骨原发恶性淋巴瘤5例临床分析

骨原发恶性淋巴瘤（Primary lymphoma of bone,PLB）是一种罕见类型的结外淋巴瘤,占所有非霍奇金淋巴瘤（NHL）的1%,占结外NHL的4%～5%,占全部骨原发肿瘤的7%.现总结我院2000年1月～2007年1月收治的5例PLB患者的临床资料,结合文献分析其临床特征、诊断、治疗和预后.[第一段]     

原发性骨淋巴瘤的诊疗进展

原发性骨淋巴瘤（PLB）是一类少见的结外淋巴瘤,占骨恶性肿瘤的3%~7%,占结外淋巴瘤约5%,占成年人所有淋巴瘤的2%。PLB可发生于任何年龄阶段,但好发于中老年人,平均发病年龄为45~60岁,男性略多于女性。PLB通常以病变部位疼痛或局部软组织肿胀起病,影像学表现无特异性,诊断必须通过手术取样行病理活组织检查来确诊。目前PLB以全身化疗联合受累骨放疗为主。文章对PLB的诊疗现状及进展进行综述。     

原发性骨淋巴瘤诊疗现状及进展

原发性骨淋巴瘤是一种罕见的结外淋巴瘤，缺少特征性的临床症状和影像学表现。化疗、放疗或联合治疗是原发性骨淋巴瘤患者常用的治疗方法。原发性骨淋巴瘤预后优于其他结外淋巴瘤。文章就原发性骨淋巴瘤的临床表现、病理诊断、治疗方法进行综述，以加深对原发性骨淋巴瘤的理解。     

新疆维吾尔自治区乌鲁木齐地区466例非霍奇金淋巴瘤临床病理特征分析

 目的　探讨新疆维吾尔自治区乌鲁木齐地区不同民族非霍奇金淋巴瘤（NHL）的临床病理特点。方法　收集466例NHL标本,复查HE和免疫组织化学染色结果,重新诊断、分型。结果　466例中B细胞性NHL 369例（79.2 %）,T细胞性NHL 97例（20.8 %）;结内193例（41.4 %）,结外273例（58.6 %）;最常见组织学类型为弥漫大B细胞淋巴瘤、小淋巴细胞淋巴瘤／慢性淋巴细胞白血病、黏膜相关淋巴组织结外边缘区B细胞淋巴瘤、NK／T细胞淋巴瘤、外周T细胞淋巴瘤、滤泡性淋巴瘤。维吾尔族T淋巴母细胞淋巴瘤（T-LBL）和间变性大细胞淋巴瘤（ALCL）比例分别为7.5 %（9／120）和4.2 %（5／120）,高于汉族的1.3 %（4／308）和2.3 %（7／308）（χ2＝11.276,P＝0.001;χ2＝1.137,P＝0.286）。而汉族结外NK／T细胞淋巴瘤比例为7.1 %（22／308）,高于维吾尔族的3.3 %（4／120）,差异无统计学意义（χ2＝2.196,P＝0.138）。其余各亚型在不同民族间差异皆无统计学意义（均P＞0.05）。结论　乌鲁木齐地区NHL结外发病高于结内,B细胞性淋巴瘤的发病明显高于我国内地。维吾尔族和汉族B-NHL发病构成在整体上差别不大,但是在T细胞NHL中,维吾尔族T-LBL和ALCL高于汉族,而汉族结外NK／T细胞淋巴瘤高于维吾尔族,这些差异是否与新疆维吾尔自治区的民族与地域差异有关,还需要进一步的研究证实。     

