原发性乳腺淋巴瘤27例临床分析并文献复习

背景与目的:原发性乳腺淋巴瘤(primary breast lymphoma,PBL)发病率低,预后较差.本研究旨在分析该病例的临床和病理特征,从而探讨PBL的合理治疗模式.方法:收集并回顾性分析1976年到2005年间在中山大学肿瘤防治中心诊断为PBL,并接受治疗的27例患者的临床资料和治疗情况.结果:27例中有26例女性和1例男性患者;年龄12～84岁;90%的患者为Ⅰ E期或ⅡE期.按照WHO 2001淋巴瘤病理分类系统,有22例B细胞性淋巴瘤(17例弥漫大B细胞性淋巴瘤,2例黏膜相关性淋巴瘤,1例边缘区淋巴瘤,2例未能分类),3例外周T细胞性淋巴瘤,2例患者的病理类型未能分类.初始治疗时有20例患者接受了综合治疗,其中8例患者为根治术加术后化疗,12例患者为肿物切除术后加全身化疗,两组的5年生存率分别为23.0%和58.0%(P=0.006);其余有5例患者仅接受全身化疗,2例患者仅接受肿物切除手术.24例患者在初始治疗后取得完全缓解,1例患者部分缓解,2例患者疾病进展.随访时间1个月～10年,中位随访时间38个月.全组患者的5年总生存率和无病生存率分别是47.0%和23.0%;其中20例中高度恶性淋巴瘤患者(17例弥漫大B细胞性淋巴瘤和3例外周T细胞性淋巴瘤)的5年总生存率和无病生存率分别是48.0%和27.0%;随访中有16例复发,部位见于同侧乳腺6例、对侧乳腺4例、中枢神经系统(central nervous system,CNS)3例、骨髓1例和淋巴结侵犯2例.结论:PBL的病理类型以中高度恶性淋巴瘤为主;根治性手术在其治疗中作用有限,肿物切除术加术后化疗和放疗的效果较好.PBL患者易发生CNS复发,在随访中应定期进行颅脑CT或MR检查.     

Human immunodeficiency virus (HIV)-negative plasmablastic lymphoma: a single institutional experience and literature review

Plasmablastic lymphoma (PBL) is a rare aggressive B-cell lymphoproliferative disorder. HIV-negative PBL has not been extensively reported. Nine HIV-negative PBL patients evaluated at Moffitt Cancer Center were studied. Eight patients had extranodal diseases. All patients were treated with CHOP or hyper-CVAD. Responses were observed in 8 cases (7 complete, 1 partial responses). Four patients underwent consolidation with autologous hematopoietic stem cell transplant (HSCT) in first complete remission (CR1). At median follow-up of 23.9 months, 7 patients were alive and 5 were disease-free. Aggressive induction chemotherapy and consolidation with autologous HSCT in CR1 might be considered for patients with HIV-negative PBL.     

37例原发骨恶性淋巴瘤的临床特点及预后因素分析

探讨原发骨恶性淋巴瘤（primary bone lymphoma,PBL）的临床特点及其与预后的相关性。方法：回顾性分析1995年6月至2009年5月本院收治的37例PTL患者的临床资料,以Kaplan-Meier法绘制生存曲线,用Log-rank检验进行单因素分析,多因素分析采用Cox回归模型以评估独立的预后因素。结果：37例患者的中位发病年龄为61（18～85）岁,首发症状主要表现为骨痛,局部软组织肿胀、肿块形成和病理性骨折。78%患者的病理类型为弥漫大B细胞淋巴瘤。经化疗和/或放疗,18例完全缓解（complete response,CR）,13例部分缓解（partial response,PR）,3例稳定（stable disease,SD）,2例进展（progressive disease,PD）。中位随访时间32（7～171）个月,5年和10年总生存率分别为59.5%和43.2%。患者接受4周期以上化疗,B细胞淋巴瘤加用利妥昔单抗者疗效较好。多因素分析显示：Ann Arbor分期、B症状、年龄和结外受侵数是PBL的独立预后因素。结论：PBL应采取综合治疗,同时给予蒽环类药物为主的全身化疗,B细胞淋巴瘤首选利妥昔单抗联合化疗,给予帕米膦酸盐治疗骨病变。Ann Arbor分期、B症状、年龄和结外受侵数为PBL预后的独立影响因素。     

Early locoregional high-dose radiotherapy is associated with long-term disease control in localized primary angiocentric lymphoma of the nose and nasopharynx.

