睾丸的原发性NK/T细胞淋巴瘤一例报告及文献复习

Objective： To study the clinical and pathological features of primary NK/T cell lymphoma of testis and to investigate the effective diagnosis and treatment of this disease. Methods： The surgical specimens of a patient with primary NK/T cell lymphoma of the testis were observed by light microscopy, immunohistochemistry and examined by the polymerase chain reaction （PCR） for Epstein-Barr virus （EBV） DNA and T-cell receptor （TCR） gene rearrangement, and the literature were reviewed. Results： The patient presented with left-sided painless testicular enlargement and the lymphoma had a propensity to spread to the contralateral testis, spleen, central nervous system, and so on. The neoplastic cells were positive for CD56, CD45R0 and CD3ε, while the expressions of CD20, CD79α, CD5, Bcl-2 and PLAP were negative. In addition, the EBV DNA was detected in the lymphoma by PCR. And the results of gene rearrangement studies for the y chain of the T-cell receptor were negative. The pathological diagnosis was NK/T cell lymphoma of the left testis. Conclusion： Primary NK/T cell lymphoma of the testis is a rare entity and progressed rapidly. The histopathological, immunohistochemical, EBV examination and TCR gene rearrangement studies should be carried out as soon as possible in order to get the defined diagnosis. Currently, the therapeutic efficacy is poor and the new measures should be investigated to improve the survival rate.     

Nasal-type NK/T cell lymphoma: clinical features and treatment outcome

Nasal-type NK/T cell lymphoma is an increasingly recognised disease entity of aggressive clinical behaviour. The objective of this study was to investigate clinical features and treatment outcomes in patients with nasal-type NK/T cell lymphoma. From January 1991 to December 2003, 26 patients diagnosed as nasal-type NK/T cell lymphoma were included in the analysis. One half of patients presented with poor performance status (ECOG > or =2); 46% of patients were categorised as high intermediate or high-risk group according to IPI; and 46% of patients were diagnosed at advanced stage. The median survival for 26 patients with nasal-type NK/T cell lymphoma was 7.4 months (95% CI, 0.1, 16.9). The treatment outcome of primary anthracycline-based chemotherapy was poor: 60% CR rate in localised disease and 0% CR rate in advanced disease. After a median follow-up of 24.4 months (range 3.1-99.0) in patients with localised disease who had achieved a CR (range 29.6-165.7), three patients (50.0%) developed disease recurrence at 6.1, 21.8, and 52.1 months, respectively, and all patients presented with locoregional failure. The predictive factors for poor survival were of age greater than 60, advanced stage and poor performance in multivariate analysis. In conclusion, Nasal-type NK/T cell lymphomas showed a poor response to the conventional anthracycline-based chemotherapy, and thus an investigation for an innovative therapy is urgently needed to improve survival in these patients.     

THE DETECTION OF CD56 AND EPSTEIN-BARR VIRUS IN T-CELL LYMPHOMAS

Ｒｅｃｅｎｔｌｙ，ｔｈｅｉｍｐｏｒｔａｎｃｅｏｆＥＢＶｉｎＴＣＬｐａｔｈｏｇｅｎｅｓｉｓｗａｓｎｏｔｉｃｅｄｍｏｒｅａｎｄｍｏｒｅ．１Ｆｕｒｔｈｅｒ，ｉｔｈａｓｂｅｎｄｉｓｃｏｖｅｒｅｄｔｈａｔＴＣＬｓｗｉｔｈＥＢＶｉｎｆｅｃｔｉｏｎｏｆｔｅｎｅｘｐ...     

L-Asparaginase for newly diagnosed extra-nodal NK/T-cell lymphoma: systematic review and meta-analysis

Objectives: The aim of this review was to compare the efficacy of asparaginase (ASP)-containing vs ASP-absent regimens in the first-line treatment of ENKTL patients.

Methods: The PRISMA protocol was used to search PubMed and Embase for both controlled and uncontrolled studies of ASP or alternative chemotherapy (CT) for newly diagnosed ENKTL, published in English by March 2017. The regimens were compared to calculate relative risk (RR) with 95% confidence interval (CI) of the overall response rate (ORR), complete response (CR) or partial response (PR).

