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间变性大细胞淋巴瘤的生存故事——有时不仅仅取决于间变性淋巴瘤激酶状态:102例病例的病理预后因素评估

Story of survival in anaplastic large cell lymphoma - sometimes more than the anaplastic lymphoma kinase status: An evaluation of pathologic prognostic factors in 102 cases.

作者信息

Agrawal Komal, Shet Tanuja, Sridhar Epari, Dhende Suhas, Sengar Manju, Arora Brijesh, Laskar Siddhartha, Gujral Sumeet, Menon Hari, Banavali Shripad

机构信息

Department of Pathology, Tata Memorial Hospital, Mumbai, Maharashtra, India.

Department of Medical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India.

出版信息

Indian J Pathol Microbiol. 2017 Oct-Dec;60(4):533-540. doi: 10.4103/IJPM.IJPM_778_15.

DOI:10.4103/IJPM.IJPM_778_15
PMID:29323068
Abstract

INTRODUCTION

Systemic anaplastic large cell lymphoma (ALCL) accounts for 5%-10% of adult non Hodgkin's lymphoma (NHL) and 10%-30% of childhood NHL. Owing to significant differences in survival and gene expression profile, current WHO classifies ALCL into two distinct entities as anaplastic lymphoma receptor tyrosine kinase (ALK) positive and ALK negative ALCL with ALK expression by tumour as a good prognostic indicator. However, in our institute which is a cancer referral institute, our preliminary experience was that even ALK positive tumours did not fare well as compared to ALK- negative ALCL. So, the current study aims at exploring more clinical and pathological factors impacting survival in ALCL patients.

OBJECTIVE

To study clinical and pathological prognostic factors in cases of ALCL.

METHODS

102 cases of ALCL were retrieved from pathology database. Pathological features and clinical features of these cases were recorded and factors found to impact overall survival (OAS) and disease-free survival (DFS) curves were identified based on univariate and multivariate analysis.

RESULTS

ALK 1 expression was seen in 71/102 (69.6%) cases and was not found to impact OAS or DFS. The 2 year OAS rate for ALK positive patients was 63.5% and DFS rate was 54.4%, while for ALK negative patients, the OAS was 60.5% and DFS was 43.5%. The Ann Arbor stage, performance status, international prognostic index, histological subtype, and the degree of the background inflammatory infiltrate were found to impact the OAS significantly. Increased reactive inflammatory component also negatively impacted DFS. In the multivariate analysis, only the histologic type emerged as significant for OAS.

CONCLUSION

Though ALK plays a role in prognostication of systemic ALCL, advanced stage disease and an inflammatory milieu may modulate the final outcome. We report a study of clinical and pathologic prognostic features in 102 cases of anaplastic large cell lymphoma (ALCL) from a cancer referral institute in India. Anaplastic lymphoma receptor tyrosine kinase (ALK-1) expression was seen in 71/102 (69.6%) cases and was not found to impact overall survival (OAS) or disease-free survival (DFS). The 2-year OAS rate for ALK-positive patients was 63.5% and DFS rate was 54.4%, while for ALK-negative patients, the OAS was 60.5% and DFS was 43.5%. The Ann Arbor stage, performance status, international prognostic index, histological subtype, and the degree of the background inflammatory infiltrate were found to impact the OAS significantly. Increased reactive inflammatory component also negatively impacted DFS. In the multivariate analysis, only the histologic type emerged as significant for OAS. Thus, though ALK plays a role in prognostication of systemic ALCL, advanced stage disease and an inflammatory milieu may modulate the final outcome.

摘要

引言

系统性间变性大细胞淋巴瘤(ALCL)占成人非霍奇金淋巴瘤(NHL)的5%-10%,占儿童NHL的10%-30%。由于生存率和基因表达谱存在显著差异,目前世界卫生组织将ALCL分为两个不同的实体,即间变性淋巴瘤受体酪氨酸激酶(ALK)阳性和ALK阴性ALCL,肿瘤ALK表达是一个良好的预后指标。然而,在我们这家癌症转诊机构,我们的初步经验是,与ALK阴性ALCL相比,即使是ALK阳性肿瘤的预后也不佳。因此,本研究旨在探索更多影响ALCL患者生存的临床和病理因素。

目的

研究ALCL病例的临床和病理预后因素。

方法

从病理数据库中检索出102例ALCL病例。记录这些病例的病理特征和临床特征,并基于单因素和多因素分析确定影响总生存(OAS)和无病生存(DFS)曲线的因素。

结果

102例中有71例(69.6%)出现ALK 1表达,未发现其影响OAS或DFS。ALK阳性患者的2年OAS率为63.5%,DFS率为54.4%,而ALK阴性患者的OAS为60.5%,DFS为43.5%。发现Ann Arbor分期、体能状态、国际预后指数、组织学亚型和背景炎症浸润程度对OAS有显著影响。反应性炎症成分增加也对DFS有负面影响。在多因素分析中,只有组织学类型对OAS有显著意义。

结论

虽然ALK在系统性ALCL的预后评估中起作用,但晚期疾病和炎症环境可能会调节最终结果。我们报告了一项对印度一家癌症转诊机构102例间变性大细胞淋巴瘤(ALCL)的临床和病理预后特征的研究。102例中有71例(69.6%)出现间变性淋巴瘤受体酪氨酸激酶(ALK-1)表达,未发现其影响总生存(OAS)或无病生存(DFS)。ALK阳性患者的2年OAS率为63.5%,DFS率为54.4%,而ALK阴性患者的OAS为60.5%,DFS为43.5%。发现Ann Arbor分期、体能状态、国际预后指数、组织学亚型和背景炎症浸润程度对OAS有显著影响。反应性炎症成分增加也对DFS有负面影响。在多因素分析中,只有组织学类型对OAS有显著意义。因此,虽然ALK在系统性ALCL的预后评估中起作用,但晚期疾病和炎症环境可能会调节最终结果。

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