ALK-anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified:: report from the International Peripheral T-Cell Lymphoma Project
ALK-anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified:: report from the International Peripheral T-Cell Lymphoma Project
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DOI：
10.1182/blood-2008-01-134270
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发表时间：
2008-06-15
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：
Savage, Kerry J.;Harris, Nancy Lee;Weisenburger, Dennis D.
国际外周T细胞淋巴瘤项目是一项旨在更好地了解外周T细胞和自然杀伤（NK）/T细胞淋巴瘤（PTCL）的合作努力。北美、欧洲和亚洲共有22家机构提交了在各自中心诊断和治疗的PTCL的临床和病理信息。在1314例合格患者中，181例在共识审查时患有间变性大细胞淋巴瘤（ALCL; 13.8%）：159例患有系统性ALCL（12.1%），22例患有原发性皮肤ALCL（1.7%）。间变性淋巴瘤激酶阳性（ALK（+））ALCL患者的结局上级ALK（-）ALCL患者（5年无失败生存率[FFS]，60% vs 36%; P = 0.015; 5年总生存率[OS]，70% vs 49%; P = 0.016）。然而，与既往报告相反，ALK（-）ALCL的5年FFS（36% vs 20%; P = 0.012）和OS（49% vs 32%; P = 0.032）上级PTCL，未另行说明（PTCL-NOS）。原发性皮肤ALCL患者的5年OS非常有利（90%），但有复发倾向（5年FFS，55%）。总之，ALK（-）ALCL应继续与ALK（+）ALCL和PTCL-NOS分开。虽然ALK（-）ALCL的预后似乎优于PTCL-NOS，但仍不令人满意，需要更好的治疗。原发性皮肤ALCL与无痛病程相关。
The International Peripheral T-Cell Lymphoma Project is a collaborative effort designed to gain better understanding of peripheral T-cell and natural killer (NK)/T-cell lymphomas (PTCLs). A total of 22 institutions in North America, Europe, and Asia submitted clinical and pathologic information on PTCLs diagnosed and treated at their respective centers. Of the 1314 eligible patients, 181 had anaplastic large-cell lymphoma (ALCL; 13.8%) on consensus review: One hundred fifty-nine had systemic ALCL (12.1%) and 22 had primary cutaneous ALCL (1.7%). Patients with anaplastic lymphoma kinase-positive (ALK(+)) ALCL had a superior outcome compared with those with ALK(-)ALCL (5-year failure-free survival [FFS], 60% vs 36%; P =.015; 5-year overall survival [OS], 70% vs 49%; P =.016). However, contrary to prior reports, the 5-year FFS (36% vs 20%; P =.012) and OS (49% vs 32%; P =.032) were superior for ALK(-)ALCL compared with PTCL, not otherwise specified (PTCL-NOS). Patients with primary cutaneous ALCL had a very favorable 5-year OS (90%), but with a propensity to relapse (5-year FFS, 55%). In summary, ALK(-)ALCL should continue to be separated from both ALK(+) ALCL and PTCL-NOS. Although the prognosis of ALK(-) ALCL appears to be better than that for PTCL-NOS, it is still unsatisfactory and better therapies are needed. Primary cutaneous ALCL is associated with an indolent course.