Results: Out of 38 studies included, eight were controlled trials, with the pooled RR of ORR in stage I-II 1.54 (95% CI 1.34–1.77); stage I-IV 1.34 (95% CI 1.09–1.64). In stage III-IV CT combined with radiotherapy (RT), RR of ORR was 2.30 (95% CI 1.66–3.18). ASP was also superior in achieving CR. When all single arms combined, RR of ORR after CT with ASP was 1.52 (95% CI 1.38–1.67) in stage I-II (15 studies); 1.44 (95% CI 1.32–1.57) in all stages (29 studies); 1.31 (95% CI 1.24–1.38) and 1.66 (95% CI 1.18–2.34) in stages I-II and III-IV combined with RT, correspondingly.

Conclusions: ASP-based CT significantly improved ORR and CR in patients with newly diagnosed both early-stage and advanced-stage ENKTL.     

Primary Testicular NK/T-Cell Lymphoma：A Study of Two Cases and Review of Literature

Primary testicular NK/T-cell lymphoma is an extremely rare entity progressed rapidly. The aim of this study was to examine clinical and pathological features of primary testicular NK/T-cell lymphoma and to investigate the effective diagnosis and prognosis. In this paper, the two cases of primary testicular NK/T-cell lymphoma were observed by light microscopy, immunohistochemistry and examined by in situ hybridization for Epstein-Barr Virus (EBV) DNA and the literatures were reviewed. The two patients respectively present with bilateral and right-side painless testicular enlargement. The morphology of neoplastic cells of case 1 were small to medium, tumor cells of case 2 were small, medium and large mixed. The tumor cells grew diffusely with irregular and distort nuclear, destructed the organizational structure of the normal testis, and damaged blood vessels, meanwhile, coagulation necrosis was exist. Immunohistochemical staining of neoplastic cells showed positive for CD45, CD2, CD56, CD3ɛ (cytoplasm staining pattern), CD45RO and Granzyme B, and negative for CD57, CD20, CD79α, CD30, CK, MPO, TdT, Bcl-2 and PLAP were negative. In addition, the EBV DNA was detected in the lymphoma by In situ hybridization. In conclusion, the expression of CD56, CD3ɛ, and Granzyme B associated proteins and EBV examination by in situ hybridization play a vital role in diagnosis and differential diagnosis of primary testicular NK/T-cell lymphoma.     

CD30 expression in extranodal natural killer/T-cell lymphoma,nasal type among 622cases of mature T-cell and natural killer-cell lymphoma at a single institution in South China

Background: Mature T-cell and natural killer (NK)-cell lymphomas compose a heterogeneous group of non-Hodgkinlymphomas, and extranodal NK/T-cell lymphoma, nasal type (ENKTL) is an aggressive subtype with sporadic CD30expression. However, the significance of CD30 expression in ENKTL is controversial. We aimed to classify a large cohortof patients with mature T-cell and NK-cell lymphomas according to the 2016 World Health Organization (WHO) classificationguidelines and to study the association between CD30 expression and prognosis of patients with ENKTL.Methods: We selected consecutive patients with mature T-cell and NK-cell lymphomas who attended our institutionbetween September 1, 2009 and August 31, 2013. We classified the lymphomas according to the 2016 revision of theWHO classification of lymphoid neoplasms, analyzed the associations between CD30 expression and clinicopathologicfeatures of ENKTL patients, and evaluated the prognostic implications of CD30 expression.Results: We identified 622 consecutive patients with mature T-cell and NK-cell lymphomas, including 317 (51.0%)patients with ENKTL. In addition, CD30 expression was detected in 43 (47.3%) of a subset of 91 patients with ENKTL.No clinicopathologic features were associated with CD30 expression, and CD30 positivity showed no prognosticsignificance in patients with ENKTL.Conclusions: ENKTL is the most common type of mature T-cell and NK-cell lymphoma diagnosed at our institution.CD30 is frequently expressed in ENKTL and represents a therapeutic target; however, it may not be a prognosticmarker.     