Nasal NK/T cell is a rare form of usually localized non-Hodgkin's lymphoma (NHL) which generally carries a poor prognosis when treated with conventional NHL chemotherapy protocols. We reviewed 20 consecutive localized stage I/II nasal NK/T cell lymphomas treated at our institution over a 29 year period. Median age was 44 (range 23-71). Front-line therapy was generally radiotherapy alone (35-70 Gy) before 1980 and combination chemotherapy after 1980. Six patients were treated with first-line radiotherapy and they achieved complete remission (CR). Two subsequently received combination chemotherapy. Five of those patients remained in complete remission, after 97+ to 277+ months. Twelve patients were treated with first-line chemotherapy including CHOP or CHOP-like regimen in seven cases, and COP in five cases. Only three of them achieved CR, five had partial response and four had progressive disease. Five of the seven patients treated with CHOP did not achieve complete remission. The nine patients who failed to achieve CR with chemotherapy subsequently received salvage radiotherapy but only two of them obtained CR. Finally, two patients were treated with alternated chemotherapy and radiotherapy and achieved CR, which persisted after 14+ and 26+ months. Median survival was not reached in patients who received front-line radiotherapy, and was 35 months in patients who received front-line chemotherapy. These findings confirm that chemotherapy gives a low complete remission rate in localized nasal NK/T cell lymphoma. By contrast, first-line radiotherapy seems to give favorable results, whereas its results are poorer when administered after resistance to chemotherapy. Whether the use of chemotherapy after radiotherapy, or alternated chemotherapy-radiotherapy regimens give better clinical results than radiotherapy alone will have to be evaluated prospectively in this type of NHL.     

Diffuse large B-cell lymphoma involving the breast. A report of four cases

Non-Hodgkin lymphoma of the breast is an uncommon form of lymphoma occurring either primary disease (PBL) or part of systemic involvement. We report the clinical outcome of 4 consecutive cases with CD20+ diffuse large B-cell lymphoma (DLBCL) of the breast, in the attempt to further clarify the management of this disease. The median age was 53 years (39-61), stages were IIE (n=2), IIIE (n=1), and IV (n=1); IPI scores were 0 (n=2), 2 (n=2). Two cases were PBL, and 2 were secondary involvement of the breast. Two stage IIE patients received MACOP-B, radiation therapy was given to one of them and both achieved CR. The stage IIIE patient treated with MACOP-B plus Rituximab was in PR at the beginning of the Rituximab and achieved CR at the end of the treatment. The 61-year-old stage IV patient and bilateral involvement received P-VNBEC as first line treatment, achieving PR; she was then treated with 4 cycles of MACOP-B plus Rituximab obtaining CR. After a median follow-up of 40 months (31-50) all patients are alive and in CR. No CNS prophylaxis was given and no incidence of CNS relapse was observed. In our experience DLBCL of the breast shows chemosensitivity to MA-COP-B regimen but the intensification with Rituximab seems to be effective especially in the advanced stages. Further and comparative studies are required to confirm the validity of our results.     

Phase II study of rituximab in combination with chop chemotherapy in patients with previously untreated, aggressive non-Hodgkin's lymphoma.

