原发性乳腺T淋巴母细胞淋巴瘤/白血病一例并文献复习

  目的　探讨1例原发性乳腺T淋巴母细胞淋巴瘤／白血病（T-LBL／ALL）的临床病理特征及治疗策略,提高对原发性乳腺T-LBL／ALL的认识。方法　回顾分析1例原发性乳腺T-LBL／ALL患者的临床病理资料并复习相关文献。结果　患者为17岁女性,双侧乳房多发肿物伴左腋窝淋巴结肿大2个月,明确诊断为原发性乳腺T-LBL／ALL,经积极支持治疗同时给予改良BMF-90方案化疗达完全缓解,随访12个月仍然处于完全缓解状态。结论　原发性乳腺T-LBL／ALL是一种高度侵袭性疾病,临床罕见,预后差,选择T细胞急性淋巴细胞白血病（T-ALL）治疗方案有效。     

原发性乳腺淋巴瘤15例临床分析

目的：探讨原发性乳腺淋巴瘤（ PBL）临床病理特点、诊断、预后及治疗方法。方法：收集并分析延安大学附属医院2003年12月-2013年12月15例PBL患者的临床资料和治疗随访情况。结果：15例患者全部为女性,发病年龄25-68岁,中位年龄47.8岁。据2001年WHO淋巴瘤病理分类标准,14例为B细胞来源,1例为T细胞来源。具体分类为12例弥漫大B 细胞淋巴瘤（ DLBCL）,1例黏膜相关淋巴瘤（ MALT）,1例滤泡性淋巴瘤（ FL）,1例T淋巴母细胞淋巴瘤（ T-LBL）。10例行乳腺局部肿块切除,3例行乳腺改良根治术,1例行单侧乳腺切除。14例患者术后接受了全身化疗,1例患者因经济原因放弃化疗。所有患者随访时间为6-112月,中位随访时间24个月。3、5年生存率60%、46.7%。结论：PBL的预后与分期、淋巴瘤预后指数（ IPI）、病理组织学、治疗方式有关。PBL多为弥漫大B细胞型,预后差。PBL采用局部切除术联合放、化疗的综合治疗。对于中高度恶性的PBL,首次治疗时接受CNS预防是必要的。     

原发性乳腺恶性淋巴瘤六例临床分析

目的 分析原发性乳腺恶性淋巴瘤的临床特点,探讨其诊断、分期和治疗方法。方法回顾分析我院自1995～2002年收治的6例原发性乳腺恶性淋巴瘤和1980～2002年国内主要文献报道的279例原发性乳腺恶性淋巴瘤的临床特征、诊断情况和治疗方法,进行对比分析。结果 285例病例均为非霍奇金淋巴瘤(NHL),免疫学检查证实有282例为B细胞源性(98.9％);女性268例,占94.0％;病灶位于右侧163例,占57.2％;Ⅰ期和Ⅱ期的原发性乳腺恶性淋巴瘤占89.8％。经手术、化疗、放疗等综合治疗后,生存期2～206个月,中位生存期最短23个月,最长56个月。结论 原发性乳腺恶性淋巴瘤绝大部分为B细胞源性非霍奇金淋巴瘤(NHL),Ⅰ期、Ⅱ期多见。对于原发性乳腺恶性淋巴瘤,诊断是关键,确诊后经手术、化疗、放疗等综合治疗,可以获得较长的生存期,疗效十分满意。     

T淋巴母细胞淋巴瘤的治疗方案选择

目的：探讨T淋巴细胞淋巴瘤(T-LBL)治疗的更有效化疗方案。方法：回顾性分析近4a入院的7例T-LBL,比较用不同类型化疗方案治疗前后骨髓原始细胞比例和临床情况的变化。结果：7例用NHL类型方案化疗瘤块缩小或消失。但骨髓原始细胞未达缓解,有的甚至增多;其中4例采用ALL类型方案化疗者获骨髓完全或部分缓解。结论：T-LBL选择NHL和ALL类型化疗方案均有效,瘤块缩小或消失;但ALL类型方案疗效更好,尤其对累及骨髓者应选择ALL类型化疗方案。     

