Duodenal-type follicular lymphoma (DFL) is a newly recognised variant of follicular lymphoma (FL), although little is known about its biology and clinical evolution. In general, patients tend to have mild symptoms and do not require therapy, comparable with other forms of low-tumour burden asymptomatic FL. Specific pathological features, such as a dendritic cell meshwork, low expression of CD10 and upregulation of activation-induced cytidine deaminase can help the diagnosis. The molecular landscape of DFL is similar to the classical nodal presentation of FL, although studies using gene expression profiling demonstrate a close relation with MALT lymphomas. Markers associated with inflammation have suggested that the microenvironment plays a likely role in the pathogenesis of DFL and its low progression rate. Clinical series published vary between 20-63 patients with an estimated overall survival between 92-100% and a median follow-up ranging between 20 and 107 months. Treatment options include a watch and wait strategy, rituximab monotherapy and radiotherapy. In this review, we summarise current pathological data and treatment studies in DFL.

中文翻译：

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我们对十二指肠型滤泡性淋巴瘤了解多少？从病理学定义到治疗选择。

十二指肠型滤泡性淋巴瘤（DFL）是一种新发现的滤泡性淋巴瘤（FL）变体，尽管对其生物学和临床进展知之甚少。通常，与其他形式的低肿瘤负荷无症状FL相比，患者倾向于有轻度症状且不需要治疗。特定的病理特征，例如树突状细胞网状结构，CD10的低表达和激活诱导的胞苷脱氨酶的上调可以帮助诊断。DFL的分子结构类似于FL的经典淋巴结表现，尽管使用基因表达谱的研究表明与MALT淋巴瘤密切相关。与炎症相关的标记表明，微环境可能在DFL的发病机理及其低进展率中发挥了作用。已发表的临床系列研究在20-63位患者之间有所不同，估计总体生存率在92-100％之间，中位随访时间在20-107个月之间。治疗选择包括观察和等待策略，利妥昔单抗单药治疗和放疗。在这篇综述中，我们总结了DFL中当前的病理数据和治疗研究。