Extranodal NK / T-cell lymphoma, nasal type: New staging system and treatment strategies

Extranodal NK/T-cell lymphoma (NTCL) is characterized by clinical heterogeneity based on clinical prognostic factors and survival outcome. NTCL subsets are classified as upper aerodigestive tract (UAT) NTCL or non-UAT NTCL; non-UAT has pathologic similarity to UAT-NTCL but is a clinically distinct subtype. Due to the clinical heterogeneity of NTCL, optimal treatment modalities and prognostic factors have been difficult to determine. Ann Arbor staging for lymphomas and the International Prognostic Index (IPI) have been used to predict prognosis for UAT-NTCL; however, local tumor invasiveness (bony invasion or perforation or invasion of the overlying skin) is the most significant factor for poor outcomes in localized UAT-NTCL. Thus, a new staging system is proposed: limited disease (stage I/II UAT-NTCL without local tumor invasiveness) and extensive disease (stage I/II with local invasiveness or stage III/IV disease of UAT NTCL, and non-UAT NTCL) based on treatment outcomes. NTCL is resistant to anthracycline-based chemotherapy, whereas non-anthracycline combination chemotherapy (such as ifosfamide, methotrexate, etoposide, and prednisolone) has an activity against NTCL as either a front-line or as a second-line treatment. The effectiveness of radiotherapy is evident in limited disease, but questionable in extensive disease. ( Cancer Sci 2009; 100: 2242–2248)     

结外NK/T细胞淋巴瘤检测EB病毒与疗效关系的研究*

目的：探讨血浆中EB病毒阳性在评价结外NK/T细胞淋巴瘤近期疗效和远期疗效中的临床意义。方法：收集2011年1 月至2014年4 月郑州大学第一附属医院经病理及免疫组化确诊为结外NK/T细胞淋巴瘤的患者109 例,应用qRT-PCR方法检测其血浆中EBV-DNA拷贝数,比较EBV 阴性和EBV 阳性患者对治疗疗效与预后的差异。结果：109 例结外NK/T细胞淋巴瘤患者,53例阳性患者中有34例（64.2%）为晚期（Ⅲ～Ⅳ期）,56例阴性患者中22例（39.3%）为晚期（Ⅲ～Ⅳ期）,EBV 阳性组中伴有B 症状
33例（62.3%）,阴性组患者21例（37.5%）,EBV 阴性患者与EBV 阳性患者在分期及B 症状方面均具有统计学意义（P<0.05）。 34例（60.7%）EBV 阴性结外NK/T细胞淋巴瘤患者达到客观缓解率,显著高于EBV 阳性患者的客观缓解率（22例,41.5%）（P<0.05）。EBV 阴性组与EBV 阳性组相比,其2 年无进展生存期更高（P<0.05）。 结论：检测结外NK/T细胞淋巴瘤患者血浆中EB病毒对评价其近期疗效及2 年无进展生存期具有重要的临床意义。     

Disease patterns in pediatric classical Hodgkin lymphoma: a report from a developing area in Brazil

Epidemiological patterns established about 20 years ago, divided classical Hodgkin lymphoma (cHL) in three entities with regard to Epstein–Barr virus (EBV) status and histological subtypes and suggested different epidemiological patterns associated with degree of economic development. Here, we investigated histopathological features and EBV association in 100 consecutive pediatric cHL cases occurring in Rio de Janeiro (Brazil). Age at diagnosis ranged from 3 to 18 years (median 14 years) with 27% of cases ≤10 years. Unexpectedly, we did not observe an early childhood peak with most cases occurring in the >10 years age group. Nodular sclerosis (NS) was the most frequent subtype (69%) and was more frequently observed in the >10 years age group, followed by mixed cellularity (MC, 23%) which was distributed equally between age groups. EBV was identified in 44.8% of cases, without preferential association with age groups (≤10 years vs. >10 years). MC cases were independently associated with EBV infection of tumour cells (p = 0.045) and with a CD4/CD20 ratio <1 in the microenvironment (p = 0.014). Our results suggest that a gradual shift from childhood peak to early adulthood peak may be observed in developing regions. The development of MC subtype may result from early exposure to EBV in the context of an impaired immune system reflected by a CD4/CD20 ratio <1. Conversely, it is possible that NS originates predominantly in the context of a better immune response against EBV and/or tumour antigens expressed in the neoplastic cells. Copyright © 2011 John Wiley & Sons, Ltd.     

