鼻型NKT淋巴瘤患者血清生存素和P53表达及其临床意义研究

目的 探讨鼻型NKT淋巴瘤患者血清生存素(survivin)和P53表达及与其临床病理因素的相关性.方法 收集鼻型NK/T淋巴瘤患者45例,同期纳入体检健康者50例作为对照组;比较两组血清survivin和P53水平.分析鼻型NKT淋巴瘤患者血清survivin和P53的水平与患者性别、年龄以及TNM分期、全身B症状、Ann Arbor分期、IPI评分等临床病理因素的相关性.所有患者均随访2年,记录死亡病例,比较死亡患者和存活患者在入院时的survivin和P53水平.结果 与对照组比较,NK/T淋巴瘤组患者血清survivin和P53水平均明显升高(P＜0.01).血清survivin和P53的水平与NK/T淋巴瘤患者年龄、性别无显著相关性(P＞0.05),而与TNM分期、全身B症状、Ann Arbor分期、IPI评分呈显著性相关,(P＜0.01).随访2年:10例死亡,35例存活;死亡患者在入院时血清survivin和P53水平均明显高于存活者(P＜0.01).结论 鼻型NKT淋巴瘤患者血清survivin和P53水平均显著升高,与其恶性程度密切相关,也可能是临床评价该病患者预后的重要标志物.     

不同治疗方案对鼻、鼻型NK/T细胞淋巴瘤疗效分析

目的 探讨鼻、鼻型NK/T细胞淋巴瘤的临床特征、治疗方法和预后.方法 收集自2004年1月-2010年1月在我院住院治疗的29例鼻、鼻型NK/T细胞淋巴瘤患者临床资料,分析其临床特点、治疗方案及预后.结果:治疗分CHOP方案组及L-ASP组,总有效率分别为56.3%和75.9%（P=0.045）.两组5年OS率和DFS率分别为43.75%、61.54%（P=0.038）及18.8%、46.15%（P=0.009）,具有统计学意义.难治组7例经VDLP方案补救化疗后总有效率为71.3%.B组症状、临床分期及KPS评分可能对患者的生存产生影响.结论:对于临床早期的鼻、鼻腔NK/T细胞淋巴瘤患者,初治时可以选择CHOP方案治疗,而中、晚期病例及CHOP方案治疗无效的病例,选择以左旋门冬酰胺酶为主的联合化疗结合放疗综合治疗可取得较好疗效.     

鼻型T/NK细胞淋巴瘤累及皮肤1例报道

[目的]探讨鼻型T/NK细胞淋巴瘤累及皮肤的临床表现、病理、免疫表型及EB病毒相关性.[方法]应用免疫组织化学染色,选用CD45RO、CD3、TIA-1、CD20、Ki-B5、CD68和LMP1等抗体;用E-BER1/2原位杂交检测EB病毒编码的小分子RNA.[结果]此例皮肤及鼻部取材组织病理表现为坏死及中等大小的异形性肿瘤细胞增生浸润,CD56 ,CD3 ,CD8,CD20-,为T/NK细胞淋巴瘤表型.EBV瘤细胞阳性,进一步证实为鼻型T/NK细胞淋巴瘤,EBV相关性.[结论]支持鼻型T/NK细胞淋巴瘤是具有特殊免疫表型,与EB病毒有高度相关性的独立疾病.     

不同治疗方案对鼻、鼻型NK/T细胞淋巴瘤疗效分析

目的：探讨鼻、鼻型NK/T细胞淋巴瘤的临床特征、治疗方法和预后。方法：收集自2004年1月-2010年1月在我院住院治疗的29例鼻、鼻型NK/T细胞淋巴瘤患者临床资料,分析其临床特点、治疗方案及预后。结果：治疗分CHOP方案组及L-ASP组,总有效率分别为56.3%和75.9%（P=0.045）。两组5年OS率和DFS率分别为43.75%、61.54%（P=0.038）及18.8%、46.15%（P=0.009）,具有统计学意义。难治组7例经VDLP方案补救化疗后总有效率为71.3%。B组症状、临床分期及KPS评分可能对患者的生存产生影响。结论：对于临床早期的鼻、鼻腔NK/T细胞淋巴瘤患者,初治时可以选择CHOP方案治疗,而中、晚期病例及CHOP方案治疗无效的病例,选择以左旋门冬酰胺酶为主的联合化疗结合放疗综合治疗可取得较好疗效。     

