Primary non-Hodgkin's lymphoma of the nose and nasopharynx: Clinical features, tumor immunophenotype, and treatment outcome in 113 patients

Primary non-Hodgkin's lymphoma of the nose and nasopharynx: Clinical features, tumor immunophenotype, and treatment outcome in 113 patients
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DOI:
10.1200/jco.1998.16.1.70
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发表时间:
1998-01-01
影响因子:
45.3
通讯作者:
Ngan, RKC
Ngan, RKC
中科院分区:
医学1区
文献类型:
--
作者:
Cheung, MMC;Chan, JKC;Ngan, RKC

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目的:根据免疫表型研究原发性鼻/鼻咽部非霍奇金淋巴瘤 (NNP-NHL) 的临床特征和结局。 患者和方法:本回顾性分析了 113 例中国原发性 NNP-NHL 患者,这些患者根据工作方案 (WF) 属于 E、F、G 或 H 类,具有完整的免疫表型数据和完整的临床随访数据研究结果:90 名 (79.6%) 患者患有局限性(I 期或 II 期)疾病,23 名 (20.4%) 患者患有 III 期或 IV 期疾病。 51例(45.1%)、24例(21.3%)和38例(33.6%)患者的淋巴瘤分别表现出自然平衡(NK)/T-(CD56阳性)、T细胞和B细胞免疫表型。73例患者(65.8%)获得完全缓解,其中34例(46.6%)随后复发。存活者的中位随访时间为88个月,5年精算无病生存率和总生存率分别为34.4%和37.9%。多变量分析显示,只有分期和免疫表型对生存具有重要意义。 NK/T淋巴瘤在三种免疫表型中具有以下特点:男女比例最高,更频繁地仅累及鼻腔,传播到皮肤的风险更高,更频繁地发生噬血细胞综合征,预后最差(总体中位生存期,12.5个月)。结论:研究的三种猿免疫表型表现出不同的临床模式,由于NK/T表型预后最差,出现这种情况的患者对于患有 NNP-NHL 的患者,应分析其肿瘤的 CD56 表达。 (C) 1998 年美国临床肿瘤学会。
Purpose: To study the clinical features and outcome for primary non-Hodgkin's lymphomas of the nose/nasopharynx (NNP-NHLs) according to immunophenotype.Patients and Methods: One hundred thirteen Chinese patients with primary NNP-NHLs that belonged to the categories E, F, G, or H according to the Working Formulation (WF), with full immunophenotypic data and complete clinical follow-up data, were analyzed in this retrospective study.Results: Ninety (79.6%) patients had localised (stage I or II) disease, while 23 (20.4%) had stage III or IV disease. The lymphomas in 51 (45.1%), 24 (21.3%), and 38 (33.6%) patients showed natural kilter (NK)/T-(CD56-positive), T-cell, and B-cell immunophenotype, respectively, Seventy-three patients (65.8%) achieved a complete remission, of whom 34 (46.6%) subsequently relapsed. The median follow-up time for those alive was 88 months, The 5-year actuarial disease-free and overall survival rates were 34.4% and 37.9%, respectively. Multivariate analysis showed that only stage and immunophenotype were significant for survival. NK/T lymphomas were distinctive among the three immunophenotypes in the following aspects: the highest male-to-female ratio, more frequent involvement of the nasal cavity alone, higher risk of dissemination to the skin, more frequent development of hemophagocytic syndrome, and the worst prognosis (overall median survival, 12.5 months).Conclusion: The three immunophenotypes studied ape shown to exhibit different clinical patterns, Since the NK/T phenotype carries the worst prognosis, patients who present with NNP-NHL, should have their tumors analyzed for CD56 expression. (C) 1998 by American Society of Clinical Oncology.