Natural killer/natural killer-like T-Cell lymphoma, CD56+, presenting in the skin: An increasingly recognized entity with an aggressive course
Natural killer/natural killer-like T-Cell lymphoma, CD56+, presenting in the skin: An increasingly recognized entity with an aggressive course
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DOI：
10.1200/jco.2001.19.8.2179
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发表时间：
2001-04-15
影响因子：
45.3
通讯作者：
Kim, YH
中科院分区：
文献类型：
--
作者：
Mraz-Gernhard, S;Natkunam, Y;Kim, YH
目的：描述和确定皮肤表现的自然杀伤细胞（NK）和NK样t细胞（NK/ t细胞）淋巴瘤的临床和病理特征，患者和方法：本研究回顾性分析了30例以皮肤病变为首发的CD56+淋巴瘤，并分析了临床和组织病理学参数。结果：所有患者的中位生存期为15个月。与没有皮外受累的患者（17例）相比，首发时有皮外表现的患者（11例）的中位生存期较短，为7.6个月，而没有皮外受累的患者（17例）的中位生存期较好，为44.9个月（P = 0.0001）。年龄、性别、皮肤受累程度和对治疗的初始反应对生存率没有统计学上的显著影响。7例患者（24%）在肿瘤细胞内检测到eb病毒（EBV）。共表达CD30的肿瘤细胞患者（7例）在随访35个月后仍未达到中位生存期，而CD30-肿瘤细胞患者（20例）的中位生存期为9.6个月（P < 0.02）。常规组织病理学特征没有预后意义，CD3（）、EBV或多药耐药的存在也没有预后意义。结论：NK/ t细胞淋巴瘤是一种侵袭性肿瘤；然而，在本研究中发现了一个预后较好的子集，表现为皮肤外疾病是最重要的临床变量，预示着预后不良。最初的皮肤受累程度不能可靠地预测结果。来自美国的皮肤NK/ t细胞淋巴瘤患者在其肿瘤细胞中可证实的EBV发病率低。CD56淋巴瘤中CD30共表达的患者往往有更有利的结果。(C) 2001年美国临床肿瘤学会。
Purpose: To describe and identify the clinical and pathologic features of prognostic significance for natural killer (NK) and NK-like T-cell (NK/T-cell) lymphoma presenting in the skin,Patients and Methods: This study was a retrospective review of 30 patients with CD56+ lymphomas initially presenting with cutaneous lesions, with analysis of clinical and histopathologic parameters,Results: The median survival for all patients was 15 months. Those with extracutaneous manifestations at presentation (11 patients) had a shorter median survival of 7.6 months as compared with those without extracutaneous involvement (17 patients), who had a more favorable median survival of 44.9 months (P = .0001). Age, gender, extent of cutaneous involvement, and initial response to therapy had no statistically significant effect an survival. Seven patients (24%) had detectable Epstein-Barr virus (EBV) within neoplastic cells. The patients with tumor cells that coexpress CD30 (seven patients) have not yet reached a median survival after 35 months of follow-up as compared with those with CD30- tumor cells (20 patients), who had a median survival of 9.6 months (P < .02). Routine histopathologic characteristics had no prognostic significance nor did the presence of CD3(), EBV, or multidrug resistance.Conclusion: NK/T-cell lymphoma is an aggressive neoplasm; however, a subset with a more favorable outcome is identified in this study, The presence of extracutaneous disease at presentation is the most important clinical variable and portends a poor prognosis. The extent of initial skin involvement does not reliably predict outcome. Patients from the United States with NK/T-cell lymphoma presenting in the skin have a low incidence of demonstrable EBV in their tumor cells. Patients with coexpression of CD30 in CD56 lymphomas tend to have a more favorable outcome. (C) 2001 by American Society of Clinical Oncology.