原发于女性乳腺非何杰金淋巴瘤IE期病例疗效分析

作者分析了２１例原发于女性乳腺非何杰金淋巴瘤的疗效。９例接受手术加局部放疗，１２例接受手术加联合化疗。全组中位生存期３１个月。手术加放疗组的中位生存期３９＾＋个月，两组比较有显著性差异（Ｐ＜０．０１）。结果表明，术后局部放疗并不能使乳腺非何杰金淋巴瘤患者获益，而联合化疗对延长乳腺非何杰金淋巴瘤患者的生存期至关重要。     

21例原发性乳腺非霍奇金淋巴瘤临床分析

目的:分析原发性乳腺非霍奇金淋巴瘤(PBNHL)的临床特征、治疗与预后,增加对该罕见病的认识.方法:回顾性分析1985年1月～2003年12月本院21例PBNHL的临床特点、治疗经过和随访结果.结果:21例PBNHL,女20例,男1例,中位年龄36岁(21～84岁).90%Ann Arbor分期为Ⅰ～Ⅱ期,所有患者预后分级均为低危或低中危.病理类型B细胞性87%,外周T细胞性13%.弥漫大B细胞性占53%.8例接受乳腺癌根治术或改良根治术,13例为肿块切除或活检;20例患者接受CHOP方案化疗;4例于化疗后局部放疗.患者双侧乳腺同时或先后发病占33%(7/21),CNS受累比例为14%.随访时间3～116月,中位17个月.中位总生存期和无事件生存期为26个月和12个月,5年总生存率和无事件生存率为39%和19%.结论:PBNHL病理以弥漫大B细胞性最常见,低度恶性NHL少见.CNS侵犯或复发是PBNHL的重要特征,对侧乳腺复发是接受患侧乳腺癌根治术或放疗的患者治疗失败的首要原因.中高度恶性PBNHL治疗可采用乳腺局部切除加全身化疗和乳腺放疗,宜接受中枢预防治疗,应密切监测对侧乳腺病变,及时治疗.     

原发性肺淋巴瘤11例临床特点分析

目的探讨原发性肺淋巴瘤的发病情况、临床特点、影像学特点、治疗及预后情况。方法回顾性分析了11例原发性肺淋巴瘤的临床资料及随访情况。结果11例患者中,霍奇金淋巴瘤2例,非霍奇金淋巴瘤9例（B细胞性4例,T细胞性4例,未分型1例）。临床症状多表现为咳嗽,咳痰,胸背部疼痛。影像学多表现为肺部肿块,边缘不规则,增强可强化,部分伴有肺不张。9例非霍奇金淋巴瘤患者中6例化疗的同时接受了放疗,3例仅接受了化疗,2例霍奇金淋巴瘤只接受了化疗。除了1名患者因各种因素造成脱落随访外,剩余10例随访患者的总生存期为15～102个月（中位生存期为84．5个月）。结论肺内淋巴瘤患者临床表、影像学检查无特异性．易误诊．治疗以化疗为主．辅以放疗．     

苯达莫司汀联合利妥昔单抗治疗胃外复发黏膜相关淋巴组织淋巴瘤

目的 探讨利妥昔单抗联合苯达莫司汀(R-Benda)治疗胃外复发黏膜相关淋巴组织淋巴瘤(MALT淋巴瘤)的疗效.方法 回顾性分析接受R-Benda方案化疗的10例诊断为复发胃外的MALT淋巴瘤患者临床资料.9例患者分别在化疗第2天和第3天接受90 mg/m2苯达莫司汀,1例患者因体能原因接受70 mg/m2苯达莫司汀;所有患者均在第1天接受375 mg/m2利妥昔单抗治疗;21 d为1个疗程.结果 8例患者接受了6个疗程化疗,1例患者因个人原因仅接受5个疗程化疗,另外1例老年患者在接受3个疗程化疗后疾病进展.8例患者达到了完全缓解,1例患者达到了部分缓解,另外1例老年患者在化疗4个疗程后因疾病进展而死亡.患者主要存在血液学不良反应事件,但程度较轻.中位随访24个月(5～43个月),9例患者生存,其中8例处于疾病缓解期.结论 R-Benda方案治疗胃外复发MALT淋巴瘤疗效好,而且不良反应轻.     

