International peripheral T-cell and natural killer/T-cell lymphoma study: Pathology findings and clinical outcomes
International peripheral T-cell and natural killer/T-cell lymphoma study: Pathology findings and clinical outcomes
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DOI：
10.1200/jco.2008.16.4558
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发表时间：
2008-09-01
影响因子：
45.3
通讯作者：
Harris, Mildred E.
中科院分区：
文献类型：
--
作者：
Vose, Julie M.;Neumann, M.;Harris, Mildred E.
目的外周T细胞淋巴瘤（PTCL）和自然杀伤/T细胞淋巴瘤（NKTCL）是非霍奇金淋巴瘤（NHL）中罕见的异质性类型，一般与不良临床结局相关。包括1990年至2002年期间诊断的既往未经治疗的PTCL或NKTCL患者。组织活检，免疫表型标志物，分子遗传学研究，并从连续的患者在每个站点的临床信息进行了审查，由四个专家血液病理学家小组和分类根据WHO classification.Results PTCL或NKTCL的诊断确认在1,153例（87.8%）。最常见的亚型为PTCL（未另行说明）（NOS; 25.9%）、血管免疫母细胞型（18.5%）、NKTCL（10.4%）和成人T细胞白血病/淋巴瘤（ATLL; 9.6%）。10.4%的病例发生错误分类，包括霍奇金淋巴瘤（3%）、B细胞淋巴瘤（1.4%）、无法分类的淋巴瘤（2.8%）或淋巴瘤以外的诊断（2.3%）。我们发现不同地理区域的各种亚型的频率有显著差异。蒽环类药物治疗方案的使用与PTCL-NOS或血管免疫母细胞型的预后改善无关，但与间变性大细胞淋巴瘤ALK阳性的预后改善相关。结论WHO分类有助于确定PTCL和NKTCL的亚型。然而，专家血液病理学审查是重要的准确诊断。大多数这些淋巴瘤亚型患者的临床结果与标准治疗是差的，需要新的药物和新的方式来提高生存率。
Purpose Peripheral T-cell lymphoma (PTCL) and natural killer/T-cell lymphoma (NKTCL) are rare and heterogeneous forms of non-Hodgkin's lymphoma (NHL) that, in general, are associated with a poor clinical outcome.Patients and Methods A cohort of 1,314 cases of PTCL and NKTCL was organized from 22 centers worldwide, consisting of patients with previously untreated PTCL or NKTCL who were diagnosed between 1990 and 2002. Tissue biopsies, immunophenotypic markers, molecular genetic studies, and clinical information from consecutive patients at each site were reviewed by panels of four expert hematopathologists and classified according to the WHO classification.Results A diagnosis of PTCL or NKTCL was confirmed in 1,153 (87.8%) of the cases. The most common subtypes were PTCL not otherwise specified (NOS; 25.9%), angioimmunoblastic type (18.5%), NKTCL (10.4%), and adult T-cell leukemia/ lymphoma (ATLL; 9.6%). Misclassification occurred in 10.4% of the cases including Hodgkin's lymphoma (3%), B-cell lymphoma (1.4%), unclassifiable lymphoma (2.8%), or a diagnosis other than lymphoma (2.3%). We found marked variation in the frequency of the various subtypes by geographic region. The use of an anthracycline-containing regimen was not associated with an improved outcome in PTCL-NOS or angioimmunoblastic type, but was associated with an improved outcome in anaplastic large-cell lymphoma, ALK positive.Conclusion The WHO classification is useful for defining subtypes of PTCL and NKTCL. However, expert hematopathology review is important for accurate diagnosis. The clinical outcome for patients with most of these lymphoma subtypes is poor with standard therapies, and novel agents and new modalities are needed to improve survival.