33例结外NK/T细胞淋巴瘤临床分析

目的:分析结外NK/T细胞淋巴瘤的临床特征、生存时间及影响预后的因素。方法:回顾性分析我院收治的结外NK/T细胞淋巴瘤33例患者的临床资料,分析临床特征、生存时间及影响预后的因素。结果:共收集结外NK/T细胞淋巴瘤患者,男女比例为2∶1,发病年龄30～50岁。Ann Arbor分期:Ⅰ期10例(30.3%),Ⅱ期5例(15.2%),Ⅲ期2例(6.06%),Ⅳ期16例(48.5%)。B组患者21例(63.6%),乳酸脱氢酶升高23例(69.7%),血β2-微球蛋白升高26例(78.8%),病变组织中EBER阳性21例(63.6%)。33例患者中死亡22例,生存11例,中位生存时间12个月,2年总生存(OS)率39.4%。单因素分析结果显示,存在B组症状、体能状况差(ZPS≥2)、LDH/血β2-微球蛋白升高、EBER阳性、临床分期≥Ⅲ期者预后差。结论:NK/T细胞淋巴瘤患者预后差,影响预后的因素包括B组症状、ZPS评分、LDH/血β2-微球蛋白水平、EBV感染、临床分期等因素。     

鼻型结外NK/T细胞淋巴瘤一例误诊分析

NK/T细胞淋巴瘤是一种少见的恶性疾病,曾被称为多形性网织细胞增生症、血管中心性T细胞淋巴瘤、致死性中线肉芽肿等.该病主要多发于亚洲及南美洲,有报道中国人T细胞淋巴瘤仅占所有淋巴瘤的10％,NK细胞淋巴瘤也仅约占6％[1].由于该病发病率低,早期临床表现多样,临床易误诊.我科收治了1例以反复咽痛起病的NK/T细胞淋巴瘤,发病半年后方确诊.现报道如下.     

NK／T细胞淋巴瘤的治疗进展

NK／T细胞淋巴瘤属于结外非霍奇金淋巴瘤（NHL）的一种少见特殊类型，占NHL的5％～15％左右。1982年Karcher等报道了口咽部发生T细胞淋巴瘤，同年Ishii等提出了鼻T细胞淋巴瘤为致死性中线肉芽肿的特殊类型。由于该肿瘤常侵犯并破坏血管，故1994年欧美改良淋巴瘤分类将其命名为血管中心性淋巴瘤。1997年WHO提出结外NK／T细胞淋巴瘤的概念，2001年将其作为一种独立的临床病理分型正式列入WHO关于恶性淋巴瘤的新分类，由于NK／T细胞淋巴瘤多原发于鼻腔，因此称鼻NK／T细胞淋巴瘤；原发于鼻以外的NK／T细胞淋巴瘤则称鼻型NK／T细胞淋巴瘤。     

不同部位原发性胃肠道淋巴瘤的临床特点

目的 研究不同部位原发性胃肠道淋巴瘤(PGIL)的临床特点,以提高对该疾病的诊断水平.方法 回顾性分析武汉地区8家医院1999年1月至2007年6月经病理确诊的PGIL患者,共有202例资料完整的病例用于最后统计,按部位分为胃、小肠、大肠淋巴瘤三组,并比较三组间的临床特点.结果 PGIL发生于胃113例(56.0%)、小肠37例(18.3%)、大肠52例(25.7%);男130例(64.4%),女72例(35.6%),均以男性发病率为高.胃淋巴瘤组比小肠淋巴瘤组的病程长(3个月比1个月,P=0.013).三组PGIL均以腹痛、贫血发生率最高,其中伴有贫血症状者大多为轻度(57.9%).PGIL临床分期均以Ⅰ E期、ⅡE期为主,占71.3%;与胃淋巴瘤组比较,大肠淋巴瘤组的临床分期相对较重(P=0.014);PGIL主要病理类型为黏膜相关淋巴组织(MALT)淋巴瘤、弥漫大B细胞淋巴瘤、T细胞淋巴瘤.胃淋巴瘤组以低度恶性MALT淋巴瘤多见(56.9%),而小肠淋巴瘤组以T细胞淋巴瘤多见(34.4%),大肠淋巴瘤组以高恶性B细胞淋巴瘤多见(51.1%).PGIL病灶类型以隆起肿块型、溃疡型为主,但与胃淋巴瘤组相比,大肠淋巴瘤组以隆起肿块型居多而溃疡型少见(P=0.000).胃、小肠、大肠淋巴瘤组经内镜并活检的确诊率分别为58.7%(61/104)、25.0%(4/16)、48.2%(13/27).结论 不同部位PGIL的临床症状、病理分型,临床分期、病灶类型,内镜检出率各有特点,这些差异有助于临床医师对PGIL的认识及诊断.     