Risk stratification in extranodal natural killer/T-cell lymphoma

Extranodal natural killer/T-cell lymphoma (ENKL), a subtype of natural killer/T-cell malignancies, is a rare subset of lymphomas with significant biological and clinical heterogeneity. The prognosis of ENKL is variable and therapeutic approaches are not well established. The optimal dose, combination, and sequence of radiotherapy and chemotherapy are evolving, as is the role of stem cell transplantation. Radiotherapy is an essential component of therapy for early-stage disease. The clinical course of advanced disease is highly aggressive, with frequent chemotherapy resistance and a poor prognosis. For relapsed disease, asparaginase-based regimens have provided encouraging results and are currently under investigation in the frontline setting. Our article discusses the key aspects of biology, pathogenesis and clinical presentation that contribute to the heterogeneity, and proposes a stratified approach to the treatment of ENKL based on clinical, pathologic and biologic risk factors. Although considerable advances have been made in our understanding of the biology and prognosis of this lymphoma, it remains critical that all patients with a diagnosis of ENKL are enrolled and treated in clinical trials so that optimal therapies can be identified.     

Monocytes enhance cell proliferation and LMP1 expression of nasal natural killer/T-cell lymphoma cells by cell contact-dependent interaction through membrane-bound IL-15

Nasal natural killer (NK)/T-cell lymphoma (NNKTL) is an Epstein-Barr virus (EBV)-related malignancy with poor prognosis and has distinct histological features characterized by angiocentric and polymorphous lymphoreticular infiltrates including inflammatory cells such as granulocytes, monocytes, macrophages and lymphocytes. Here, we show that the monocytes enhance proliferation as well as LMP1 expression of NNKTL cells by cell contact-dependent interaction through membrane-bound interleukin (IL)-15. We used two EBV-positive NK-cell lines, SNK6 and KAI3, which originated from two patients-SNK6 from a patient with NNKTL and KAI3 from a patient with a severe mosquito allergy. We cocultured the cell lines with granulocytes or monocytes and examined whether proliferation, survival and LMP1 expression of the cells changed. Although cocultured granulocytes did not affect proliferation, survival or LMP1 expression of the cells, cocultured monocytes enhanced both proliferation and LMP1 expression in a dose-dependent manner. These phenomena were not seen when monocytes were placed in a separate chamber. Moreover, the monocyte-inducible proliferation and LMP1 expression were inhibited by treatment with an antibody against IL-15. Furthermore, production of interferon-gamma-inducible protein (IP)-10 were enhanced by coculture with monocytes and were inhibited by the antibody. Immunohistological studies confirmed that a number of infiltrating CD14-positive monocytes contacted CD56-positive lymphoma cells in all of 20 NNKTL tissues tested. These results suggest that monocytes enhance cell growth as well as LMP1 expression of NNKTL cells by cell contact-dependent interaction through membrane-bound IL-15. In the microenvironment of NNKTL tissue, a positive feedback loop of interaction between lymphoma cells and monocytes may be present and contribute to lymphoma progression.     

Natural killer cell lymphoma/leukemia: pathology and treatment

Malignancies arising from cells of putative natural killer (NK) cell origin have increasingly been recognized as distinct clinicopathological entities. These malignancies are marked by tumour cells with NK cell characteristics, including the immunophenotype of CD2⁺, surface CD3⁻, cytoplasmic CD3ϵ+, CD7±, and CD56+, and the genotype of germline T cell receptor gene. A consistent association with monoclonal Epstein–Barr virus infection in the tumour cell has been observed. These tumours are now regarded as putative NK cell lymphoma/leukemia. Pathologically, tumour cells show variable cytological appearances, with frequent angiocentricity and angioinvasion, associated with zonal necrosis. Clinically, most cases occur in the nasal area and upper aerodigestive tract. However, occurrence in non-nasal sites such as the skin, gastrointestinal tract and testis is also observed. A particularly aggressive form of NK lymphoma/leukemia presents fulminantly as disseminated disease sometimes with a leukemic phase. All types of NK lymphoma/leukemia have an extremely poor prognosis with a median survival of less than a year. New modalities of treatment, including the use of high dose chemotherapy and stem cell rescue may be needed to improve treatment outcome. © 1997 John Wiley & Sons, Ltd.     