山东地区520 例淋巴瘤临床病理特点分析

目的:分析山东省地区近3 年淋巴瘤患者的病理类型及临床特点。方法:回顾性分析山东省肿瘤医院及山东省医学科学院附属医院2011年9 月至2014年9 月收治的520 例淋巴瘤患者资料,从性别、发病年龄、病理类型、发病部位等方面进行总结分析。结果:520 例淋巴瘤患者中,男女比例为1. 2 :1,中位年龄51岁,霍奇金淋巴瘤（HL）67例占12.9% ,非霍奇金淋巴（NHL ）453 例占87.1% 。NHL 中75.9% 为B 细胞淋巴瘤,24.1% 为T/NK细胞淋巴瘤。HL以经典型霍奇金淋巴瘤结节硬化型为主,占52.2% ,年龄分布未见双峰性。NHL 最常见类型为弥漫大B 细胞淋巴瘤（DLBCL ）占54.1% ,其构成比高于其他国家和地区（18% ～42.7%）,男女比例为0. 9:1,发病年龄略早（中位年龄54岁）。 HL和NHL 发病部位均以颈部淋巴结多见,且NHL 结外起病多于结内起病。结论:520 例淋巴瘤以DLBCL 最常见,多数亚型以男性发病比例较高,但DLBCL 以女性发病率较高。结外淋巴瘤以鼻腔及胃肠道多见。      

B细胞性非霍奇金淋巴瘤中BCL-6、CD10和BCL-2的蛋白表达及其临床病理意义

背景与目的：BCL-6、CD10均为淋巴结生发中心B淋巴细胞（GC—B细胞）的标志，它们在结内外弥漫性大B细胞淋巴瘤（DLBCL）及其它类型淋巴瘤中的表达特征及意义值得研究。本研究分析了B细胞性非霍奇金淋巴瘤（B—NHL）中BCL-6、CD10和BCL-2的蛋白表达及其临床病理意义。方法：免疫组化EnVision两步法分析135例B—NHL常见类型[包括DLBCL 22例，滤泡性淋巴瘤（FL）18例，B小淋巴细胞性淋巴瘤（B—SLL）18例，套细胞淋巴瘤（MCL）15例，淋巴浆细胞性淋巴瘤（LPL）7例，Burkitt’s淋巴瘤（BL）5例，B淋巴母细胞性淋巴瘤（LBL）3例；结外DLBCL 29例，胃粘膜相关淋巴样组织结外边缘区B细胞淋巴瘤（MALT-L）18例]和对照组5例T—NHL、5例结节型淋巴细胞为主型霍奇金淋巴瘤（NLPHL）以及10例淋巴结反应性增生（RLH）石蜡包埋组织中BCL-6、CD10以及BCL-2蛋白的表达。结果：①BCL-6和CD10阳性表达均只见于RLH（100％和100％）、结内DLBCL（72．7％和40．9％）、结外DLBCL（75．9％和41.4％）、FL（88．9％和72．2％）以及BL（100％和100％），其余B—NHL如B-SLL、MCL、MALT—L、LPL、LBL以及T-NHL和NLPHL中均未见BCL-6和CD10蛋白的表达。BCL-2蛋白表达可见于结内、结外DLBCL、FL、B—SLL、MCL、MALT—L以及LBL，阳性率分别为：36．4％、27．6％、83．3％、88．9％、86．7％、72．7％和33．3％；而LPL、BL、T—NHL以及NLPHL未见BCL-2蛋白表达；②DLBCL中BCL-6的表达形式可以分为四种类型：GC／FL型、中间型、散在型和阴性型；③40．9％的结内DLBCL、41．4％的结外DLBCL、72．2％的FL以及100％的BL为BCL-6＋／CD10＋表达，其中BCL-6蛋白表达均为GC／FL型；④在临床特征上，BCL-6＋／CD10＋的结内DLBCL与非BCL-6＋／CD10＋的结内DLBCL相比，前者的临床分期低于后者（P〈0．05）。结论：BCL-6、CD10和BCL-2蛋白的联合检测，可以用于部分B—NHL的诊断和鉴别诊断；BCL-6＋／CD10＋的结内淋巴瘤可能具有更好的临床预后。     