T和NK细胞性白血病的诊断分析

目的:分析成熟NK/T细胞性白血病/淋巴瘤的临床特点,提高其诊断率。方法:对3例确诊为成熟NK/T细胞淋巴瘤/白血病患者的临床资料进行回顾性分析,分析其临床表现、血常规、骨髓细胞学、外周血或骨髓细胞流式免疫分型及TCR、IgH基因重排的特点。结果:3例均有不同程度乏力、发热、浅表淋巴结和脾肿大、贫血。血常规淋巴细胞比例(2例为62%、69%)或单核细胞比例(1例,25%)增高。白细胞计数(WBC)减少2例,正常1例,外周血细胞流式免疫分型+TCR、IgH基因重排,2例呈成熟T淋巴细胞白血病,1例呈异常NK细胞性。骨髓象及骨髓活检发现白血病/淋巴瘤细胞侵润。结论:对临床表现乏力、发热,白细胞计数减少或正常,而淋巴细胞或单核细胞比例增高的患者,除注意检查皮疹、结节,淋巴结、脾、肝肿大,骨髓象及骨髓活检外,检测外周血细胞或骨髓细胞的流式免疫表型、TCR及IgH基因重排,有助于成熟NK/T细胞白血病/淋巴瘤的诊断。     

原发于胰腺的NK/T细胞淋巴瘤1例

原发于胰腺的结外NK/T细胞淋巴瘤极其罕见, 其术前较难明确诊断且预后较差。本文报道1例该类淋巴瘤的诊治经验, 结合影像学及病理检查, 以提高临床对结外NK/T细胞淋巴瘤的认识。     

原发性肠道T细胞淋巴瘤临床特点分析

背景:原发性肠道T细胞淋巴瘤是一类起源于肠上皮内T淋巴细胞的恶性肿瘤,过去被称为“肠道恶性组织细胞增生症”,近年来发现这一疾病的本质是肠道T细胞淋巴瘤。该病临床表现复杂,病程进展迅猛,内镜和消化道钡餐检查很难确诊。目的:了解原发性肠道T细胞淋巴瘤的临床特点。方法:对仁济医院1994年9月～2004年9月6例原发性肠道T细胞淋巴瘤的病史资料进行回顾性分析。结果:本组原发性肠道T细胞淋巴瘤患者年龄34～73岁,男女比例为5∶1。病变多位于空、回肠,可有结肠累及。临床表现以腹痛、腹泻、发热、消瘦为主,部分患者并发肠穿孔、肠梗阻、消化道出血,无患者伴有乳糜泻。内镜下3例患者表现为溃疡病灶,术中见溃疡5例,肿块1例。1例患者经术前内镜活检病理检查确诊,5例由术后病理检查确诊,4例曾被误诊为炎症性肠病。淋巴瘤细胞的免疫表型为白细胞共同抗原(LCA)( )、CD45RO( )、CD3( )、CD30(-)。所有患者均接受手术治疗,部分结合术后化疗。3例患者于术后3个月内死亡。结论:不伴有乳糜泻是本组原发性肠道T细胞淋巴瘤的特点之一。患者的临床表现以一些非特异性症状为主,常被误诊为炎症性肠病。内镜活检和手术标本的病理学检查是目前确诊原发性肠道T细胞淋巴瘤的主要依据。该病预后极差。     

