Primary bladder lymphoma: management and outcome of 12 patients with a review of the literature

Primary bladder non-Hodgkin's lymphoma (NHL) is rare. Optimal management remains controversial. Using the Scotland and Newcastle lymphoma group database, 12 patients with primary bladder lymphoma were identified between 1980 and 2001, the largest single group of patients available to date. Histology and immunocytochemistry was reviewed in 9 of the 12 cases. Six cases were low-grade extranodal marginal zone lymphoma, 4 diffuse large B-cell lymphoma, one an ALK 1 positive anaplastic large cell lymphoma (ALKoma) and one a low-grade lymphoma unspecified. Two patients (low-grade NHL) were treated with oral antibiotics (n = 1) or diathermy (n = 1) alone with complete resolution of disease. One patient with high-grade NHL gained complete remission without conventional therapy. Nine patients were treated with single or combined modality surgery, chemotherapy and/or radiotherapy. Overall survival was 75%, mean follow up of 4.8 (range 1 - 10) years. A review of 88 additional cases in the literature support the findings that primary bladder lymphoma is associated with a good prognosis. Patients with low-grade extranodal marginal zone lymphoma may respond well to simple therapies. Patients with diffuse large B-cell lymphoma respond well to first-line chemotherapy regimens. Ureteric obstruction and acute renal failure are serious complications. Repeat cystoscopy is mandatory for follow-up.     

PSC方案治疗复发难治结外NK/T细胞淋巴瘤鼻型的疗效观察

目的:探讨PSC方案治疗复发难治结外NK/T细胞淋巴瘤(ENKTCL)的疗效。方法:回顾性分析自2019年01月01日至2021年01月01日我科收治的ENKTCL采用信迪利单抗、西达苯胺联合培门冬酶(PSC)方案的治疗效果。结果:共收集到13例具有完整临床资料的复发难治ENKTCL患者,男女比例为5.5∶1,男性多见。中位发病年龄51岁(11～74岁)。复发ENKTCL 8例(61.54%),难治ENKTCL 5例(38.46%),13例R/R NKTCL中9例(69.23%)为结外累及。13例复发难治ENKTCL患者中,2周期CR为7例(53.85%),PR 3例(23.08%),ORR 10例(76.92%),其中1例患者CR后再次治疗2周期疾病进展家属放弃治疗死亡。随访至2021年1月31日中位OS为未达到,中位无进展生存(PFS)4个月。13例复发难治ENKTCL,采用PSC方案治疗后出现的主要不良反应有肝功能损伤、低蛋白血症、低纤维蛋白原血症、甲状腺功能减低及血液学毒性等。给予对症治疗后好转。结论:PSC方案治疗复发难治ENKTCL疗效确定,不良反应可耐受。     

结外NK/T细胞淋巴瘤，鼻型的诊断与治疗进展

结外NK/T细胞淋巴瘤,鼻型（extranodal natural killer/T-cell lymphoma ,nasal type,ENKL）是非霍奇金淋巴瘤（non-hodgkin lymphoma,NHL ）的一种少见亚型,其侵袭性强且预后较差。ENKL 主要发生于鼻腔,其次是皮肤、胃肠道等。该病以血管的侵犯和组织破坏为主要病理学表现。ENKL 与EBV 的感染密切相关,EBV 水平对其辅助诊断有重要的意义。NK/T细胞表面的特征性标志物和特异性遗传学改变也可以帮助诊断该病。目前对于ENKL 的治疗尚在讨论中,虽然对早期患者采用放疗± 化疗联合治疗,以及对中晚期患者采用以左旋门冬酰胺酶为基础的化疗和造血干细胞移植得到了一定疗效,但仍需进一步的研究探索以形成规范的治疗原则。     

Prognostic value of the Follicular Lymphoma International Prognostic Index score in marginal zone lymphoma

BACKGROUND:

In marginal zone lymphoma (MZL), clinical and follow‐up data on large cohorts of patients are difficult to obtain. The objective of this single‐center, retrospective analysis of a large cohort of 144 patients with MZL was to elucidate the role of prognostic markers, treatments, and outcomes in this disease.

METHODS:

In total, 144 patients were identified who were diagnosed with MZL at the authors' institution between 2003 and 2010. Data on clinical parameters, treatments, response, and survival were analyzed. In addition, the validity of the International Prognostic Index (IPI) and Follicular Lymphoma International Prognostic Index (FLIPI) prognostic scores were tested in patients with MZL.

