sofrer transformação maligna. Esta ideia é apoiada pelos vários casos em que a HNL coincidiu com o aparecimento posterior de linfoma, pelos casos em que se verifica histologicamente transição gradual de áreas de hiperplasia para neoplasia, bem como o próprio curso da doença com regressão aquando do tratamento do linfoma e reaparecimento aquando da recaída do mesmo. Ainda que não haja um tratamento comprovado para a HNL, tratar as possíveis patologias associadas é relativamente consensual. Nos casos sem patologias tem sido sugerida vigilância ou mesmo cirurgia de ressecção, devido ao risco de proliferação maligna.
Introduction: Mantle Cell Lymphoma (MCL) is a type of B-cell non-Hodgkin lymphoma. Its diagnosis is usually made only in advanced stages, at a median age of 60 years, mostly in men. Nodular lymphoid hyperplasia (NLH), which most often appears as an incidental finding on endoscopy, has emerged as a possible risk factor for the development of lymphoma on the gastrointestinal tract. Case Report: A 48-year-old patient with chronic diarrhea and weight loss underwent a colonoscopy. Polypoid lesions were observed in the terminal ileum. The result of the biopsies led to the diagnosis of NLH. Approximately three years later, the same patient was sent to the emergency department because of abdominal pain and hematochezia. After investigation, ileocecal intussusception by MCL was diagnosed. Discussion and Conclusion: The gastrointestinal tract is described as being the most common site of extranodal involvement in MCL. Although it usually manifests as abdominal pain, diarrhea, or weight loss, intussusception is a possible presentation, especially when the lymphoma presents itself as multiple lymphomatous polyposis. In adults, there is often an underlying cause for NLH, ranging from immunodeficiencies to Giardia Lamdlia or Helicobacter Pylori infections. In addition, it has also been suggested that NLH may undergo malignant transformation. This idea is corroborated by the several case reports in which NLH is associated with lymphoma, by the studies where a histologically gradual transition from the areas of hyperplasia to neoplasia was observed, and by the course of the disease itself, with regression at the time of lymphoma treatment and recurrence after relapse. Although there is no definitive treatment, it is relatively consensual that the underlying cause must be treated. However, the controversy arises in cases where there are no associated diseases. Surgical treatment may result in unnecessary radical surgery, but lack of treatment carries the risk of malignant progression.
Introduction: Mantle Cell Lymphoma (MCL) is a type of B-cell non-Hodgkin lymphoma. Its diagnosis is usually made only in advanced stages, at a median age of 60 years, mostly in men. Nodular lymphoid hyperplasia (NLH), which most often appears as an incidental finding on endoscopy, has emerged as a possible risk factor for the development of lymphoma on the gastrointestinal tract. Case Report: A 48-year-old patient with chronic diarrhea and weight loss underwent a colonoscopy. Polypoid lesions were observed in the terminal ileum. The result of the biopsies led to the diagnosis of NLH. Approximately three years later, the same patient was sent to the emergency department because of abdominal pain and hematochezia. After investigation, ileocecal intussusception by MCL was diagnosed. Discussion and Conclusion: The gastrointestinal tract is described as being the most common site of extranodal involvement in MCL. Although it usually manifests as abdominal pain, diarrhea, or weight loss, intussusception is a possible presentation, especially when the lymphoma presents itself as multiple lymphomatous polyposis. In adults, there is often an underlying cause for NLH, ranging from immunodeficiencies to Giardia Lamdlia or Helicobacter Pylori infections. In addition, it has also been suggested that NLH may undergo malignant transformation. This idea is corroborated by the several case reports in which NLH is associated with lymphoma, by the studies where a histologically gradual transition from the areas of hyperplasia to neoplasia was observed, and by the course of the disease itself, with regression at the time of lymphoma treatment and recurrence after relapse. Although there is no definitive treatment, it is relatively consensual that the underlying cause must be treated. However, the controversy arises in cases where there are no associated diseases. Surgical treatment may result in unnecessary radical surgery, but lack of treatment carries the risk of malignant progression.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2019
Palavras-chave
Linfoma de células do manto Hiperplasia nodular linfóide Intussuscepção Trato gastrointestinal Gastroenterologia