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[在胃肠结肠区域活检中诊断出的滤泡性和套细胞淋巴瘤]

[Follicular and mantle cell lymphoma diagnosed in biopsies of gastroenterocolic region].

作者信息

Plank Lukáš, Balhárek Tomáš, Szépe Peter

出版信息

Cesk Patol. 2016;52(1):31-9.

Abstract

The authors present a retrospective analysis of follicular lymphomas (FL) and mantle cell lymphomas (MCL) diagnosed according to the WHO classification (2008) in consecutive biopsies of GI organs in a period of 11 years. The series includes 18 patients with FL verified in 22 biopsies and 44 patients with MCL diagnosed in 54 biopsies. FL represented always a solitary tumor, most often - up to ¾ of all the cases - of a small intestine, more often in its jejunoileal than duodenal parts. The biopsies were obtained almost equally by endoscopical approach, they were usually mucosal and rarely polypectomic, as well as by surgical resections (54,5 % and 45,5 % of the cases respectively). FL of grade 3 was identified in approximately 11 % of the cases, while majority of the patients showed FL of grade 1 or 2. Only 2 patients with duodenal FL relapsed and bioptically verified recidives did not show signs of a high grade transformation. Although it was difficult to identify a nodular growth pattern in more common small biopsies, a typical histomorphology and phenotype mostly allowed the FL diagnosis in the majority of the cases. The FL diagnosis had to be supported by detection of BCL2 translocation only in one case. MCL appeared most often in the stomach and large intestine, the small intestinal cases represented less than 23 %. In ¼ of the patients the lymphoma was multifocal and manifested as lymphomatoid polyposis affecting most often both large and small intestine. In a majority of the MCL patients, the diagnosis was done in mucosal and polypectomic endoscopic biopsies, surgical intervention and resection was recorded in less than 10 % of the cases. Most of the patients showed conventional "centrocytic" MCL morphology and approximately ¼ of the cases showed blastoid MCL. The rebiopsies of 9 patients revealed a relaps of the disease which was locoidentical in 5 of them; in other 4 patients the biopsies documented a dissemination to other GI organs. The blastic transformation was recognized only in one of the relapsing patients. In contrast to FL, in 18 % of the biopsies the MCL diagnosis required a CCND1 gene rearrangement detection by FISH. In summary both of the studied GI lymphomas showed different and heterogenous incidence, clinical manifestation and necessity to support the biopsy diagnosis also by FISH examination. The correct FL and MCL diagnosis represents a conditio sine qua non for an indication of different therapeutical modalities as well as for the prognostic stratification of the patients.

摘要

作者对11年间连续进行的胃肠道器官活检中根据世界卫生组织(2008年)分类诊断的滤泡性淋巴瘤(FL)和套细胞淋巴瘤(MCL)进行了回顾性分析。该系列包括在22次活检中确诊的18例FL患者和在54次活检中诊断的44例MCL患者。FL总是表现为孤立性肿瘤,在所有病例中,多达3/4的肿瘤位于小肠,且更多见于空肠回肠段而非十二指肠段。活检标本几乎同样通过内镜途径获取,通常为黏膜活检,很少为息肉切除术,也有通过手术切除获取(分别占病例的54.5%和45.5%)。约11%的病例为3级FL,而大多数患者表现为1级或2级FL。仅2例十二指肠FL患者复发,活检证实的复发未显示高级别转化迹象。尽管在较常见的小活检标本中难以识别结节状生长模式,但典型的组织形态学和表型在大多数病例中大多能确诊FL。仅1例病例的FL诊断需通过检测BCL2易位来支持。MCL最常出现在胃和大肠,小肠病例占比不到23%。1/4的患者淋巴瘤为多灶性,表现为淋巴瘤样息肉病,最常累及大肠和小肠。大多数MCL患者通过黏膜和息肉切除内镜活检确诊,手术干预和切除记录在不到病例数的10%。大多数患者表现为传统的“中心细胞型”MCL形态,约1/4的病例表现为母细胞样MCL。9例患者的再次活检显示疾病复发,其中5例复发部位相同;其他4例患者的活检记录显示疾病已扩散至其他胃肠道器官。仅1例复发患者出现母细胞转化。与FL不同,18%的活检标本中MCL诊断需要通过荧光原位杂交(FISH)检测CCND1基因重排。总之,两种研究的胃肠道淋巴瘤在发病率、临床表现以及通过FISH检查支持活检诊断的必要性方面均表现出不同和异质性。正确诊断FL和MCL是指示不同治疗方式以及对患者进行预后分层的必要条件。

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