84例鼻型NK/T细胞淋巴瘤的治疗与预后分析

目的 分析鼻型NK/T细胞淋巴瘤的临床特征、不同治疗方法的疗效及影响预后的因素.方法 回顾性分析经病理证实的84例鼻型NK/T细胞NHL患者的临床资料,其中48例经免疫组化证实.根据Ann Arbor分期,Ⅰ期46例,Ⅱ期19例,Ⅲ期16例,Ⅳ期3例.单纯放疗29例,单纯化疗5例,放疗、化疗结合50例.预后判断采用Cox多因素回归模型分析.结果 全组5年总生存（OS）和无病生存（DFS）率分别为48.8%与35.7%.首程治疗后达CR的5年OS为59.4%,未达CR的5年OS为15.0%（P＜0.01）.单纯化疗中1例达CR（20.0%）,单纯放疗21例达CR（72.4%）,放疗、化疗结合42例达CR（84.0%）,放疗、化疗结合与单纯放疗后达CR明显高于单纯化疗（P＜0.01）.单纯放疗和放疗、化疗结合的5年OS率分别为44.8%和54.0%（P＞0.05）,均明显高于单纯化疗20.0%（P＜0.05）;单纯放疗和放疗、化疗结合的5年DFS分别为34.5%、40.0%,明显高于单纯化疗（P＜0.01）.多因素回归分析显示：IPI、首程CR、B症状、鼻中隔和（或）硬腭穿孔及治疗方法为影响生存的独立预后因素,其中以IPI评分最明显.结论 对鼻型NK/T淋巴瘤采用单纯化疗疗效差,而放疗、化疗结合疗效较好,但远期生存情况仍不满意.     

鼻和鼻型NK/T细胞淋巴瘤的治疗进展

鼻和鼻型NK/T细胞淋巴瘤是一种起源于NK细胞的少见的非霍奇金淋巴瘤,其临床病理表现独特,肿瘤浸润以血管为中心,伴有血管破坏和灶性坏死。其几乎可以侵及所有结外器官,侵袭性高,极易产生耐药性而疗效差,预后差,生存时间短,目前尚无最佳的治疗模式。本文对近年来采用的放疗,传统化疗,大剂量化疗结合干细胞移植和基于左旋门冬酰胺酶的联合化疗等治疗模式进行综合论述,比较各疗法的优缺点并介绍了最新发展趋势。     

鼻型NK/T细胞淋巴瘤临床病理特征及预后分析

目的探讨鼻型NK/T细胞淋巴瘤临床病理特征及预后影响因素。方法回顾性分析经病理证实的31例该病患者的临床资料。结果鼻型NK/T细胞淋巴瘤容易误诊;3年及5年生存率分别为58.1%和33.3%;随着分期增高,生存率明显下降;有B症状者或IPI评分≥2者,其生存率分别显著低于无B症状者或IPI评分<2者;综合治疗组3年生存率显著高于单纯化疗组。结论鼻型NK/T细胞淋巴瘤的诊断应结合其临床特征、形态学、免疫表型及遗传学改变综合确定;AnnArbor分期、IPI及B症状是影响预后的重要因素;综合治疗可能是目前最佳的治疗选择。     

鼻外NK/T细胞淋巴瘤21例临床特征及预后分析

目的 探讨鼻外NK/T细胞淋巴瘤的临床特征与预后.方法 对21例鼻外NK/T细胞淋巴瘤的病例资料进行回顾性分析,并复习相关文献.结果 患者以晚期、有B症状、体能状态（ECOG评分）2～4分、国际预后指数（IPI）3～5分和乳酸脱氢酶（LDH）升高者为多,除1例放弃治疗外,20例患者中,完全缓解5例,部分缓解1例,总生存率为30.0％.早期患者疗效较好,2例晚期合并噬血细胞综合征和弥散性血管内凝血的患者预后最差.结论 鼻外NK/T细胞淋巴瘤患者的预后较差,临床分期、ECOG、IPI和LDH是影响预后的主要因素.     