结直肠原发性非霍奇金淋巴瘤的临床研究

目的探讨结直肠原发性非霍奇金淋巴瘤（NHL）的诊断和治疗。方法回顾性分析2005至2011年收治的19例结直肠原发性NHL患者的临床资料,所有患者均经手术切除或探查病理活检证实。结果临床表现缺乏特异性,类似于同部位的结直肠癌。术前确诊率为57.9%（11/19）,19例患者均行手术探查,手术切除率为89.5%（17/19）,其中根治性手术为68.4%（13/19）。术后病理均诊断为非霍奇金淋巴瘤。临床分期 ⅠE期4例,Ⅱ E期9例,Ⅲ E期6例。15例患者术后辅以化疗,采用CHOP方案为基础的化疗方案,CD20阳性患者加用利妥昔单抗,化疗加放射治疗4例。结论结直肠原发性NHL术前诊断困难,早期诊断对改善预后十分重要,应综合临床表现、影像学及内窥镜检查结果,确诊依靠病理检查。治疗采用根治性手术切除联合CHOP为基础的方案化疗。     

67例原发性结外非霍奇金淋巴瘤早期误诊分析及首发症状回顾

目的提高临床各专科医师对原发性结外非霍奇金淋巴瘤（PENL）的认识，减少PENL的早期误诊。方法对早期均误诊为其他疾病、后经病理学检查确诊的67例PENL患者的误诊原因及首发症状进行分析及回顾。结果误诊原凼：1）PENL早期临床表现多样，无特异性；2）各专科医师对PENL认识不足；3）病理活检取材困难或患者早期不接受病理检查而贻误诊断；4）病理误导；5）对诊断NHL有一定提示作用的LDH和β2-MG的检查重视不够。结论对町疑为PENL的病例要尽早对可疑病变部位进行病理检查，并要注意多次、多部位检查和动态观察，必要时应开胸、开腹探察和切脾病理活检。     

原发性肾上腺非霍奇金淋巴瘤五例并文献复习

 目的　研究原发性肾上腺恶性淋巴瘤（PAL）的临床特征、诊治要点及预后。方法　回顾性分析1975年1月至2008年10月间收治的5例PAL患者的症状、体征,影像学表现及实验室检查等临床资料,并结合国内外文献进行分析。结果　5例患者中,1例因常规体检发现,4例以腰痛或腹痛就诊。实验室检查无明显异常。影像学检查仅肾上腺区发现肿物,临床诊断为"肾上腺癌"或"嗜铬细胞瘤",但术后病理诊断却证实为非霍奇金淋巴瘤（NHL）,其中B细胞淋巴瘤（B-NHL）3例,T细胞淋巴瘤（T-NHL）2例。5例患者术后均接受了以CHOP或CHOP样方案化疗为主的综合治疗。随访至2008年10月,1例小B细胞性淋巴瘤患者生存8年,余4例均在1年内死亡。结论　PAL是极为罕见的结外淋巴瘤,临床和影像学表现不典型,误诊率极高,确诊依靠组织病理学检查。病理类型主要为NHL,B-NHL和T-NHL均不少见。术后5年生存率低,病理类型影响该病的预后。     

18F-FDG PET/CT显像特征与恶性淋巴瘤病理类型的相关性

摘 要：［目的］ 探讨18F-FDG PET/CT显像特征与恶性淋巴瘤病理类型的关系。［方法］ 回顾性入组2013年1月至2016年6月18F-FDG PET/CT显像考虑为淋巴瘤可能的74例患者（男性41例,女性33例）,穿刺、手术病理明确最终诊断。分析18F-FDG PET/CT诊断与病理诊断一致性。同时探讨PET/CT显像特征如是否伴脾大、结外侵犯、浅表及深部淋巴结累及、SUVmax等与病理类型的相关性。［结果］ 74例18F-FDG PET/CT考虑为淋巴瘤可能的患者中,最终病理明确为淋巴瘤者占70.27%（52/74）,淋巴瘤SUVmax明显高于非淋巴瘤（10.68±4.49 vs 5.04±2.63,t=-5.490,P=0.001）,其中HD占25.00%（13/52）,NHL占75.00%（39/52）。HD组发病年龄小于NHL组（P=0.001）;18F-FDG PET/CT 显像HD组累及深部淋巴结及结外器官与NHL组相比,差异均有统计学意义（P=0.031,P=0.013）;浅表淋巴结累及组间差异无统计学意义。NHL组SUVmax高于HD组（11.47±4.86 vs 8.55±2.29,Z=-2.188,P=0.029）。［结论］ 不同病理类型淋巴瘤18F-FDG PET/CT显像特征不同,其中弥漫大B细胞淋巴瘤累及范围广泛,NK/T细胞淋巴瘤以累及鼻腔多见。     

Primary non-Hodgkin's lymphoma of bone.