原发于乳腺恶性淋巴瘤1例报告

原发于乳腺的恶性淋巴瘤罕见 ,现报道 1例如下。患者女性 ,41岁 ,于 1 994年 4月无意中发现右乳和右腋窝包块 ,在昆明医学院第二附属医院诊断为乳腺癌 ,而行“右乳癌根治术”。术后病检 :乳腺及腋窝包块均为瘤细胞 ,大小一致 ,核分裂像多见。免疫组化示 :非霍奇金淋巴瘤 ( B系无裂细胞型 )。术后3个月到本院治疗 ,给予 COPP及 BCOPP方案交替化疗 7个周期及 CHOP方案化疗 1周期 ,后期因出现皮肤、双肺广泛受侵并伴右胸腔大量积液、心包积液。后因治疗无效而死亡 ,生存期 1 5个月。讨　论　原发于乳腺的淋巴瘤罕见 ,据 1 967年Latts统计…     

乳腺原发性淋巴瘤的临床病理及预后

目的 对乳腺原发性淋巴瘤的临床病理诊断、免疫表型进行分析.方法 对1998年1月至2007年5月诊治的乳腺原发性淋巴瘤8例,回顾性分析其临床表现、病理特征及免疫组织化学标记分型.结果 8例乳腺原发性淋巴瘤中,弥漫性大B细胞淋巴瘤5例,黏膜相关淋巴组织淋巴瘤2例,淋巴浆细胞性淋巴瘤1例.免疫组织化学标记,LCA、CD20、CD79a肿瘤细胞膜阳性;CD45RO、CD3、CD23、CD10、CD5肿瘤细胞阴性.临床分期,5例为ⅠE期,3例为ⅡE期.采用CHOP方案化疗,随访2～110个月,其中1例随访108个月后,另一侧乳腺肿瘤复发.结论乳腺原发性淋巴瘤主要为B细胞淋巴瘤,以弥漫性大B细胞淋巴瘤和黏膜相关淋巴组织淋巴瘤较多见,而淋巴浆细胞性淋巴瘤少见.对于临床ⅠE期和ⅡE期的早期患者,采用CHOP方案化疗有较好的治疗效果.     

74例淋巴母细胞淋巴瘤的临床特点及预后分析

目的 分析淋巴母细胞淋巴瘤（lymphoblastic lymphoma, LBL）的临床特点,比较B淋巴母细胞淋巴瘤（B lymphoblasts lymphoma, B-LBL）与T淋巴母细胞淋巴瘤（T lymphoblasts lymphoma, T-LBL）的临床及预后特点及不同化疗方案对LBL的预后影响。方法 选择2007—2014年天津医科大学肿瘤医院收治的74例经免疫组织化学确诊为LBL的患者。采用描述统计方法分析LBL的疾病谱特征。结果 74例LBL患者的中位年龄为19.5岁,其中45例为男性,60例为晚期起病（Ann-Arbor分期Ⅲ~Ⅳ期）,42例存在B症状,32例发生骨髓受累。治疗总有效率为70.2%,完全缓解率为48.6%,3年和5年总生存率（overall survival, OS）分别为38.0%和26.6%,3年和5年无进展生存率（progression-free survival, PFS）分别为34.8%和23.2%。其中B-LBL患者17例,T-LBL患者57例。B-LBL较T-LBL更倾向发生于儿童,起病时多伴有贫血,二者生存意义比较差异无统计学意义。单因素分析显示年龄是否小于18岁、有无贫血、β2微球蛋白水平、诱导治疗方案、近期疗效为预后相关因素。结论 淋巴母细胞淋巴瘤是一种高度侵袭性的恶性非霍奇金淋巴瘤,生存期短,多发生于青少年,起病时多为晚期,易发生骨髓转移。采用ALL类化疗方案的患者预后可能优于CHOP样方案。     

Primary therapy for adults with T-cell lymphoblastic lymphoma with hematopoietic stem-cell transplantation results in favorable outcomes.

