文章：

原发性与继发性胃肠道套细胞淋巴瘤的临床特征及预后

Clinical characteristics and outcomes of primary versus secondary gastrointestinal mantle cell lymphoma

原文发布日期：2021-01-07

DOI: 10.1038/s41408-020-00394-z

类型: Article

开放获取: 是

英文摘要：

Primary gastrointestinal (GI) mantle cell lymphoma (MCL) is rare and the optimal management is unknown. We reviewed 800 newly diagnosed MCL cases and found 22 primary (2.8%) and 79 (9.9%) secondary GI MCL cases. Age, sex, and performance status were similar between primary and secondary cases. Secondary cases had more elevations in lactate dehydrogenase (28% vs 0%, P = 0.03) and a trend for a higher MCL international prognostic index (P = 0.07). Observation or local therapy was more common for primary GI MCL (29% vs 8%, P < 0.01), and autologous stem-cell transplant was more common for secondary GI MCL (35% vs 14%, P < 0.05). The median follow-up was 85 months. Primary and secondary GI MCL had similar 5-year progression-free survival (PFS) (30% vs 28%, P = 0.59) and overall survival (OS) (65% vs 66%, P = 0.83). The extent of GI involvement in primary GI MCL affected treatment selection but not outcome, with a 5-year PFS of 43% vs 14% vs 31% (P = 0.48) and OS of 57% vs 71% vs 69% (P = 0.54) in cases with single lesion vs multiple lesions in 1 organ vs multiple lesions in ≥2 organs. Less aggressive frontline treatment for primary GI MCL is reasonable. It is unknown whether more aggressive treatment can result in improved outcomes.
 

摘要翻译： 

原发性胃肠道套细胞淋巴瘤（MCL）罕见，其最佳治疗方案未知。我们回顾了800例新诊断的MCL病例，发现22例（2.8%）原发性和79例（9.9%）继发性胃肠道MCL。原发性和继发性病例在年龄、性别和体能状态方面相似。继发性病例的乳酸脱氢酶升高更常见（28% vs 0%，P=0.03），且MCL国际预后指数有更高趋势（P=0.07）。观察或局部治疗在原发性胃肠道MCL中更常见（29% vs 8%，P<0.01），而自体干细胞移植在继发性胃肠道MCL中更常见（35% vs 14%，P<0.05）。中位随访时间为85个月。原发性和继发性胃肠道MCL的5年无进展生存期（30% vs 28%，P=0.59）和总生存期（65% vs 66%，P=0.83）相似。原发性胃肠道MCL的胃肠道受累范围影响治疗选择但不影响结局，单发病灶、单一器官多发病灶和两个或以上器官多发病灶的5年无进展生存期分别为43%、14%和31%（P=0.48），总生存期分别为57%、71%和69%（P=0.54）。对原发性胃肠道MCL采用较少积极的一线治疗是合理的，尚不清楚更积极的治疗是否能改善结局。

原文链接：

Clinical characteristics and outcomes of primary versus secondary gastrointestinal mantle cell lymphoma