Primary non-Hodgkin's lymphoma of bone (PLB) constitutes approximately 5% of all extranodal non-Hodgkin's lymphoma (NHL) and 7% of primary bone tumors. The peak incidence for PLB is in the fifth decade, with a slight preponderance of males over females. The presenting symptoms usually consist of localized bone pain and occasionally a palpable mass. Most patients with PLB have B-cell tumors with a diffuse mixed-cell or diffuse large cell histology. While most patients present with early-stage disease, it is not clear whether such patients benefit from combined-modality therapy (CMT) consisting of radiation therapy (RT) and chemotherapy (CT) compared with either RT or CT alone. However, there is strong evidence that CMT is beneficial in the treatment of localized NHL, and these results might be applicable to the therapy for PLB. Nevertheless, only a phase III randomized, controlled clinical trial will determine whether CMT is superior to either CT or RT alone.     

非霍奇金淋巴瘤中枢神经系统累及的诊断和预防

 非霍奇金淋巴瘤（NHL）患者中枢神经系统（CNS）累及预后不良,其中位生存期2～6个月。与NHL CNS累及相关参数是年轻、进展期、累及结外部位数、乳酸脱氢酶（LDH）增高和国际预后指标（IPI）积分。最有希望的治疗为自体造血干细胞移植,可延长中数生存期10～26个月。处于CNS侵袭高危状态的某些NHL亚型患者需要早期进行CNS预防,如伯基特淋巴瘤（BL）和淋巴母细胞淋巴瘤（LBL）。弥漫性大B细胞淋巴瘤（DLBCL）初期治疗时是否需应用CNS预防久有争议,因为它属于CNS累及（≈5 ％）的低危群体。危险模式的确定有助于预示NHL的CNS复发。     

原发性结外非霍奇金淋巴瘤127例临床特征及误诊分析

目的分析淋巴结外非霍奇金淋巴瘤(NHL)的临床表现特点及误诊原因。方法回顾性分析127例原发结外非霍奇金淋巴瘤的发病情况、原发部位分布情况、首发症状及误诊情况等。结果原发于结外的非霍奇金淋巴瘤12／例，占同期非霍奇金淋巴瘤53．81％(127／236)；原发部位前5位依次为：胃肠道21．26％(27／127)，鼻腔18．9％(24／127)，Waldeyer环14．96％(19／127)，脾脏12．6％(16／127)，皮肤6．3％(8／127)；结外奇非霍金淋巴瘤误诊率为69．29％(88／127)；结外非霍奇金淋巴瘤首发症状因原发部位不同而临床表现各异，无特异性。结论结外NHL发生率较高，其临床表现缺乏特异性，早期易误诊。     

原发结内外周T细胞淋巴瘤19例临床特征及预后分析

 目的　分析原发结内外周T细胞淋巴瘤（PTCL）的临床特点、治疗和预后。方法　回顾性分析19例原发结内PTCL患者的临床资料、治疗反应以及预后因素。结果　19例患者中位发病年龄54岁,男女比例2.17∶1,其中94.7 %（18／19）为Ⅲ～Ⅳ期,84.2 %（16／19）有B症状,84.2 %（16／19）有结外器官受累,57.9 %（11／19）有骨髓浸润。化疗完全缓解（CR）率36.8 %（7／19）,2年总生存（OS）率47.4 %,2年无进展生存（PFS）率25 %。预后分析显示,结外侵犯数量（EN）≥2个、美国东部肿瘤协作组（ECOG）体能状态评分≥2分、国际预后指数（IPI）评分＞2分以及β2-微球蛋白（β2-MG）升高为不良预后因素。结论　原发结内PTCL是一类高度侵袭的异质性T细胞淋巴瘤,化疗效果差,多项因素提示不良预后。     

Treatment of localized primary non-Hodgkin's lymphoma of bone in children: a Pediatric Oncology Group study.