鼻型自然杀伤-T细胞淋巴瘤31例临床分析

 目的　分析结外鼻型自然杀伤（NK）-T细胞淋巴瘤的临床特点及治疗方案，提高对鼻型NK-T细胞淋巴瘤的认识。方法　对经病理证实的鼻型NK-T细胞淋巴瘤31例的临床资料进行回顾性的分析。结果　31例患者中，EB病毒（EBV）感染24例（77.42 %）。近期疗效结果显示，放疗、化疗、放疗+化疗差异无统计学意义（χ2＝3.61，P＞0.05）。随访截至2010年10月，31例患者中死亡24例，7例生存，其中4例无瘤生存。19例死于肿瘤复发或进展。全组中位生存时间为32个月。患者5年生存情况与临床分期、区域淋巴结受累、Ｂ症状、乳酸脱氢酶（LDH）水平、局部肿瘤浸润、EBV感染、早期治疗有关（χ2值分别为8.88、7.25、16.95、6.00、7.23、7.44、7.80，均P＜0.05）。结论　放疗、化疗、放疗+化疗治疗鼻型NK-T细胞淋巴瘤疗效差异不大；患者的生存情况与临床分期、区域淋巴结受累、Ｂ症状、LDH水平、局部肿瘤浸润、EBV感染、早期治疗等临床特征有关。     

鼻型NK/T细胞淋巴瘤研究进展

目的:总结鼻型NK/T细胞淋巴瘤的研究进展。方法:应用Medline及CNKI期刊全文数据库检索系统,检索1992-01-2007-12关于鼻型NK/T细胞淋巴瘤分子发病机制、临床病理和免疫组化研究及治疗方面的文献。最后纳入32篇。结果:鼻型NK/T细胞淋巴瘤主要发生于鼻腔及面中线部位,也可以发生于其他部位,如皮肤、胃肠道、中枢神经系统、肺以及睾丸等。病理学上,肿瘤细胞胞质CD3及CD56阳性,同时表达细胞毒性颗粒蛋白,如TIA-1等,往往也表达EBV病毒抗原。鼻型NK/T细胞淋巴瘤预后不良,易复发且对治疗不敏感。新的国际预后指数已用于评价该肿瘤的预后。从单独放疗到高剂量的化疗以及造血干细胞移植术等几种治疗手段已应用于临床。结论:鼻型NK/T细胞淋巴瘤与EB病毒有关,具有独特的临床病理特点,放化疗联合运用可提高其治疗效果。     

Clinicopathologic and genotypic study of extranodal nasal-type natural killer/T-cell lymphoma and natural killer precursor lymphoma among Koreans

BACKGROUND: This study aimed to define genotypic profile and to describe the clinicopathologic features of nasal-type natural killer (NK)/T-cell lymphoma of nasal and extranasal origin and NK precursor lymphoma. METHODS: NK/T-cell lymphomas from the upper aerodigestive tract (n = 45), skin (n = 2), gastrointestinal tract (n = 3), and soft tissue (n = 2) and NK precursor neoplasms (n = 3) were studied. Immunophenotype was analyzed by immunohistochemistry and flow cytometry. In situ hybridization with EBER 1/2 RNA probes was performed. T-Cell Receptor (TCR)-gamma gene rearrangement was analyzed by seminested polymerase chain reaction with heteroduplex analysis. Overall survival rate was correlated with clinicopathologic parameters and compared by Wilcoxon test. RESULTS: Clonal TCR-gamma gene rearrangement was detected in 3 of 31 upper aerodigestive and 1 of 2 skin tumors. When immunostained using paraffin embedded tissue, 6 upper aerodigestive lymphomas were negative for CD56 in which 4 cases lacked clonal TCR gene rearrangement. Epstein-Barr virus (EBV) mRNA was detected in 33 upper aerodigestive tumors including 26 of 29 nasal tumors (90%), and 7 of 10 extranasal tumors (70%). There was no histologic, immunophenotypic, or genotypic differences according to the lineage and EBV association in upper aerodigestive lymphomas. Among the patients with upper aerodigestive tumors, overall 1-year survival rate was 41%, and correlated well with the stage (P < 0.05) but not with the size of tumor cells, EBV status, and lineage (P > 0.05). Median survival rate of lymphomas from other sites excluding upper aerodigestive tract was not significantly different from that of upper aerodigestive lymphomas with same stage (P > 0.05). Unlike nasal-type NK/T-cell lymphomas, NK precursor lymphoma involved the bone marrow and lymph nodes at initial presentation or in the course of disease. Tumor cells were positive for TdT in all and myeloid markers in two. TCR gene rearrangement was germ line. CONCLUSIONS: Most upper aerodigestive nasal-type NK/T-cell lymphomas among Koreans are genotypically of NK derivation and few belong to T lineage. Presence or absence of EBV has no significant correlation with the histologic changes and the lineage of these lymphomas.     