BACKGROUND: Controversy exists regarding the role of high-dose therapy followed by stem-cell transplant (SCT) in the treatment of T-cell lymphoblastic lymphoma (T-LBL). We conducted an intention-to-treat analysis of the strategy of SCT as definitive treatment of T-LBL. PATIENTS AND METHODS: From July 1987 to March 2005, 34 adults with T-LBL were diagnosed and treated in British Columbia. Treatment, before planned SCT, consisted of a non-Hodgkin's lymphoma (NHL)/acute lymphoblastic leukemia hybrid chemotherapy protocol (28 patients) or a standard NHL chemotherapy regimen (six patients). RESULTS: Median follow-up of the 23 surviving patients is 51 months (range 13-142 months). Twenty-nine proceeded to SCT (four allogeneic, 25 autologous). For all 34 patients, 4-year overall survival (OS) and event-free survival (EFS) are 72% and 68%, respectively. For patients proceeding to SCT, the 4-year OS and EFS are 79% and 73%, respectively. All patients who received allografts are alive without disease at 38-141 months since diagnosis. For patients who received autografts, the 4-year EFS is 69%. Bone marrow involvement was a significant prognostic factor predicting for a worse survival (P = 0.02). CONCLUSION: A treatment strategy for adults with chemosensitive T-LBL that includes planned consolidation with SCT in first response produces favorable long-term outcome.     

Primary non-Hodgkin's lymphoma of the breast: retrospective analysis of prognosis and patterns of failure in two Australian centers

The breast is an uncommon site of presentation for primary non-Hodgkin's lymphoma, with prognosis and patterns of relapse still not clearly defined. A retrospective analysis of 21 patients presenting to 2 Australian centers during a 20-year period is presented. All patients were women and had a median age of 62 years. Fifteen patients (71%) had localized disease (12 unilateral and 3 bilateral), and 6 (29%) had regional lymph-node involvement. Histology was predominantly intermediate grade, with diffuse large B-cell lymphoma (DLBL) in 16 cases (76%). The most common treatment program was partial mastectomy followed by chemotherapy and radiation therapy (n = 12). Complete response (CR) to treatment was exhibited in 19 patients (90%), 11 of whom subsequently experienced relapse. Including the 2 patients who failed to exhibit an initial CR, the median time to disease progression was 23.4 months (range, 0-143 months), with a 5-year disease-free survival rate of 38% (+/- 12%). The actuarial median survival of all patients was 3.8 years, with bilateral breast involvement at presentation the only significant prognostic factor. The contralateral breast was the site of initial relapse in 3 patients (17%), all of whom subsequently died of disease. The actuarial rate of central nervous system (CNS) recurrence at 8 years was 39% (+/- 14%), occurring only in patients with diffuse large-cell histology. Our analysis suggests that DLBL presenting in the breast has a poor prognosis and characteristic patterns of failure. Targeted strategies such as CNS prophylaxis and contralateral breast irradiation might therefore improve prognosis and should be prospectively studied.     

The role of radiation therapy in the treatment of pediatric non-Hodgkin's lymphomas.

Between 1971 and 1976, 64 patients less than 18 years of age with non-Hodgkin's lymphoma were treated at Boston's Children's Hospital Medical Center-Joint Center for Radiation Therapy. A multimodality approach was used, consisting of radiation therapy (3500--4500 rad), surgery, and chemotherapy. Since 1973, all patients have received a regimen initially comprising Adriamycin, Prednisone, 6-Mercaptopurine, Vincristine, and L-Asparaginase. Methotrexate was substituted for Adriamycin following a cumulative total dose of 450 mg/m2. The 5-year actuarial survival for all patients was 61% while relapse-free survival was 54%. The actuarial and relapse-free survival for patients presenting with localized disease was 75% and 72%, respectively. Median follow-up was 40 months and all relapses occurred within 24 months of initial therapy. A multidisicplinary approach, such as the current regimen, offers a good prognosis for this disease.     

