原发性中枢神经系统无性大细胞淋巴瘤的临床病理特征:一项多中心研究发现年龄和ALK状态是预后因素。

IF 0.6 4区 医学 Q4 HEMATOLOGY
William Patrick Morrow, Nicholas S Milligan, Robert S Ohgami, Ken H Young, Bangchen Wang, Francisco Vega, Mario L Marques-Piubelli, Andrew L Feldman, Graham W Slack, Kerry J Savage, Xiaoxian Zhao, James L Rubenstein, Eric D Hsi
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引用次数: 0

摘要

原发于中枢神经系统且病变局限于中枢神经系统的无性大细胞淋巴瘤(原发性中枢神经系统 ALCL)是一种罕见的侵袭性淋巴瘤,好发于敏感的解剖部位。我们报告了六个学术医疗中心新近报告的 17 例原发性中枢神经系统 ALCL 的临床和病理特征。我们正在研究这些病例的特征,以及它们与发生在传统解剖部位的全身性 ALCL 的共同点和不同点。我们通过病历审查提取了临床、病理和结果数据。患者年龄中位数为32岁,男女比例为2.4:1。病例表现为局部或多灶性中枢神经系统(CNS)疾病,但不伴有全身性疾病。在组织学上,常见的模式是泛T细胞标志物缺失。原发性中枢神经系统 ALCLL 中无性淋巴瘤激酶(ALK)阳性的比例(12/17,71%)与全身性 ALCLL 中无性淋巴瘤激酶阳性的比例(70-80%)相似。我们的数据显示,5年总生存(OS)率为65%,5年无进展生存(PFS)率为48%。本研究中共有5名患者死亡,其中全部为ALK阴性组患者,且年龄均超过40岁。ALK阳性患者明显比ALK阴性患者年轻,生存期分析表明,ALK阳性和年龄较小(≤40岁)都是有利的预后因素。这是迄今为止报道的最大规模的原发性中枢神经系统ALCL系列病例,表明ALK阳性病例比例很高,尽管累及敏感的解剖部位,但年轻患者和ALK阳性患者的预后都很好。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Clinicopathologic features of primary central nervous system anaplastic large cell lymphoma: a multicenter study identifies age and ALK status as prognostic factors.

Anaplastic large cell lymphoma with primary presentation in, and disease limited to, the central nervous system (primary CNS ALCL) is a rare and aggressive lymphoma found in a sensitive anatomic site. We report the clinical and pathologic characteristics of 17 primary CNS ALCL cases that are newly reported from six academic medical centers. We are investigating the characteristics of these cases, alongside their commonalities and differences from systemic ALCL arising at conventional anatomic sites. Clinical, pathologic, and outcome data were extracted by medical record review. The median patient age was 32 years with a male-to-female ratio of 2.4:1. Cases presented with either localized or multifocal central nervous system (CNS) disease without coinciding systemic disease. Histologically, the common pattern prevailed, and loss of pan-T-cell markers was frequent. There was a similar proportion of anaplastic lymphoma kinase (ALK) positivity in primary CNS ALCL (12/17, 71%) compared to that reported in systemic ALCL (70-80%). Our data indicate a 5-year overall survival (OS) rate of 65% and a 5-year progression-free survival (PFS) rate of 48%. Five patient deaths occurred in this study of which all were in the ALK-negative group, and all were patients over 40 years old. ALK-positive patients were significantly younger than ALK-negative patients, and survival analyses showed that both ALK-positive and younger age (≤ 40 years) were favorable prognostic factors. This is the largest series of primary CNS ALCL reported to date, which demonstrates a high proportion of ALK-positive cases and favorable outcomes for both younger and ALK-positive patients despite the involvement of a sensitive anatomic site.

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来源期刊
Journal of Hematopathology
Journal of Hematopathology HEMATOLOGYPATHOLOGY-PATHOLOGY
CiteScore
0.80
自引率
0.00%
发文量
45
期刊介绍: The Journal of Hematopathology aims at providing pathologists with a special interest in hematopathology with all the information needed to perform modern pathology in evaluating lymphoid tissues and bone marrow. To this end the journal publishes reviews, editorials, comments, original papers, guidelines and protocols, papers on ancillary techniques, and occasional case reports in the fields of the pathology, molecular biology, and clinical features of diseases of the hematopoietic system. The journal is the unique reference point for all pathologists with an interest in hematopathology. Molecular biologists involved in the expanding field of molecular diagnostics and research on lymphomas and leukemia benefit from the journal, too. Furthermore, the journal is of major interest for hematologists dealing with patients suffering from lymphomas, leukemias, and other diseases. The journal is unique in its true international character. Especially in the field of hematopathology it is clear that there are huge geographical variations in incidence of diseases. This is not only locally relevant, but due to globalization, relevant for all those involved in the management of patients.
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