Combined chemotherapy and external beam radiation for stage IE and IIE natural killer T-cell lymphoma of nasal cavity

To evaluate the outcome of CHOP chemotherapy and radiotherapy in Stage IE and IIE nasal natural killer (NK)/T-cell lymphoma, 53 patients with stage IE and IIE nasal NK/T-cell lymphoma were studied. By the Ann Arbor Lymphoma Staging Classification, 41 patients (77%) had Stage IE disease and 12 patients (23%) had Stage IIE disease. All patients were treated curatively using chemotherapy, followed by radiotherapy. Chemotherapy consisted of up to six cycles of the standard CHOP based regimen. The median radiation dose to the tumor bed was 45 Gy for all patients. The median follow-up for all 39 surviving patients was 30.2 months (range, 6 - 104 months). Twenty-six patients had complete response after chemotherapy, and all patients who completed first line chemotherapy achieved complete response after radiotherapy. The 2-year overall survival and progression-free survival rates were 75.6% and 61.8%, respectively. Multivariate analysis revealed that perforation as a presenting symptom, elevated pretreatment serum lactate dehydrogenase level, and ECOG performance status >or=2 were significant independent prognostic factors for this group of patients. Combined chemotherapy followed by involved field radiation produced suboptimal outcome for patients with early stage nasal NK/T-cell lymphoma. Further investigations, preferably prospective clinical trials, for more efficacious treatment strategies are needed to improve the treatment outcome of this malignancy.     

Unfavorable prognosis of elderly patients with early-stage extranodal nasal-type NK/T-cell lymphoma

BackgroundExtranodal nasal-type NK (natural killer)/T-cell lymphoma in elderly patients is rare, and its prognosis is unclear. This study aims to investigate the clinical characteristics and prognosis of this lymphoma.Patients and methodsWe analyzed 24 patients (age, >60 years old) with early-stage extranodal nasal-type NK/T-cell lymphoma. Among these patients, 23 received radiotherapy alone (n = 15) or radiotherapy and chemotherapy (n = 8) and 1 received chemotherapy alone.ResultsElderly patients with early-stage extranodal nasal-type NK/T-cell lymphoma were characterized by male predominance, good performance, large proportion of localized disease, more comorbidities, low-risk international prognostic index, and unfavorable prognosis. The 5-year cancer-specific survival (CSS), overall survival (OS), and progression-free survival (PFS) rates for all patients were 54.3%, 42.0%, and 40.2%, respectively. The 5-year CSS, OS, and PFS rates were 43.5%, 36.6%, and 34.1% in patients who received radiotherapy alone, and 50%, 50%, and 50% in patients who received radiotherapy and chemotherapy, respectively (P = 0.852 for CSS, P = 0.801 for OS, and P = 0.694 for PFS). Four patients died of treatment-related mortality.ConclusionElderly patients with early-stage extranodal nasal-type NK/T-cell lymphoma exhibit poor prognosis and need special attention because of high treatment-related mortality.     