RESULTS:

Among 144 patients with MZL, 96 patients (67%) had extralymph node (extranodal) MZL, 32 patients (22%) had lymph node (nodal) MZL, and 16 patients (11%) had splenic MZL. The 5‐year progression‐free survival rate was 82% in the nodal MZL group, 88% in the extranodal MZL group, and 74% in the splenic MZL group and did not different between the 3 groups (P = .60). The 5‐year overall survival rate was excellent in all 3 MZL groups (nodal MZL, 89%; extranodal MZL, 92%; splenic MZL, 82%; P = .46). In our cohort, the FLIPI score was a significant prognostic marker: The 5‐year progression‐free survival rate for patients who had FLIPI scores of 0 to 2 (low or intermediate risk) was excellent at 92%, whereas it was only 62% for patients who had FLIPI scores of 3 to 5 (poor risk; P = .003). Similarly, the 5‐year overall survival rate for patients who had FLIPI scores of 0 to 2 was 95%, whereas it was only 62% for patients who had FLIPI scores of 3 to 5 (P = .0009).

CONCLUSIONS:

The FLIPI score had strong prognostic value in patients with MZL. Patients who have low‐risk or intermediate‐risk FLIPI scores have an excellent prognosis, whereas patients with poor‐risk FLIPI scores are candidates for novel treatment approaches. Cancer 2013. © 2012 American Cancer Society.     

Long‐term safety and activity of cladribine in patients with extranodal B‐cell marginal zone lymphoma of the mucosa‐associated lymphoid tissue (MALT) lymphoma

The purine analogue 2‐chloro‐deoxyadenosine (2‐CDA, cladribine) +/? rituximab has been successfully tested in mucosa‐associated lymphoid tissue lymphoma (MALT lymphoma) patients. However, studies using cladribine in other indications have reported the potential for prolonged hematological side effects and secondary hematologic and non‐hematologic malignancies. To date, there have been no data on long‐term effects of cladribine in MALT lymphoma patients. We have analyzed a large number of 49 patients treated with cladribine at our institution 1997–2011. All patients were treated within clinical trials and had undergone a standardized follow‐up protocol minimizing a potential bias in the detection of late sequels and relapses. After a median follow‐up time of 61 months (interquartile range: 43–72) for 49 analyzed patients, 35 (71%) are alive, while 14 (29%) have died. In the entire collective, three cases (6%) of prolonged pancytopenia including manifest myelodysplastic syndrome in one patient (2%), three cases (6%) of secondary lymphoid malignancies, and five cases (10%) of non‐hematologic cancers were documented. In terms of outcome, 42/49 (86%) patients responded to cladribine‐containing treatment, and only 10/42 (24%) responding patients needed further treatment after a median time to progression of 14 months (interquartile range, 8–34). Currently, 25/35 (71%) patients being alive are in ongoing complete remission and 2/35 (6%) in ongoing stable disease, respectively. Eight patients (23%) are free of lymphoma after second‐line therapy, with the median overall survival not having been reached. Our data suggest that cladribine might be safely applied in patients with MALT lymphoma, also in terms of long‐term toxicities. These data also confirm the potential of cladribine to induce durable remissions. Copyright © 2015 John Wiley & Sons, Ltd.     

A phase 2 study of methotrexate,etoposide, dexamethasone,and pegaspargase chemotherapy for newly diagnosed,relapsed, or refractory extranodal natural killer/T‐cell lymphoma,nasal type: a multicenter trial in Northwest China

The nasal type of extranodal natural killer/T‐cell lymphoma is a rare aggressive lymphoma with poor prognosis. To discover a successful treatment, we investigated the efficacy and safety of chemotherapy with methotrexate, etoposide, dexamethasone, and polyethylene glycol‐asparaginase (MESA). Three cycles of MESA were administered to 46 patients with new or relapsed/refractory natural killer/T‐cell lymphoma. Complete response after 3 treatment cycles was 43.5%, the overall response rate was 87%, and 2‐year overall survival was 83.4%. Complete response was significantly better for newly diagnosed patients than for patients with relapsed/refractory disease. Patients with newly diagnosed disease had a significantly better overall response rate after 1, but not after 2 or 3 treatment cycles. Overall survival and progression‐free survival did not differ over 2 years. Grade 1/2 toxicities were frequent, but MESA was associated with fewer grade 3/4 events or treatment‐related deaths. These results will require confirmation in larger prospective trials.     

