ABSTRACT
ABSTRACT Intrahepatic biliary proliferations represent a spectrum from reactive (ductular reaction, some with atypical architecture), hamartomatous (von Meyenburg complex), benign (bile duct adenoma) and precursor/borderline entities (biliary intraepithelial neoplasia, intraductal papillary neoplasm of the bile duct) to fully malignant (cholangiocarcinoma) neoplasms. Clinical pictures and even imaging patterns may be similar, requiring refined studies aiming at histopathological and immunohistochemistry for more precise diagnosis, essential for correct patient management. This article discusses updated concepts and definitions of most relevant entities aiming more specifically at the differential diagnosis in practice, focusing on morphology and immunohistochemistry, with a discussion of potential markers to help distinguishing between benign and malignant lesions.
RESUMO As proliferações biliares intra-hepáticas representam um espectro que abrange desde entidades reativas (reação ductular, algumas com arquitetura atípica), hamartomatosas (complexo de von Meyenburg), benignas (adenoma de ductos biliares) e precursoras/limítrofes (neoplasia intraepitelial biliar, neoplasia papilar intraductal de ducto biliar) até neoplasias totalmente malignas (colangiocarcinoma). Os quadros clínicos e até mesmo os padrões de imagem podem ser semelhantes, exigindo estudos refinados visando critério histológicos e imuno-histoquímicos para diagnósticos mais precisos, essenciais para o correto manejo do paciente. Este artigo discute conceitos atualizados e definições das entidades mais relevantes visando mais especificamente ao diagnóstico diferencial na prática, com foco na morfologia e imuno-histoquímica, com discussão de potenciais marcadores para ajudar na distinção entre lesões benignas e malignas.
ABSTRACT
ABSTRACT Introduction: Hemophilia is well known in males, but poorly recognized in hemophilia carriers, who may have a hemorrhagic tendency, and the symptoms may be frequent and severe. Few studies have been done evidencing this bleeding in female carriers of the hemophilia gene. Objectives: To verify the prevalence of hemorrhagic symptoms in HC, compared to women in the general population. Material and method: The articles published between October 1996 and November 2016 were searched in the PubMed, Scielo, Lilacs, Web of Science, Scopus and Cochrane Central databases. Results and discussion: Seventy-five articles were found in electronic databases and 2 additional articles, through manual search in journal summaries and bibliographical references of other review articles. There is a limitation as to the number of studies that explore the association between the risk of hemorrhagic events and HC A or B. Among the few existing studies, there is a methodological difference, evidenced by control groups with distinct recruitments, divergent questionnaires and non-standardized concepts. Conclusion: This review verified the existence of a higher prevalence of hemorrhagic symptoms in the HC in some outcomes, however, due to the limitations of the few studies found, there is still insufficient evidence to state that the HC has a greater hemorrhagic tendency in relation to the general population.