鼻NK/T细胞淋巴瘤的免疫表型和细胞毒颗粒蛋白的表达及其意义

目的 观察鼻NK／T细胞淋巴瘤的免疫表型和细胞毒颗粒蛋白的表达及其意义：方法：采用DAKOEn Vision两步法在44例鼻NK／T细胞淋巴瘤病例中检测CD3、CD20、CD43、CD45、CD45RO、CD56、CD57和CD79α等8种常用淋巴细胞表面标记物和3种细胞毒颗粒蛋白TIA-1、Granzyme-B、perforjn的表达，观察和分析检测结果，并与发生在相同部位的其他常见恶性淋巴瘤的检测结果进行比较分析。结果：44例鼻NK／T细胞淋巴瘤均表达CD45；CD3阳性率为43％，阳性定位在细胞质；CD56、CD43和CD45RO的阳性率分别为52％、45％和52％，CD20、CD79α和CD57均为阴性。TIA-1、Granzyme-B和perfoljn的阳性率分别为100％、93％和95％，对照组的23例鼻腔和鼻咽T、B细胞淋巴瘤中均不表达。结论：鼻NK／T细胞淋巴瘤的免疫表型不一致，并非所有病例显示CD56阳性，石蜡切片中CD3的阳性定位与通常的外周T细胞淋巴瘤不同。细胞毒性颗粒蛋白在肿瘤细胞中的高表达，提示它们可能为NK细胞来源。细胞毒性颗粒蛋白是鼻NK／T细胞淋巴瘤诊断和鉴别诊断重要标志。     

外周T/NK细胞淋巴瘤治疗的进展

 外周T细胞淋巴瘤（PTCL）是一组异质性非常明显的淋巴瘤,起源于成熟T淋巴细胞,对传统化疗反应不佳,大部分患者预后差。对复发难治患者的治疗选择尚未达成共识。目前正在进行一些新的临床试验,探讨一些新的治疗方法和药物,如第一次缓解后采用自体干细胞移植进行巩固治疗的前瞻性临床研究,以及denileukin diftitox或者CD52单抗（alemtuzumab）等靶向治疗药物与化疗的联合等。文章对外周T／NK细胞淋巴瘤的流行病学、预后因子和目前的治疗进行了探讨。     

Synergistic anticancer effect of a combination of chidamide and etoposide against NK/T cell lymphoma

Natural killer/T cell lymphoma (NKTCL) is a highly aggressive hematological malignancy. However, there is currently no consensus on therapies for refractory/relapsed patients. In this study, we investigated the synergistic anticancer effect and potential mechanism of combining chidamide, a histone deacetylases (HDACs) inhibitor, and etoposide, a DNA-damaging agent, in NKTCL. We demonstrated that chidamide or etoposide alone dose- and time-dependently inhibited the cell viability of NKTCL cell lines, YT, NKYS and KHYG-1. Functional experiments suggested that combined chidamide and etoposide treatment exerted synergistic antiproliferation effect and enhanced cell apoptotic death in vitro and in vivo. Furthermore, the expression of DNA damage related proteins was detected and we also examined the alternations in histone acetylation, cell cycle progression, and mitochondrial membrane potential (MMP). The results suggested that increased histone acetylation, cell cycle arrest at the G2/M phase and loss of MMP, converging to greater DNA damage, might account for the synergism of the combination of chidamide and etoposide in NKTCL. Taken together, our study provides an evident for possible application on combining HDACs inhibitors and DNA-damaging agents for the treatment of NKTCL.     