NK／T 细胞淋巴瘤的治疗

结外 NK／T 细胞淋巴瘤（ENKTL）属于结外非霍奇金淋巴瘤（NHL）的一种少见特殊类型,约占所有淋巴瘤的6％。ENKTL 在亚洲多见,而在欧美国家少见。其恶性度高,对常规化疗敏感性较低,容易产生耐药、常见治疗后早期复发,预后不佳。早期无伴高危因素的 ENKTL以放射治疗为主,治愈率较高;而中晚期疾病采用以化疗为主的综合治疗模式,整体预后较差。近年来,关于ENKTL 的综合治疗在不断进步,本文综述如下。     

以急性呼吸窘迫症为表现的原发性肺NK/T细胞淋巴瘤1例临床分析

目的报道1例以急性呼吸窘迫症为表现的原发性肺NK/T细胞淋巴瘤,分析NK/T细胞淋巴瘤的临床特点和诊断治疗。方法通过1例骨髓病理证实的原发性NK/T淋巴瘤的病例分析,结合文献,对原发性NK/T淋巴瘤的发病机制、临床特点、诊断、治疗及预后进行分析。结果 NK/T细胞淋巴瘤原发于肺部且以急性呼吸窘迫为表现比较罕见,具有特殊的免疫表型和临床症状特点,此病恶性程度高,疾病进展快,治疗效果及预后差。结论原发性肺NK/T淋巴瘤临床罕见,诊断难度大,预后差,目前缺少有效治疗方法。     

鼻型结外NK/T细胞淋巴瘤治疗进展

鼻型结外NK/T细胞淋巴瘤(NK/TCL)好发于结外,在我国原发于鼻腔和副鼻窦淋巴瘤中NK/TCL占40%～70%。NK/TCL主要为青年男性,2/3患者为局部累及,骨髓累及不足10%,常伴有B症状。该病与EB病毒(EBV)感染关系密切,可能为该病发病率地域性差异较大的原因之一。     

Haemophagocytic lymphohistiocytosis and Epstein–Barr virus: a complex relationship with diverse origins,expression and outcomes

Epstein–Barr virus (EBV) is a ubiquitous herpesvirus with rare but severe potential for lymphoproliferative complications. EBV is associated with a variety of presentations of haemophagocytic lymphohistiocytosis (HLH). HLH is a life-threatening hyperinflammatory syndrome that can occur in patients with genetic defects associated with dysregulation of the immune response (familial HLH) or arise in patients with underlying infection or malignancy (non-familial or secondary HLH). EBV can both serve as the incidental trigger of familial HLH or as the driving factor in patients with selective inherited vulnerability (e.g. X-linked lymphoproliferative disease). Alternatively, acute infection can idiosyncratically cause non-neoplastic HLH in patients without inherited predisposition (i.e. secondary HLH), while EBV-associated T/natural killer (NK)-cell lymphoproliferative disorders and lymphomas can cause neoplasia-associated HLH. The present review will discern between EBV-associated familial and non-familial HLH and highlight diagnostic and therapeutic considerations. Non-familial EBV-associated HLH is a major diagnostic dilemma, as it represents a diverse spectrum of disease ranging from highly curable (non-neoplastic EBV-HLH) to indolent but incurable (chronic active EBV) to acutely fatal (systemic EBV-positive T-cell lymphoma of childhood). Increased clinical awareness and understanding of this rare and potentially devastating subset of EBV-related complications is desperately needed to improve survival for patients with neoplasia-associated HLH.     