PURPOSE: The treatment of primary lymphoma of bone (PLB) in children has traditionally included radiotherapy to the primary site; more recently, it has included systemic chemotherapy. Because of concern about the untoward effects of treatment in a disease that is curable, we attempted to determine whether radiotherapy can be safely excluded from treatment. PATIENTS AND METHODS: The results of three consecutive Pediatric Oncology Group (POG) studies were examined to determine the impact on outcome of radiotherapy as adjunctive treatment in children and adolescents receiving chemotherapy for early-stage primary lymphoma of bone. RESULTS: From 1983 to 1997, 31 patients with localized PLB were entered onto POG studies of early-stage non-Hodgkin's lymphoma (NHL). Between 1983 and 1986, seven patients were treated with 8 months of chemotherapy with irradiation (XRT) of the primary site. After 1986, patients were treated without XRT; four received 8 months of chemotherapy, and 20 received 9 weeks of chemotherapy. Primary sites were the femur (nine), tibia (eight), mandible (five), mastoid (one), maxilla (one), zygomatic arch (one), rib (one), clavicle (one), scapula (one), ulna (one), talus (one), and calcaneous (one). Histologic classification revealed 21 cases of large cell lymphoma, five cases of lymphoblastic lymphoma, two cases of small, noncleaved-cell lymphoma, and three cases of NHL that could not be classified further. One patient relapsed at a distant site 22 months after completion of therapy. There have been no deaths. CONCLUSION: Localized PLB is curable in most children and adolescents with a 9-week chemotherapy regimen of modest intensity, and radiotherapy is an unnecessary adjunct.     

Primary extranodal non-Hodgkin's lymphoma (NHL): the impact of alternative definitions tested in the Comprehensive Cancer Centre West population-based NHL registry.

BACKGROUND: The definition of primary extranodal non-Hodgkin's lymphoma (NHL) is a controversial issue, especially in patients where both nodal and extranodal sites are involved. PATIENTS AND METHODS: The impact of different definitions of primary extranodal NHL on incidence and prognosis is explored using data from a population-based NHL registry. RESULTS: Using liberal criteria, 389 (34%) cases were classified as primary extranodal NHL. Overall survival (OS) rates of nodal and extranodal NHL patients defined this way were comparable; however, extranodal NHL patients had a better disease-free survival (DFS). When strict criteria were applied, 231 cases (20%) were classified as primary extranodal NHL. OS and DFS rates of extranodal NHL patients defined this way were superior to nodal NHL patients; however, the difference in OS was reversed after correction for differences in International Prognostic Index and malignancy grade. CONCLUSION: This study illustrates the selection bias that is introduced when a strict definition of primary extranodal NHL, that excludes cases with disseminated disease, is used. Patients with primary extranodal NHL were found to have a superior DFS, irrespective of which definition of primary extranodal NHL was used.     

Primary extranodal and nodal non-Hodgkin's lymphoma: A survey of a population-based registry

In a population-based registry, there were 580 patients with non-Hodgkin's lymphoma (NHL); 236 had primary extranodal lymphoma (41%). The initial localization of the primary extranodal lymphomas varied markedly, although 36% were primary gastrointestinal lymphomas. Histological classification was performed by a regional panel of pathologists according to the Kiel Classification and the International Working Formulation. Twelve per cent of the patients with nodal NHL had a localized disease in contrast to 40% with primary extranodal NHL. Low grade lymphomas were encountered in 30 and 10% of the patients with primary nodal and extranodal NHL, respectively. Recurrence-free survival rate for patients with localized low-grade malignancy and disseminated intermediate grade NHL is significantly better for extranodal lymphoma than for nodal NHL. Patients with disseminated high-grade extranodal NHL had the worst prognosis of all. We conclude that primary nodal and primary extranodal lymphomas should be considered as distinctive and separate entities.     