Primary duodenal NK/T-cell lymphoma with massive bleeding: A case report

Primary natural killer/T-cell (NK/T-cell) lymphoma of the gastrointestinal tract is a very rare disease with a poor prognosis, and the duodenum is quite extraordinary as a primary lesion site. Here, we describe a unique case of a primary duodenal NK/T-cell lymphoma in a 26-year-old man who presented with abdominal pain and weight loss. Abdominal computed tomography scan demonstrated a hypodense tumor in the duodenum. Because of massive upper gastrointestinal tract bleeding during hospitalization, the patient was examined by emergency upper gastrointestinal endoscopy. Under endoscopy, an irregular ulcer with mucosal edema, destruction, necrosis, a hyperplastic nodule and active bleeding was observed on the duodenal posterior wall. Following endoscopic hemostasis, a biopsy was obtained for pathological evaluation. The lesion was subsequently confirmed to be a duodenal NK/T-cell lymphoma. The presenting symptoms of primary duodenal NK-/T-cell lymphoma in this patient were abdominal pain and gastrointestinal bleeding, and endoscopy was important for diagnosis. Despite aggressive treatments, the prognosis was very poor.     

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.     

Nasal natural killer (NK)/T-cell lymphoma: clinical, histological, virological, and genetic features

Nasal natural killer (NK)/T-cell lymphoma (NNKTL) is a clinical illness characterized by progressive unrelenting ulceration and necrosis of the nasal cavity and midline facial tissues. Histological features of the lymphoma include angiocentric and polymorphous lymphoreticular infiltrates, called polymorphic reticulosis. Surface antigens and the NK-cell marker, CD56, as well as pan-T antigen CD2, cytoplasmic CD3 (CD3ɛ), and CD45 are expressed in the lymphoma cells. The origin of the lymphoma is thought to be either NK-cell linkage without T-cell receptor (TCR) rearrangement or γδT-cell linkage with γδTCR rearrangement. Since the authors of this study first demonstrated the presence of Epstein Barr virus (EBV)-DNA and EBV oncogenic proteins in NNKTL, the lymphoma has been classified as one of the EBV-associated malignancies. The NNKTL cells produce interleukin (IL)-9, IL-10, and interferon-γ-inducible protein-10 (IP-10), possibly due to EBV-oncogenic proteins in the lymphoma cells, and such cytokines take an important part in the cell proliferation and invasion, acting in an autocrine manner. Clinically, the serum EBV-DNA copy number is useful as a specific tumor marker and a predictive prognostic factor. Even in early clinical stages, the lymphoma shows poor prognosis caused by the rapid progression of the lesion into distinct organs. Our newly designed arterial infusion chemotherapy, from the superficial temporal artery, in combination with radiotherapy, has shown a favorable outcome in patients with NNKTL. In this article, the clinical, pathological, and virological characteristics of the lymphoma are reviewed, along with a report of our investigations.     

Decline of natural cytotoxicity of human lymphocytes following infection with human T-cell leukemia/lymphoma virus (HTLV)

Cell-mediated natural cytotoxicity (CMNC) of fresh or long-term cultured lymphocytes collected from HTLV-positive patients or infected in vitro with the virus, was tested against K562 target cells. Severe depression of reactivity was found in fresh lymphocytes of three patients with advanced disease, in 12 in vitro established T-cell malignant lines, and two HTLV-infected cord blood (C5/MJ and C91/PL) lines. Moreover, all (eight) HTLV-1 infected cell lines listed showed a significant inhibition of CMNC of peripheral blood lymphocytes of healthy donors. Whether virus infection promotes the outgrowth of pre-existing suppressor cells and/or produce changes of the T-lymphocyte function is unknown.     

NK/T细胞淋巴瘤分期及预后研究进展

NK/T细胞淋巴瘤分为上呼吸道、消化道NK/T细胞淋巴瘤和非上呼吸道、消化道NK/T细胞淋巴瘤两个亚型.Ann Arbor分期并不令人满意,建议一种新的分期系统:局限期(无局部浸润的Ⅰ、Ⅱ期上呼吸道、消化道NK/T细胞淋巴瘤)和广泛期(有局部浸润的Ⅰ、Ⅱ期上呼吸道、消化道NK/T细胞淋巴瘤,Ⅲ、Ⅳ期上呼吸道、消化道NK/T细胞淋巴瘤,以及非上呼吸道、消化道NK/T细胞淋巴瘤).Ann Arbor分期和国际预后指数在鼻型NK/T细胞淋巴瘤预后中的作用还存在争议,又发现了新的预后因素,如肿瘤局部浸润、EB病毒、肿瘤生物学和微环境因素等.