Quiescent nasal T/NK cell lymphoma manifested as primary central nervous system lymphoma

A 57-year-old man was diagnosed as primary T/NK-cell central nervous system lymphoma (CNSL) with intraocular involvement. However, review of a surgical specimen taken three years before for chronic paranasal sinusitis revealed an overlooked nasal T/NK cell lymphoma (TNKL), which showed similar histomorphology and immunophenotype with the CNS disease. Another patient, a 43-year-old woman, was initially diagnosed as a rare primary leptomeningeal T-cell lymphoma with ocular manifestation. Three years later, an isolated nasal TNKL emerged. Immunohistochemical and cytogenetic studies confirmed the same nature of the CNSL and the nasal TNKL. The nasal TNKLs of both patients had a strong expression of CD3, CD56, and Epstein-Barr virus antigens, but features of angiodestruction and mucosal ulceration were absent. We propose that: 1. a locally silent “quiescent” form of nasal TNKL may exist; and 2. a thorough examination and even blind biopsy of the nasal cavity is indicated when primary T/NK-cell CNSL is diagnosed. Am. J. Hematol. 60:161–163, 1999. © 1999 Wiley-Liss, Inc.     

Primary,solitary, adult T-cell leukemia / lymphoma of the descending colon

We herein report a patient with adult T-cell leukemia/lymphoma (ATLL) of the descending colon. A 64-year-old man was admitted to our hospital complaining of left lower abdominal pain. Endoscopic examination revealed an ulcerative tumor in the descending colon that was diagnosed as T-cell lymphoma by biopsy. Neither distant organ metastasis nor lymph node swelling was observed by radiographic examinations. Curative excision with left hemicolectomy and regional lymph node dissection was performed. Surgical sections contained ulcerative and superficially elevated lesions; these were continuous with each other. Histological examination revealed diffuse proliferation of medium-sized abnormal lymphoid cells. Immunohistochemically, these lymphoid cells were positive for UCHL-1/CD45RO and CD3 and negative for CD79a, indicating that the tumor was a primary malignant T-cell lymphoma of the descending colon. Integration of the proviral DNA of human T-lymphotropic virus type 1 (HTLV-1) was confirmed by Southern blotting analysis.     

T-cell/natural killer cell lymphoblastic lymphoma with an unusual coexpression of B-cell antigens

Received: 28 October 1999 / Accepted: 10 April 2000     

L-Asparaginase—Based Regimen in the Treatment of Refractory Midline Nasal/Nasal-Type T/NK-Cell Lymphoma

Nasal, nasal-type T-cell/natural killer cell (T/NK-cell) lymphoma is a rare disease, and its prognosis is poor. Between March 1992 and March 2002 we investigated a new L-asparaginase-based salvage regimen to treat the disease and improve response to treatment and 5-year overall survival rate. Eighteen patients with refractory midline nasal, nasal-type T/NK-cell lymphoma, who were resistant to a cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP)-like regimen, received an L-asparaginase-based salvage regimen (L-asparaginase, vincristine, and dexamethasone). Primary involved field radiation was given to the patients after chemotherapy. Ten (55.6%) of the patients achieved complete response (CR). Five patients (27.8%) achieved partial response (PR). The overall response rate (CR + PR) was 83.3%. The 5-year overall survival rate was 55.6%. Results of the preliminary clinical study indicated that the L-asparaginase-based salvage regimen significantly improved the response rate and 5-year survival rate. The findings suggested that the therapy is a promising new salvage regimen for treating refractory midline nasal, nasal-type T/NK-cell lymphoma.     

Chromosomal abnormalities in adult T-cell leukemia/lymphoma (ATL)

Summary Chromosomal studies were performed on six patients with adult T-cell leukemia (ATL). Structural abnormalities of chromosome 3 were the most common. In one case a complete loss of the short arm of chromosome 10 (10 p-) was noted while in another case a balanced translocation involving chromosome 10 p and 4q was found. These abnormalities have not been previously reported. After reviewing the literature, it was concluded that chromosomes 3, 6, 10, 13, 14, and X were most frequently involved in abnormalities. Specific and consistent chromosomal abnormalities were noted in each study. Therefore, it is hypothesised that the mutation rate for this virus may be higher than first expected. Furthermore, the relative consistency of heterogenous findings in different localities may reflect a geographic clustering of specific chromosomal abnormalities which may in turn be related to specific and geographically associated viral mutations. To support these suggestions not only are more cytogenetic data required but a molecular evaluation of these patients must be carried out to establish a relationship, it any, between genetic abnormalities and the epidemiology of ATL.     