PURPOSE: To determine the safety and efficacy of the combination of the chimeric anti-CD20 antibody Rituxan (rituximab, IDEC-C2B8; Genentech Inc, South San Francisco, CA) and cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy in patients with aggressive non-Hodgkin's lymphoma (NHL). PATIENTS AND METHODS: Thirty-three patients with previously untreated advanced aggressive B-cell NHL received six infusions of Rituxan (375 mg/m2 per dose) on day 1 of each cycle in combination with six doses of CHOP chemotherapy given on day 3 of each cycle. RESULTS: The ORR by investigator assessment confirmed by the sponsor was 94% (31 of 33 patients). Twenty patients experienced a complete response (CR) (61%), 11 patients had a partial response (PR) (33%), and two patients were classified as having progressive disease. In the 18 patients with an International Prognostic Index (IPI) score > or = 2, the combination of Rituxan plus CHOP achieved an ORR of 89% and CR of 56%. The median duration of response and time to progression had not been reached after a median observation time of 26 months. Twenty-nine of 31 responding patients remained in remission during this follow-up period, including 15 of 16 patients with an IPI score > or = 2. The most frequent adverse events attributed to Rituxan were fever and chills, primarily during the first infusion. Rituxan did not seem to compromise the ability of patients to tolerate CHOP; all patients completed the entire six courses of the combination. The bcl-2 translocation of blood or bone marrow was positive at baseline in 13 patients; 11 patients had follow-up specimens obtained (eight CR, three PR), and all had a negative bcl-2 status after therapy. Only one patient has reconverted to bcl-2 positivity, and all patients remain in clinical remission. CONCLUSION: This is the first report to demonstrate the safety and efficacy of the Rituxan chimeric anti-CD20 antibody in combination with standard-dose CHOP in the treatment of aggressive B-cell lymphoma. The clinical responses are at least comparable to those achieved with CHOP alone with no significant added toxicity. The presence or absence of the bcl-2 translocation did not affect the ability of patients to achieve a CR with this regimen. The ability to achieve sustained remissions in patients with an IPI score > or = 2 warrants further investigation with a randomized study.     

抗CD20单克隆抗体联合自体外周血干细胞移植治疗非霍奇金淋巴瘤的临床研究

 目的　探讨抗CD20单克隆抗体（利妥昔单抗,商品名：美罗华）联合自体外周血干细胞移植（APBSCT）治疗B细胞非霍奇金淋巴瘤（NHL）的疗效。方法　21例CD20阳性的NHL患者,经过前期治疗,5例达完全缓解（CR）,难治性病例为16例,包括11例部分缓解（PR）和5例疾病进展（PD）。在自体造血干细胞动员的第1、8天及预处理的-1、＋7天每天应用利妥昔单抗375 mg／m2。结果　移植前疾病达到CR的5例患者,无一例复发;移植前处于PR的11例患者,仅1例在移植后6个月疾病复发,其余均无病生存;移植前处于PD的5例患者,2例无病生存。21例患者中位随访24（1～68）个月,复发、死亡4例（19 %）,其余17例均无病生存,2年无病生存（EFS）和总生存（OS）率均为81.0 %。未观察到利妥昔单抗对采集所得干细胞的质量和数量以及移植后造血恢复有不良影响。结论　APBSCT联合利妥昔单抗做体内净化治疗B细胞NHL疗效与移植前状态有关,作为巩固治疗,能使移植前达CR的患者获得长期生存,提高治愈率;作为强化治疗,可提高缓解率,延长PR患者的EFS及OS。利妥昔单抗的加入不影响造血干细胞采集和移植后造血重建。     