Head and Neck cancers—major changes in the American Joint Committee on cancer eighth edition cancer staging manual

Answer questions and earn CME/CNE The recently released eighth edition of the American Joint Committee on Cancer (AJCC) Staging Manual, Head and Neck Section, introduces significant modifications from the prior seventh edition. This article details several of the most significant modifications, and the rationale for the revisions, to alert the reader to evolution of the field. The most significant update creates a separate staging algorithm for high‐risk human papillomavirus‐associated cancer of the oropharynx, distinguishing it from oropharyngeal cancer with other causes. Other modifications include: the reorganizing of skin cancer (other than melanoma and Merkel cell carcinoma) from a general chapter for the entire body to a head and neck‐specific cutaneous malignancies chapter; division of cancer of the pharynx into 3 separate chapters; changes to the tumor (T) categories for oral cavity, skin, and nasopharynx; and the addition of extranodal cancer extension to lymph node category (N) in all but the viral‐related cancers and mucosal melanoma. The Head and Neck Task Force worked with colleagues around the world to derive a staging system that reflects ongoing changes in head and neck oncology; it remains user friendly and consistent with the traditional tumor, lymph node, metastasis (TNM) staging paradigm. CA Cancer J Clin 2017;67:122–137. © 2017 American Cancer Society.     

Extranodal involvement in young patients with diffuse large B-cell lymphoma: distribution,prognostic value and treatment options

Objective:Extranodal involvement represents a peculiar presentation of diffuse large B-cell lymphoma (DLBCL).Previous studies have suggested that older patients are more prone to extranodal involvement.This study retrospectively addressed the distribution,prognostic value and treatment options of extranodal involvement in young patients with DLBCL.Methods:A total of 329 patients were enrolled according to the inclusion requirements.The effects of gender,extranodal involvement,age-adjusted international prognostic index (aaIPI),rituximab infusion and radiotherapy on patient outcomes were evaluated.Results:Among these patients,59％ presented extranodal involvement in 16 anatomic sites.More than one instance was linked to many poorer clinical characteristics and poorer survival compared with either nodal disease or one instance.In patients with one extranodal lesion,multivariate analysis revealed that the site of extranodal involvement,but not the aaIPI or rituximab infusion,was independently related to the outcome,and radiotherapy had a negative influence on survival.Conclusions:Extranodal involvement is common in younger patients and exhibits a ubiquitous distribution.The site of extranodal involvement is of strong prognostic significance.Radiotherapy for extranodal lesions does not improve patient outcomes.     

结外NK/T细胞淋巴瘤发生的分子机制研究进展

结外NK/T细胞淋巴瘤(extranodal NK/T cell lymphoma,ENKTCL) 是我国继弥漫大B淋巴细胞淋巴瘤之后第二常见的非霍奇金淋巴瘤(non-hadgkin lymphoma,NHL).结外NK/T细胞淋巴瘤恶性程度高、临床进展快、预后差,对其发病机制的研究尤为重要.本文综述了近年来结外NK/T细胞淋巴瘤发生的分子机制研究进展,主要涉及该淋巴瘤的常见基因突变、NF-κB信号通路及JAK-STAT信号通路的主要激活机制.     

结外侵犯非霍奇金淋巴瘤224例的临床病理特征及疗效分析

目的:探讨初治时伴结外侵犯的非霍奇金淋巴瘤(non-Hodgkins lymphoma,NHL)的临床病理特征及其与疗效的关系。方法:回顾性分析初治伴结外侵犯的NHL患者224例,分析其临床病理特征,采用χ2检验,比较不同临床特征与完全缓解率之间的关系。结果:224例伴结外侵犯的患者中B细胞NHL140例,主要为弥漫大B细胞淋巴瘤(77例);T细胞NHL84例,主要为外周T细胞淋巴瘤非特指型(53例)。伴结外侵犯的B细胞NHL,最常见的侵犯部位是咽淋巴环、胃肠道和浆膜腔;伴结外侵犯的T细胞NHL,最常见的侵犯部位是咽淋巴环和鼻腔副鼻窦。B细胞NHL完全缓解率为34.3%(48例),T细胞NHL完全缓解率为31.0%(26例)。Ⅲ～Ⅳ期、B症状、体能状况差、巨大包块者完全缓解率低;性别、LDH及是否放疗与完全缓解率无相关性,P>0.05;国际预后指数(IPI)不同的患者,其完全缓解率差异有统计学意义(B细胞性淋巴瘤P=0.023,T细胞性淋巴瘤P=0.014)。结论:伴结外侵犯的NHL最常见的病理类型是弥漫大B细胞淋巴瘤和外周T细胞淋巴瘤非特指型,其完全缓解率低,可以利用IPI指数预测其疗效与预后。