Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project
Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project
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DOI：
10.1182/blood-2011-02-335216
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发表时间：
2011-07-07
期刊：
影响因子：
20.3
通讯作者：
Weisenburger, Dennis D.
中科院分区：
文献类型：
--
作者：
Delabie, Jan;Holte, Harald;Weisenburger, Dennis D.
很少有关于肠病相关 T 细胞淋巴瘤 (EATL) 的大型国际系列报道。我们研究了来自全球 22 个中心的 1153 名外周 T 细胞或自然杀伤 (NK) 细胞淋巴瘤患者中的 62 名 EATL 患者。该诊断是由 4 名血液病理学家专家组成的共识小组根据世界卫生组织 (WHO) 标准做出的。进行了临床相关性和生存分析。 EATL 占研究中所有淋巴瘤的 5.4%，在欧洲最常见（9.1%），其次是北美（5.8%）和亚洲（1.9%）。 EATL 1 型（66%）比 2 型（34%）更常见，并且在欧洲尤其常见（79%）。 32.2% 的患者被临床诊断为乳糜泻，且与 EATL 1 型和 2 型相关。中位总生存期仅为 10 个月，中位无失败生存期仅为 6 个月。国际预后指数 (IPI) 的生存预测指标不如外周 T 细胞淋巴瘤预后指数 (PIT)。临床口炎性腹泻预测不良生存率与 PIT 无关。 EATL 亚型和其他生物学参数都不能准确预测生存率。我们的研究证实了 EATL 患者的预后不良以及需要改进的治疗方案。 （血。2011；118（1）：148-155）
Few large, international series of enteropathy-associated T-cell lymphoma (EATL) have been reported. We studied a cohort of 62 patients with EATL among 1153 patients with peripheral T-cell or natural killer (NK)-cell lymphoma from 22 centers worldwide. The diagnosis was made by a consensus panel of 4 expert hematopathologists using World Health Organization (WHO) criteria. Clinical correlations and survival analyses were performed. EATL comprised 5.4% of all lymphomas in the study and was most common in Europe (9.1%), followed by North America (5.8%) and Asia (1.9%). EATL type 1 was more common (66%) than type 2 (34%), and was especially frequent in Europe (79%). A clinical diagnosis of celiac sprue was made in 32.2% of the patients and was associated with both EATL type 1 and type 2. The median overall survival was only 10 months, and the median failure-free survival was only 6 months. The International Prognostic Index (IPI) was not as good a predictor of survival as the Prognostic Index for Peripheral T-Cell Lymphoma (PIT). Clinical sprue predicted for adverse survival independently of the PIT. Neither EATL subtype nor other biologic parameters accurately predicted survival. Our study confirms the poor prognosis of patients with EATL and the need for improved treatment options. (Blood. 2011;118(1):148-155)