青少年或儿童原发系统性间变大细胞淋巴瘤CHOP为主化疗±放疗的远期疗效

目的 分析青少年儿童原发系统性间变大细胞淋巴瘤(ALCL)患者接受CHOP方案化疗 ±受累野放疗的疗效。方法 回顾分析本院1998—2010年收治的 28例青少年、儿童ALCL患者资料。Ⅰ、Ⅱ期 12例中单纯化疗 2例、综合治疗 10例,Ⅲ、Ⅳ期 16例中单纯化疗 14例、综合治疗 2例。CHOP方案 15例、CHOP联合其他高强度化疗 13例。化疗周期数 3~17个(中位数6个)。放疗多为受累野照射,剂量 39.6~50.0 Gy (中位数45 Gy)。结果 全组患者首程疗后达CR者 25例(89%),3例病变进展。中位随访时间45.3个月。全组 5年无事件生存率为80%,5年OS为93%。疗终达CR者 5年OS为100%,而未达CR者无 5年OS (P=0.000)。≥2个结外器官受侵者 5年无事件生存率为38%,而<2个结外器官受侵者的为85%(P=0.010)。结论 青少年、儿童原发系统性ALCL按成人方案治疗效果满意,但还需要长期随访。     

Autologous bone marrow transplantation in B-cell non-Hodgkin's lymphoma: very low treatment-related mortality in 100 patients in sensitive relapse

One hundred patients with B-cell non-Hodgkin's lymphoma (NHL) in sensitive relapse or incomplete first remission underwent high-dose chemoradiotherapy and anti-B-cell monoclonal antibody (MAb)-treated autologous bone marrow transplantation (ABMT). These patients demonstrated good performance status with a Karnofsky score of 80% or greater. The majority of these patients had one or more adverse prognostic features including a failure to achieve a complete remission (CR) with conventional combination chemotherapy (37 patients), bone marrow infiltration (69 patients), a history of extranodal disease other than bone marrow infiltration (42 patients), and histologic conversion (18 patients). At the time of ABMT, only 52 patients were in CR; however, all patients achieved a minimal disease state following conventional intensive therapy. Moreover, at the time of marrow harvest, 37 of these patients had histologic evidence of lymphoma cells infiltrating the marrow. Following high-dose ablative therapy, two acute in-hospital treatment-related deaths were observed. Two late deaths were observed, not due to recurrent lymphoma. Of the remaining 96 patients, 61 are in unmaintained CR with a median follow-up of 13 months. Kaplan-Meier actuarial analysis predicts 50% probability of disease-free survival (DFS) at 37.8 months. This very low treatment-related mortality provides the rationale to apply high-dose therapy and ABMT as consolidative therapy for patients in first remission who are at high risk for relapse following conventional therapy.     

24例原发纵隔大B细胞淋巴瘤的临床分析并文献复习

背景与目的:原发纵隔大B细胞淋巴瘤(primary mediastinal large B-cell lymphoma,PMBCL)是弥漫大B细胞淋巴瘤的一种亚型,发病率较低。本研究旨在分析其临床特征,探讨合理的治疗模式及预后因素。方法:收集1995年5月至2005年9月于福建省肿瘤医院确诊并接受治疗的24例PMBCL患者的临床资料并行回顾性分析,同时结合文献加以讨论。结果:24例患者中男性16例,女性8例,年龄12～81岁,30岁以下13例。临床Ⅰ Ⅱ期20例,Ⅲ期1例,Ⅳ期3例。有纵隔巨块13例,伴上腔静脉综合征10例,邻近器官受侵14例,乳酸脱氢酶升高15例。初治时11例采用联合化放疗,10例采用单纯化疗,3例采用单纯放疗,完全缓解率41.7%,总有效率91.7%,中位生存期89个月,3年总生存率68.8%,其中初治时达完全缓解者至随访结束时均生存,国际预后指数(international prognostic index,IPI)在本组中未显示预后,多因素分析提示纵隔巨块与预后相关。结论:PMBCL在本组中男性多见,临床表现凶险,须尽快明确诊断。蒽环类为主的化疗联合放疗可取得较好疗效,初治时获得完全缓解尤为关键,伴有巨块者预后差。     

Anthracycline-based chemotherapy of primary non-Hodgkin's lymphoma of the testis: the hellenic cooperative oncology group experience