Treatment of secondary central nervous system lymphoma with intrathecal rituximab,high‐dose methotrexate,and R‐DHAP followed by autologous stem cell transplantation: results of the HOVON 80 phase 2 study

The prognosis of central nervous system (CNS) relapse of systemic non‐Hodgkin lymphoma is poor with 1‐year survival historically at 0% to 20%. Aiming to improve these results, we performed a multicenter phase 2 study in patients with a CNS relapse, with or without concurrent systemic relapse. Treatment consisted of 2 cycles of R‐DHAP alternating with high‐dose methotrexate (MTX) and was combined with intrathecal rituximab. Responding patients received a third R‐DHAP‐MTX cycle followed by busulfan and cyclophosphamide myeloablative therapy and autologous stem cell transplantation. In patients with persistent cerebrospinal fluid lymphoma after cycle 1, the intrathecal rituximab was replaced by intrathecal triple therapy, with MTX, cytarabine, and dexamethasone. Thirty‐six patients were included. Eighteen had evidence of cerebrospinal fluid lymphoma, 24 had brain parenchymal disease, and 20 (56%) had concurrent systemic disease. The overall response rate after 2 R‐DHAP‐MTX was 53% (19/36), with 22% (8/36) complete remission. Fifteen patients (42%) underwent a transplant. One‐year progression‐free survival was 19% (95% confidence interval, 9‐34): 25% in patients without and 15% in patients with systemic disease. One‐year overall survival was 25% (95% confidence interval, 12‐40). This treatment regimen did not result in a major improvement of outcome of secondary CNS lymphoma, especially when concurrent systemic disease was present. Registered in the Dutch trial register www.trialregister.nl , NTR1757; EudraCT number 2006‐002141‐37.     

单纯CHOP样方案与CHOP样方案联合造血干细胞移植巩固治疗淋巴母细胞淋巴瘤的疗效分析

目的 探讨单纯CHOP样方案与CHOP样方案+高剂量治疗联合造血干细胞移植(HDT-HSCT)一线巩固治疗淋巴母细胞淋巴瘤(LBL)的疗效.方法 63例有完整治疗及随访记录的LBL患者,初治均采用标准CHOP样方案,42例获得完全缓解(CR)或不确定CR(CRu).其中26例接受HDT-HSCT巩固治疗,16例单纯进行6～8个周期CHOP样方案治疗.结果 63例患者中,初治总缓解率为82.5%.中位随访24个月时,5年生存率为31.2%,5年无病生存率为29.3%.接受HDT-HSCT巩固治疗的26例患者,5年生存率为59.8%;单纯CHOP样方案治疗的16例患者,5年生存率为14.6%,差异有统计学意义(P=0.004).单因素预后分析结果显示,年龄、骨髓侵犯、初治缓解情况与预后有关(均P<0.05).18例骨髓受侵的患者中,3例接受异基因造血干细胞移植(allo-HSCT)的患者在随访22、32和37个月时仍生存,而4例接受自体造血干细胞移植(auto-HSCT)的患者,均在14个月内死亡.结论 单纯应用CHOP样方案治疗LBL疗效欠佳.HDT-HSCT作为一线巩固治疗有可能提高LBL患者的总生存率和无病生存率.骨髓受侵的LBL患者,allo-HSCT的效果优于auto-HSCT.     

Use of trastuzumab beyond disease progression: observations from a retrospective review of case histories

HER2 overexpression is associated with poor breast cancer prognosis and is the target for the humanized monoclonal antibody trastuzumab. This novel agent, when administered until disease progression in combination with chemotherapy, extends the survival of women with HER2-positive metastatic breast cancer (MBC). However, the optimal duration of trastuzumab therapy remains to be confirmed. We conducted a retrospective case review study of women with HER2-positive MBC who continued to receive trastuzumab beyond disease progression. Objectives were to assess whether treatment beyond disease progression shows any evidence of efficacy and to evaluate the feasibility of this approach. One hundred five patients (median age, 47 years; range, 24-77 years) were identified in 13 centers. Women had received /=1 more trastuzumab regimen. Trastuzumab treatment beyond progression appears to be of value, producing responses and clinical benefit, and is well tolerated without significant cardiac toxicity. The feasibility of this approach warrants examination in prospective trials.     