THP‐COP regimen for the treatment of peripheral T‐cell lymphoma and adult T‐cell leukemia/lymphoma: a multicenter phase II study

Objective: The efficacy of pirarubicin (THP)‐COP was previously compared with cyclophophamide + doxorubicin + vincristine + prednisolone (CHOP) in elderly patients with lymphoma. The subset analysis showed that T‐cell lymphoma had a significantly better response with THP‐COP, whereas no such difference was observed in B‐cell lymphoma. The aim of this study is to confirm the efficacy of THP‐COP in the treatment of T‐cell lymphoma. Methods: We underwent a multicenter phase II study of THP‐COP as a first‐line treatment for T‐cell lymphoma. The overall response rate, survival period, and toxicity were analyzed. Results: Fifty‐three patients were enrolled in this study. Seventeen patients had peripheral T‐cell lymphoma (PTCL), including nine of PTCL not otherwise specified (PTCL‐NOS) and eight of angioimmunoblastic T‐cell lymphoma (AITL). Thirty‐six patients had adult T‐cell leukemia/lymphoma (ATLL), including 20 of acute type and 16 of lymphoma type. A treatment response was obtained in 35 (66%) patients, including 17 (32%) complete responses. Median overall survival (OS) and progression‐free survival (PFS) times were 14.3 months and 5.2 months, respectively. Patients with ATLL showed a tendency to obtain low response rate (61% vs. 77%, P = 0.27) and had a significantly inferior OS (13.3 vs. 28.6 months, P = 0.04) and PFS (4.6 vs. 8.1 months, P = 0.01) in comparison with PTCL. Grade 3 to 4 neutropenia, anemia, and thrombocytopenia occurred in 72%, 34%, and 58% of the patients, respectively. Febrile neutropenia was observed in 51% and grade 3 non‐hematological toxicities in 2–9% of the patients. Conclusion: The efficacy of THP‐COP is equivalent to that of CHOP for the first‐line therapy in T‐cell lymphoma.     

Clonal chromosomal abnormalities as direct evidence for clonality in nasal T/natural killer cell lymphomas

Nasal T/natural killer (NK) cell lymphoma is a distinct clinicopathologic entity which is more prevalent in Asia than in America and Europe. The clonal nature of the infiltrating lymphoid cells is difficult to demonstrate because of the lack of immunologic markers for clonality and the absence of clonal T-cell receptor gene rearrangement in most cases. In this study, clonal chromosomal abnormalities were detected in the tumour cells from four patients with nasal T/NK cell lymphoma. This finding provided direct evidence for clonality of the disease. Moreover, nonrandom cytogenetic abnormalities, including isochromosome for the short arm (p) of chromosome 6, isochromosome for the long arm (q) of chromosome 1, partial deletion of 6q, and aberrations at 11q, were disclosed. Isochromosome 6p was the sole structural abnormality in one patient, which may be a pathognomonic change in nasal lymphoma.     

Diagnosis and management of cutaneous lymphomas and lymphoid proliferations in children,adolescents and young adults (CAYA)

Cutaneous lymphomas and lymphoid proliferations (LPD) in children, adolescents, and young adults (CAYA) are a heterogeneous group of lymphoid neoplasms that present formidable diagnostic challenges to clinicians and pathologists alike. Although rare overall, cutaneous lymphomas/LPD occur in real-world settings and awareness of the differential diagnosis, potential complications, and various therapeutic approaches will help ensure the optimal diagnostic work-up and clinical management. Lymphomas/LPD involving the skin can occur as primary cutaneous disease in a patient that characteristically has lymphoma/LPD confined to the skin, or as secondary involvement in patients with systemic disease. This review will comprehensively summarize both primary cutaneous lymphomas/LPD that occur in the CAYA population as well as those CAYA systemic lymphomas/LPD with propensity for secondary cutaneous involvement. Focus on the most common primary entities occurring in CAYA will include lymphomatoid papulosis, primary cutaneous anaplastic large cell lymphoma, mycosis fungoides, subcutaneous panniculitis-like T-cell lymphoma, and hydroa vacciniforme lymphoproliferative disorder.