Testicular non-Hodgkin's lymphoma is an uncommon disease and its outcome following chemotherapy and/or radiotherapy has been variable. A retrospective analysis was performed on 26 patients with primary testicular lymphoma treated predominantly with anthracycline-based chemotherapy between 1984 and 1999. The patients' median age was 60 years (range 19-82 years) with 17 (65.4%) patients being older than 60 years. Four (15.4%) patients had constitutional B symptoms. There were 11 (42.3%) patients with high grade lymphoma, 12 (46.2%) with intermediate grade, 1 (3.8%) with low grade and 2 (7.7%) were not classified. According to the Ann-Anbor staging system, 18 patients (69.2%) had early (stage I/II) and 8 (30.8%) advanced (stage III/IV) disease. Chemotherapy was administered to 24 patients including 22 patients who received anthracycline-based chemotherapy. Two stage IEA patients were treated with orchidectomy and adjuvant radiotherapy to the regional lymph nodes without systemic chemotherapy. Chemotherapy alone resulted in a complete remission (CR) in 14 (58.3%) of 24 patients and partial remission in 1 (4.2%), amounting to an overall response rate (RR) of 62.5%. Of the 5 stage I patients who had chemotherapy on an adjuvant basis, 4 (80%) had CR/no evidence of disease. Of the 11 stage II patients, 8 (72.7%) achieved CR and 1 (9.1%) PR (overall RR of 81.8%). CR was obtained in 2 (25%) of 8 stage III/IV patients. Both patients remain disease free for 26 and 65 months. Excluding the 5 stage I patients, chemotherapy resulted in a CR in 10/19 (52.6%) patients and a PR in 1/19 (5.2%), inducing an overall RR of 57.8%. The mean duration of response was 75 months (range 8-145.5+ months). After a median follow-up of 87 months (range 0.13-145.5+ months) the median survival time was 31 months (range 0.13-145.5+ months) and the median time to progression (TTP) 17 months (range 0.13-145.5+ months). The median TTP was significantly higher in early disease compared to that of advanced disease (52 vs. 3 months, p = 0.02). Of the 3 patients who relapsed following disease-free status, CNS involvement occurred in 2 stage II patients and contralateral testis involvement in 1 stage IEA, respectively. The latter remained disease free for 2 years following orchidectomy alone. The other 2 patients who relapsed did not respond to salvage chemotherapy and died. There was no significant relationship between the values of LDH and beta(2)-microglobulin with the outcome except for ESR which was significantly related with the CR (p = 0.005) or RR (p = 0.005). In conclusion, patients with primary testicular lymphoma have a poor outcome, despite the treatment with anthracycline-containing regimens. Treatment with anthracycline-based chemotherapy is recommended in patients at early stages. In advanced disease, more intensive or investigational regimens should be considered. Because the relapse rate in the CNS and contralateral testis is quite high in most studies, prophylactic CNS treatment and radiotherapy to the other testis should be included in the management of testicular lymphoma.     

鼻型NK/T细胞淋巴瘤的临床和预后分析

目的 分析鼻型NK／T细胞淋巴瘤的临床特点、治疗方法和预后情况,并探讨其疗效的改进。方法 39例患者多数做了化放综合治疗,化疗主要为含葸环类的CHOP或类似方案,中位5个周期。放射治疗采用高能光子射线配合高能电子线,常规分割,中位根治剂量56Gy。对临床资料进行双变量相关分析和单因素生存分析。结果 39例患者中,存活患者21例,其中位随访时间22.5个月。首程治疗后总缓解率66．7％（21例完全缓解,3例部分缓解）,单纯化疗完全缓解率37．5％。全组肿瘤局部控制率59．5％,根治性放疗的局部失败率25．0％,放射治疗与局部控制（P=0．000）、疾病进展时间（TTP,P=0．002）呈正相关。治疗失败的结外部位还包括皮肤和肠道,15例患者病程急骤,中位生存5个月。生存分析显示,生存有利的预后因素包括放射治疗（P=0．001）、国际预后指数（IPI）低危（P=0．001）、首程治疗完全缓解（P=0．000）、病史〉2个月（P=0．024）和无皮肤浸润（P=0,034）。结论 此型淋巴瘤确诊时多为早期病变,放射治疗是重要的治疗手段。放化综合治疗对进展期患者的疗效尚待改进。部分患者病程急骤、预后不良,需要进一步的研究以确定新的预后监测指标和个体化治疗方案。     