PTEN、MLL基因在T淋巴母细胞淋巴瘤／白血病的表达及其意义

 目的　分析肿瘤抑制基因PTEN、混合系白血病（MLL）基因等在T淋巴母细胞淋巴瘤／白血病（T-LBL／ALL）的表达及意义。方法　选用76例T-LBL／ALL患者淋巴结存档蜡块,应用免疫组织化学EnVision法进行PTEN标记,用20例反应性增生淋巴结标本作正常对照。并用荧光原位杂交（FISH）技术检测MLL基因所在11q23染色体的断裂和扩增情况。结果　76例T-LBL／ALL中,PTEN的表达率为64.47 %（49／76）,低于淋巴结反应性增生的100 %（20／20）（χ2＝19.220,P＜0.05）。PTEN表达与临床分期、Ki-67、乳酸脱氢酶（LDH）呈负相关（P＜0.05）。76例T-LBL／ALL中,MLL基因发生11q23染色体断裂13例（17.11 %）,扩增18例（23.68 %）。MLL基因断裂组总体生存率（25.0 %）低于非断裂组（43.6 %）（χ2＝11.357,P＜0.05）。MLL基因扩增组总体生存率（17.1 %）低于非扩增组（42.7 %）（χ2＝4.533,P＜0.05）。结论　抑癌基因PTEN表达降低在T-LBL／ALL的发生发展中可能具有重要作用。MLL基因发生染色体11q23断裂和扩增有助于对T-LBL／ALL预后的判断,发生MLL基因断裂或扩增的T-LBL／ALL预后较差,提示MLL基因断裂或扩增可能为T-LBL／ALL的一种分子亚型。     

凋亡调控基因Mcl-1在T细胞性淋巴瘤中的表达及临床意义

背景与目的:T细胞性非霍奇金淋巴瘤(T-cell non-Hodgkin's lymphoma,T-NHL)预后不良,有体外研究表明淋巴瘤细胞株的髓细胞白血病1(myeloid cellleukemia-1,Mcl-1)基因过表达能够拮抗放射和药物诱导的细胞凋亡.本研究探讨Mcl-1在各类型T-NHL中的表达及其与治疗反应和预后的关系.方法:收集临床资料完整的各类型T-NHL病例72例,回顾性分析其临床特点、治疗经过和随访结果,免疫组化染色方法检测Mcl-1表达,并进行相关性分析.结果:Mcl-1在T淋巴母细胞性NHL(precursor T lymphoblastic lymphoma,T-LBL)、间变T细胞性NHL(anaplastic large T-cell lymphoma,ALCL)和其他外周T细胞性NHL(peripheral T-cell lymphoma,PTL)中的弱阳性率分别为44.4%、0%和18.9%,阳性率分别为0%、100%和49.1%(P=0.000).Mcl-1在T-LBL中呈胞浆均匀一致的浅染色,在ALCL中呈核周块状棕黄色强染色.Mcl-1阳性与弱阳性/阴性PTL患者的中位总生存期(overall survival,OS)分别＞32个月和15个月(P=0.007),对化疗有效率分别为75%和53%(P=0.18),多因素分析结果显示国际预后指数(international prognostic index,IPI)和Mcl-1阳性为PTL的独立预后因素;Mcl-1阴性与弱阳性T-LBL患者的中位OS分别为21个月和7个月(P=0.58).结论:不同病理亚型的T-NHL的Mcl-1表达差异具有显著性.Mcl-1在ALCL呈特征性高表达.PTL中Mcl-1阳性患者预后优于阴性/弱阳性患者.