非霍奇金淋巴瘤1012例临床病理分析

目的:了解我院近10年来非霍奇金淋巴瘤(NHL)的发病特点,分析影响NHL预后的相关因素.方法:回顾性分析了近10年来我院收治的1012例NHL患者的临床病理特点,对影响新疆地区NHL生存率及预后的临床病理因素进行分析.结果:1012例NHL中以40～60岁汉族男性发病多见,最主要病理类型依次为弥漫性大B细胞淋巴瘤(DLBCL)346例(34.1%),外周T细胞淋巴瘤(PTCL)185例(18.3%),滤泡淋巴瘤(FL)97例(9.6%),黏膜相关淋巴组织淋巴瘤(MALT)94例(9.3%),NK/T细胞淋巴瘤62例(6.1%),T-淋巴母细胞淋巴瘤(T-LBL)47例(4.6%).结性起病的淋巴瘤619例(61.2%),结外起病的淋巴瘤393例(38.8%).本组维吾尔族女性FL淋巴瘤患者人数比例较汉族女性患者高(P=0.002),汉族男性PTCL患者人数比例高于维吾尔族男性患者(P=0.015).5年总生存率为45.8%.单因素分析显示临床分期,行为状态评分(PS),B症状,年龄,肿块大小,血清乳酸脱氢酶(LDH),结外器官受侵数目及IPI是NHL的预后因素(P＜0.05).多因素分析提示T细胞来源,Ⅲ～Ⅳ临床分期,IPI评分3～5分及LDH增高是NHL独立的预后不良因素(P＜0.05).结论:新疆地区NHL发病以中年多见,结性起病者多于结外起病,B细胞淋巴瘤多于T细胞淋巴瘤.免疫分型、临床分期、IPI、血清LDH水平与NHL预后相关.     

Natural killer cell neoplasms

Lymphoid neoplasms that are derived from natural killer (NK) cells are uncommon but distinct clinicopathologic disease entities. Three types have been recognized and categorized in the latest World Health Organization classification: extranodal NK cell lymphoma, nasal-type; aggressive NK cell leukemia; and blastic NK cell lymphoma. All NK tumor cells express the NK cell marker CD56, but they lack the expression of surface CD3 and the rearrangement of T-cell receptor genes, which distinguish them from T-lymphoid neoplasms. There is also a strong association with the Epstein-Barr virus, except in blastic NK cell lymphoma. Extranodal involvement by the NK cell tumor is common, especially in the nasal cavity, the skin, and the gastrointestinal tract. All 3 NK cell neoplasms are characterized by aggressive clinical course and poor response to treatment. Although the optimal treatment modality remains to be determined, good initial response to combined radiation therapy and chemotherapy has been observed in localized disease. Further studies in the basic biology of the NK cell and the pathology of NK cell neoplasms may shed light on the development of newer and more effective therapy.     

CD56+ hematological neoplasms presenting in the skin: a retrospective analysis of 23 new cases and 130 cases from the literature.

BACKGROUND: The aim of this study was to define prognostic parameters and guidelines for diagnosis and treatment for CD56+ hematological neoplasms with first presentation in the skin. PATIENTS AND METHODS: The study group included 153 cases (23 new and 130 from the literature). According to the World Health Organization classification, the group included 15 nasal and 38 nasal-type natural killer (NK)/T-cell lymphomas, 63 blastic NK-cell lymphomas, 14 cutaneous CD30+ lymphoproliferations, 10 cases of myeloid leukemia, six cases of subcutaneous panniculitis-like T-cell lymphoma (SCPLTCL) and seven peripheral T-cell lymphomas, unspecified. RESULTS: In general, these CD56+ hematological neoplasms had a poor prognosis, with only 27% of patients alive after a median follow-up of 12 months. The median survival was 13 months. Nasal and nasal-type NK/T-cell lymphomas and CD56+ SCPLTCL had the worst prognosis, with a median survival of 5, 6 and 5 months, respectively. Only nasal-type NK/T-cell lymphomas presenting with only skin lesions had a somewhat better prognosis (median survival 27 months). In blastic NK-cell lymphomas (median survival 14 months), age 相似文献   

疗前血清Hb水平在早期结外鼻型NK/T细胞淋巴瘤中的预后价值

目的 探讨治疗前血清Hb水平对早期结外鼻型NK/T细胞淋巴瘤预后的影响。 方法 回顾分析2000—2015年间收治的 175例Ⅰ、Ⅱ期结外鼻型NK/T细胞淋巴瘤。纳入标准为原发病灶位于上消化呼吸道,不合并其他恶性疾病,治疗及随访记录完整病例纳入分析。接受单纯化疗 67例、单纯放疗 8例、放化疗 100例。Kaplan-Meier法计算生存率,Logrank法单因素分析,Cox模型多因素分析。 结果 175例患者的变量单因素分析显示疗前血清Hb水平(≥120 g/L)、LDH水平(正常)、ECOG评分(0~1)、Ann Arbor分期(Ⅰ_{E)、接受放疗显著提高PFS及OS (P=0.000~0.046)。多因素分析显示血清Hb水平、LDH水平、ECOG评分、Ann Arbor分期是患者PFS、OS影响因素(P=0.000~0.040)。 结论 疗前Hb≥120 g/L者预后好于<120 g/L者。}     