Chemotherapy in patients who fail radiotherapy for diffuse aggressive non-Hodgkin's lymphoma

Twenty patients with diffuse aggressive non-Hodgkin's lymphoma were treated primarily with radiotherapy for localized disease and had subsequent disease progression. All 20 patients then received an adriamycin based, combination chemotherapy regimen, but only seven patients (35%) achieved a complete remission, and only four patients (20%) currently remain in remission. When 15 patients treated with the CHOP regimen were compared to 62 patients treated with CHOP during the same time period but who did not have preceding radiotherapy, patients with previous radiotherapy had a lower complete remission rate (i.e. 66% versus 33%) and a slightly lower 5-year survival rate (i.e. 41% versus 20%). Thus, unlike patients with Hodgkin's disease, patients with diffuse aggressive non-Hodgkin's lymphomas who fail radiotherapy for localized disease might not have an especially good outlook with subsequent chemotherapy.     

CHOP或CHOP样方案一线治疗晚期血管免疫母细胞型T细胞淋巴瘤效果观察

目的 分析CHOP或CHOP样方案一线治疗血管免疫母细胞型T细胞淋巴瘤(AITL)的效果.方法 回顾性分析2006年8月至2014年2月收治的29例应用CHOP或CHOP样方案一线治疗的晚期AITL患者的临床资料,总结其临床特征,分析疗效及其预后因素.结果 患者中位发病年龄为59岁,全部为Ⅲ～Ⅳ期,17例(58.6％)伴B症状,26例(89.7％)IPI评分≥2分,20例(69.0％)起病时乳酸脱氢酶升高,9例(31.0％)出现≥2个结外器官受侵.中位随访20个月,总有效率为69.0％(20/29),其中17.2％(5/29)的患者初治达完全缓解或未确定完全缓解,51.7％(15/29)达部分缓解,20.7％(6/29)出现疾病进展,10.3％(3/29)稳定.中位无进展生存(PFS)期为6个月.1、2年PFS率分别为39.0％、20.0％.1、2、5年总生存(OS)率分别为76.8％、53.4％和17.1％.治疗有效患者PFS长于无效患者(P＜ 0.001),有效与无效患者OS差异无统计学意义(P＞0.05).结论 CHOP或CHOP样方案一线治疗AITL的总体疗效不满意,有待进一步探索新的治疗方法.     

Chemotherapy with dexamethasone, high-dose cytarabine, and cisplatin for parenchymal brain lymphoma

Ten patients with parenchymal CNS lymphoma received combination chemotherapy with dexamethasone, high-dose cytarabine, and cisplatin (DHAP), and additional therapy according to clinical circumstances. Two of four patients with primary CNS lymphoma achieved and remain in complete remission (CR). Four of six patients with CNS lymphoma at relapse also achieved CR. DHAP is active against CNS lymphoma. Further trials with DHAP, particularly in conjunction with other treatment modalities, are warranted.     

Salvage therapy with ProMACE-MOPP followed by intensive chemoradiotherapy and autologous bone marrow transplantation for patients with non-Hodgkin's lymphoma who failed to respond to first-line CHOP.

