Primary gastrointestinal anaplastic large cell lymphoma
Primary gastrointestinal anaplastic large cell lymphoma
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DOI：
10.1016/j.pathol.2017.05.007
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发表时间：
2017-08-01
期刊：
影响因子：
4.5
通讯作者：
Chuang, Shih-Sung
中科院分区：
文献类型：
--
作者：
Lee, Yi-Ying;Takata, Katsuyoshi;Chuang, Shih-Sung
原发性胃肠道间变性大细胞淋巴瘤(GI-ALCL)是一种罕见的疾病。我们报告了8个新病例。中位年龄61.5岁(10-88岁)，最常累及胃部(n=3)和小肠(n=4)。所有病例的肿瘤标志细胞均表达CD30。间变性淋巴瘤激酶(ALK)蛋白表达2例(25%)。原位杂交均为EB病毒阴性，dusp22/IRF4基因易位均为阴性。中位随访时间为37.5个月，4名患者死于疾病，1名患者带病存活，3名患者无病。我们的文献回顾显示，GI-ALCL主要发生在老年患者，其特点是ALK低表达，T细胞谱系比例高，并且经常发生在小肠。结合我们的两例ALK(+)GI-ALCL病例和文献中的四例病例，中位年龄为34岁(10-56岁)，其中4例(67%)发生在小肠。6名患者均存活，中位随访时间为21个月。我们的6例ALK(-)GI-ALCL患者的5年总存活率为40%，而ALK(+)GI-ALCL患者的5年生存率为100%。ALK-GI-ALCL预后较差，而ALK+预后较好。
Primary gastrointestinal anaplastic large cell lymphoma (GI-ALCL) is rare. We report eight new cases. The median age was 61.5 years (range 10-88), most frequently involving the stomach (n = 3) and small intestine (n = 4). The neoplastic hallmark cells in all cases expressed CD30. Anaplastic lymphoma kinase (ALK) protein was expressed in two cases (25%). By in situ hybridisation, all cases were negative for Epstein-Barr virus and for DUSP22/IRF4 gene translocation. At a median follow-up time of 37.5 months, four patients died of disease, one was alive with disease, and three were disease-free. Our literature review showed that GI-ALCL occurred mainly in older patients and was characterised by a low rate of ALK expression, a high rate of T-cell lineage, and a frequent occurrence in the small intestine. Incorporating our two ALK(+) GI-ALCL cases together with the four cases in the literature, the median age was 34 years (range 10-56), with four (67%) cases in the small intestine. The six patients were all alive with a median follow-up of 21 months. The 5-year overall survival of our six patients with ALK(-) GI-ALCL was 40%, in contrast to 100% with ALK(+) GI-ALCL. The prognosis for ALK -GI-ALCL was poor, while that for the ALK+ counterparts was good.