头颈部淋巴瘤计算机体层摄影、磁共振成像影像特点及临床病理分析

 【摘要】 目的 探讨头颈部结内型及结外型淋巴瘤的计算机体层摄影（CT）与磁共振成像（MRI）影像特点及临床病理特点。方法 分析46例经手术病理或穿刺活检证实的头颈部淋巴瘤患者临床病理及CT、MRI资料,结合文献复习,评价头颈部淋巴瘤的影像特点及临床、病理特点。结果 46例中38例为非霍奇金淋巴瘤（NHL）,8例为霍奇金淋巴瘤（HL）。结外型21例（45.65 %）,包括原发鼻腔10例、咽环（Waldeyer环）7例、喉部2例、甲状腺及腮腺各1例,其中13例伴发颈部淋巴结转移;结内型25例（54.35 %）,主要累及颈部Ⅱ～Ⅳ区淋巴结。Ann Arbor分期：Ⅰ期14例,Ⅱ期19例,Ⅲ期0例,Ⅳ期13例。根据病变形态分为4型：多发结节型25例,肿块型11例,弥漫肿胀型8例,溃疡坏死型2例。结论 头颈部淋巴瘤的CT、MRI影像表现有一定特点,影像检查对淋巴瘤的诊断、鉴别诊断及观察侵犯范围有一定临床价值。     

Non-hodgkin’s lymphoma of the frontal sinus

Non-Hodgkin’s lymphoma (NHL) of the head and neck area is rare, and constitutes 0.4%–2% of all extranodal lymphomas and 6.4%–13% of extranodal lymphomas of the head and neck area^1–6. The maxillary antrum, nasal cavity and ethmoid sinus are the most common sites for this tumor³. Prognosis of the NHL affecting the paranasal sinuses and nasal cavity is poor with 33% of survival at 5 years^5–7. Failure of the treatment is local and with distant metastasis. Radiotherapy permits disease-free survival at 5 years in T1–T2 of 78% and in T3–T4 of 18%⁷. Only three cases in the literature have been described affecting the frontal sinus, in two cases there was infiltration of the other paranasal sinuses and in one case there was an isolated primary non-Hodgkin’s lymphoma of the frontal sinus^3,4 (Medline from 1966 to 2001). We present the second case reported in the literature of NHL exclusively affecting the frontal sinus showing local control 5 years after diagnosis.     

CNS involvement in primary mediastinal large B-cell lymphoma.

PURPOSE: The risk of CNS involvement by non-Hodgkin's Lymphoma (NHL) has been associated with bone marrow and/or testicular involvement; however, it was recently reported that the number of extranodal sites is a more reliable predictor of CNS disease. Because primary mediastinal thymic large B-cell lymphoma (PMLCL) has a high propensity for involving extranodal sites, we investigated the frequency and pattern of CNS involvement in PMLCL. PATIENTS AND METHODS: The medical records of 219 patients with aggressive NHL, consecutively entered onto protocols at the National Cancer Institute between 1987 and 1998, were retrospectively reviewed. RESULTS: Twenty-three patients (11%) had clinical and pathologic features of PMLCL. These patients were young (median age, 29 years), female (61%), and presented with massive mediastinal adenopathy (70%). Extranodal disease occurred at presentation in 70% and at relapse in 93% of patients and involved contiguous intrathoracic structures and/or distant sites, including the lungs, kidneys, liver, adrenals, ovaries, pancreas, and bone. Six patients (26%) developed CNS involvement, two (9%) at presentation and four (27%) at relapse. All had extranodal disease, but only one had bone marrow involvement. Parenchymal and leptomeningeal CNS disease occurred in four and three patients, respectively. CONCLUSION: CNS involvement in PMLCL is associated with extranodal involvement other than bone marrow and may reflect the unique biology of this disease. The propensity to involve the CNS parenchyma raises the concern that intrathecal prophylaxis may not be effective and suggests that CNS imaging should be considered in patients with extranodal disease.