PURPOSE: We used alternative chemotherapy immediately followed in early-response patients by high-dose chemoradiotherapy and autologous bone marrow transplantation (ABMT) to treat patients with non-Hodgkin's lymphoma (NHL) who had failed to respond to first-line chemotherapy. PATIENTS AND METHODS: Thirty-one patients with NHL of intermediate- or high-grade malignancy who had failed to respond to first-line cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy were treated. Seventeen patients had primary refractory disease and 14 had relapsed from first complete response (CR). The treatment consisted of prednisone, methotrexate, doxorubicin, cyclophosphamide, etoposide, mechlorethamine, vincristine, and procarbazine (ProMACE-MOPP) salvage chemotherapy, followed, in case of responsive disease (at least partial response [PR]), by high-dose cyclophosphamide and total-body irradiation (TBI) with ABMT. RESULTS: Twenty-eight of 31 (90%) patients achieved PR (23 patients) or CR (five patients) with ProMACE-MOPP, and three failed to respond. Seventeen of 28 (61%) patients who responded underwent the ABMT procedure, which resulted in CR in 14 patients (82%); three failed to respond. Eleven responsive patients were not transplanted because of residual bone marrow infiltration (five patients), patient refusal (four patients), and ProMACE-MOPP-related mortality (two patients). To date, nine patients are alive and in CR, seven with a median follow-up of 41 months (range, 17 to 84 months). Referring to the original CHOP treatment, five of 17 (29%) patients with primary refractory disease remain free of disease at a median of 36 months after ABMT, and four of 14 (29%) patients in first relapse remain free of disease at a median of 33 months after ABMT. One patient died of AMBT-related toxicity. CONCLUSION: ProMACE-MOPP salvage chemotherapy produces a high early-response rate in patients who fail to respond to first-line CHOP, and more than half of the responding patients can be scheduled to receive ABMT, resulting in disease-free survival (DFS) at 3 years in 50% of the transplanted patients and in 25% of the original number of patients intended to receive this treatment.     

Dexa-BEAM: an effective regimen for cytoreduction prior to high-dose chemotherapy with autologous stem cell support for patients with relapsed/refractory mantle-cell lymphoma

Mantle-cell lymphoma (MCL) is not a curable disease using conventional chemotherapy. Patients with MCL have the shortest median time to progression and the shortest median survival of all lymphoma subtypes after first-line treatment. In the present study we determined the efficacy of maximal cytoreductive therapy with up to four cycles of Dexa-BEAM (dexamethasone, carmustine [BCNU], etoposide, cytarabine, and melphalan) followed by high-dose chemotherapy (HDCT) and autologous hematopoietic stem cell support (ASCT) for patients with advanced relapsed or refractory MCL. Nine consecutive patients with relapsed or refractory MCL were included. Three patients had partial remission (PR), three patients progressive disease (PD) upon first line tretment, and three patients first or subsequent relapse. After 2 to four cycles of Dexa-BEAM eight patients achieved complete remission (CR), resulting in a response rate of 88%. Six of 8 patients responding to Dexa-BEAM received high-dose chemotherapy HDCT (BEAM) and autologous hematopoietic stem cell transplantation (ASCT). With a median follow up of 24 months six patients are alive. Five of those six patients are still in contiuous CR (range 13-54 months).     

Efficacy of chemotherapy as a first-line treatment in ocular adnexal extranodal marginal zone B-cell lymphoma.

BACKGROUND: Radiotherapy is commonly used as a first-line treatment for localized ocular adnexal extranodal marginal zone B-cell lymphoma (EMZBL), despite its ophthalmologic complications. This study was undertaken to analyze the efficacy of first-line chemotherapy in treating EMZBL. Chemotherapy was followed by radiotherapy only in recurrent cases. PATIENTS AND METHODS: Twenty-one patients with histologically confirmed EMZBL were treated with combination of cyclophosphamide, vincristine, and prednisolone (CVP). Radiotherapy was given to CVP failure cases. RESULTS: CVP alone resulted in overall response rate of 100% [complete remission (CR), 76.2%]. After a median follow-up of 58 months, 14 (66.7%) of 21 cases were disease free with CVP alone, while seven cases showed disease progression, including two extra-orbital and five local failures. Radiotherapy was delivered to five local failure cases, who subsequently achieved CR with late ophthalmologic complications. There were tolerable adverse events associated with CVP. CONCLUSIONS: Front-line CVP, in conjunction with radiotherapy in recurrent cases, is effective and well tolerated in patients with localized ocular adnexal EMZBL.     

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.