37例鼻腔NK/T细胞淋巴瘤临床分析

目的分析鼻腔NK/T细胞淋巴瘤的临床特征、不同治疗方法的疗效及影响预后的因素。方法回顾性分析1999年12月至2008年4月收治的37例经病理证实的鼻腔NK/T细胞淋巴瘤患者的临床资料。根据AnnArbor分期,Ⅰ期17例,Ⅱ期15例,Ⅲ期1例,Ⅳ期4例。单纯放疗8例,单纯化疗9例,其余20例采用放化疗联合治疗。单因素分析采用Kap lan-M e ier法,多因素分析运用Cox比例风险模型。结果全组中位生存时间27月,3年总生存（OS）率为50.0%,放化疗联合、单纯放疗、单纯化疗3年生存率分别为77.8%、50.0%、0%。单纯放疗、放化疗联合治疗后3年生存率明显高于单纯化疗（P〈0.01）;放化疗联合与单纯放疗治疗后3年生存率无明显差异（P〉0.05）。单纯化疗CR 1例（11.1%）,单纯放疗CR 6例（75.0%）,放化疗联合CR 16例（80.0%）,单纯放疗、放化疗联合治疗后CR率明显高于单纯化疗（P〈0.01）;放化疗联合与单纯放疗治疗后CR率差异无统计学意义（P〉0.05）。单因素分析显示,PS评分、IPI、LDH水平、临床分期、治疗模式、首程治疗后CR率等与预后相关。多因素分析显示,首程治疗后CR率、PS评分是鼻腔NK/T细胞淋巴瘤预后不良的独立因素。结论鼻腔NK/T细胞淋巴瘤单纯放疗、放化疗联合近期疗效显著优于常规化疗,化疗加入放疗并未改善生存率。首程治疗后CR率、PS评分可作为判断鼻腔NK/T细胞淋巴瘤预后的参考因素。     

原发鼻腔非霍奇金淋巴瘤的治疗选择和疗效

目的 分析原发鼻腔非霍奇金淋巴瘤（NHL）放疗和化疗的近期疗效以及治疗方法对预后的影响。方法 129例经病理证实的原发鼻腔NHL患者中,经形态学诊断为鼻腔NK／T细胞淋巴瘤者116例。做免疫组化57例,其中52例为NK／T细胞来源,占91．2％;5例为B细胞来源,占8．8％。根据Ann Arbor分期,ⅠE期102例,ⅡE期22例,ⅣE期5例,ⅠE和ⅡE期患者中,单纯放疗22例,单纯化疗7例,综合治疗95例,ⅣE期以化疗为主。结果 5年总生存率（OS）和无病生存率（DFS）分别为68．0％和55．8％,ⅠE期和ⅡE期患者的5年OS分别为71．7％和70．6％（P=0．77）,DFS分别为60．9％和47．0％（P=0．09）。首程治疗后达CR患者的5年OS为83．1％,而未达CR患者的5年OS为18．0％（P=0．000）,相应5年DFS分别为68．0％和15．5％（P=0．000）。124例ⅠE和ⅡE期患者中,67例患者接受单纯放疗或放疗后化疗,放疗后完全缓解率（CR）为74．7％,其余57例为化疗后放疗或单纯化疗,化疗后CR率仅19．3％（P=0．000）,46例化疗后未达CR的患者中,42例仍局限于局部区域,31例经放疗达到CR,ⅠE和ⅡE期患者中,先放疗组（放疗＋化疗或单纯放疗）、化疗后放疗组的5年OS分别为76．0％和74．4％,DFS分别为65．0％和56．2％（P〉0．05）,ⅠE和ⅡE期单纯化疗7例,3例存活,4例死亡,中位生存时间15个月,1年生存率为26．7％。结论 中国人原发鼻腔NHL主要为NK／T细胞来源,放疗的近期疗效显著优于化疗,化疗加入放疗并未改善生存率,ⅠE和ⅡE期患者应以放射治疗为主要治疗手段。