原发性胃肠道淋巴瘤的诊断治疗进展

原发性胃肠道淋巴瘤(PGIL)是最常见的结外淋巴瘤,其组织发生、病理形态和生物学行为与结内淋巴瘤不同,是一组具有独特临床病理特征的肿瘤,新的分类、分期标准有助于提高临床病理诊断水平.其治疗和预后与结外淋巴瘤及胃肠道癌不同.治疗包括抗幽门螺杆菌治疗、以手术为主的综合治疗和非手术综合治疗,对于Ⅰ、Ⅱ期原发性胃淋巴瘤首选手术还是放、化疗尚存在争议,回顾分析近年来PGIL的诊断、治疗及预后资料,对不同病理分型、分期患者提出不同的治疗原则.     

原发性胃肠道淋巴瘤的诊断治疗进展

原发性胃肠道淋巴瘤(PGIL)是最常见的结外淋巴瘤，其组织发生、病理形态和生物学行为与结内淋巴瘤不同，是一组具有独特临床病理特征的肿瘤，新的分类、分期标准有助于提高临床病理诊断水平。其治疗和预后与结外淋巴瘤及胃肠道癌不同。治疗包括抗幽门螺杆菌治疗、以手术为主的综合治疗和非手术综合治疗，对于Ⅰ、Ⅱ期原发性胃淋巴瘤首选手术还是放、化疗尚存在争议，回顾分析近年来PGIL的诊断、治疗及预后资料，对不同病理分型、分期患者提出不同的治疗原则。     

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。     

59例原发性胃非霍奇金淋巴瘤的临床分析

背景与目的:胃肠道是非霍奇金淋巴瘤最常侵犯的结外器官,但其标准的治疗方法目前仍未确定,本文拟通过分析有关资料,探讨原发性胃非霍奇金淋巴瘤的临床特征及治疗策略。方法:收集1980年1月至2002年1月在中山大学肿瘤防治中心收治的59例原发性胃非霍奇金淋巴瘤的临床资料,回顾性分析其临床特点和治疗方式对患者生存期的影响。结果:78.0%原发性胃非霍奇金淋巴瘤Lugano分期为Ⅰ～Ⅱ期,按工作分型78.0%病理类型为中度恶性,免疫分型以B细胞为主(占93.9%)。本组患者采用手术联合化疗37例(62.7%),单纯化疗17例(28.8%),单纯手术5例(8.5%)。总的2、5和10年生存率分别76.4%、63.7%和42.5%,其中中度恶性淋巴瘤(包括免疫母细胞淋巴瘤)手术联合化疗者与单纯化疗者5年生存率差异无统计学意义(52.5%vs.57.1%)。结论:中高度恶性原发性胃非霍奇金淋巴瘤在治疗策略上应采用化疗为主治疗模式,手术的作用尚需前瞻性随机研究进一步证实。     

原发性睾丸淋巴瘤及原发性中枢神经系统淋巴瘤研究进展：第19届欧洲血液学会年会报道

原发性睾丸淋巴瘤（PTL）是一类少见的结外淋巴瘤,进展迅速,预后不良.对于早期局限性病变,可联合应用以蒽环类为基础的化疗、利妥昔单抗、鞘内注射甲氨蝶呤后行对侧睾丸放疗等.中枢神经系统复发风险极高,鞘内注射甲氨蝶呤可能是最好的防治方法.原发性中枢神经系统淋巴瘤（PCNSL）是一种侵袭性淋巴瘤,预后较差,对现有非霍奇金淋巴瘤标准治疗方案均不敏感,甲氨蝶呤联合阿糖胞苷是目前的标准诱导方案.抗CD20单克隆抗体虽然脑脊液含量低,但仍有治疗效果.高剂量化疗联合自体造血干细胞移植缓解率高,3年总生存率可达87％.     

原发性中枢神经系统淋巴瘤的影像学特点及治疗

原发性中枢神经系统淋巴瘤是一种比较罕见的结外原发非霍奇金淋巴瘤（NHL）,其细胞来源多为弥漫大B淋巴细胞,淋巴细胞影像学表现为单发或多发的深部脑实质、脑室周围或脑膜等处的病变。其治疗尚无标准方案,目前多采用放化疗结合的综合治疗。化疗采用以大剂量MTX为主的方案,全脑放疗已被公认为治疗PCNSL的有效手段。但最佳的化疗方案组合、MTX最佳剂量及放疗剂量仍无定论。我们收治一例原发性中枢神经系统淋巴瘤,于外院行肿瘤切除术,术后予阿糖胞苷加大剂量MTX化疗6程后残留,现经多学科讨论后认为应予辅助放疗加替莫唑胺维持化疗。现患者已完成放疗,替莫唑胺维持化疗中。本文就此病例的影像特点及治疗进行讨论。     

原发性骨淋巴瘤3例并文献复习

目的：探讨原发性骨淋巴瘤的临床特征、治疗方法及预后.方法：对南京八一医院确诊为原发性骨淋巴瘤的3例患者的临床资料进行回顾性分析.结果：随访8月-11年,采用化疗联合放疗2例,均存活.单纯化疗1例,因病情进展已死亡.结论：原发性骨淋巴瘤为临床少见的结外淋巴瘤,以弥漫大B细胞性非霍奇金淋巴瘤多见,预后较好.通常见为溶骨性破坏,局部症状重而全身症状轻,影像学检查无特异性,造成早期准确诊断非常困难,容易发生误诊.确诊有赖于病理及免疫组化证实.治疗以放化疗综合治疗为宜.     

原发性甲状腺恶性淋巴瘤27例临床分析

  【摘要】　目的　探讨原发性甲状腺恶性淋巴瘤的临床特点及治疗方法。方法　回顾性分析27例原发性甲状腺恶性淋巴瘤患者的临床资料。结果　27例原发性甲状腺恶性淋巴瘤病理类型均为B细胞型,多为低度恶性的结外边缘区B细胞淋巴瘤,临床分期均为Ⅰ、Ⅱ期,予以合理综合治疗,预后相对较好。结论　原发性甲状腺恶性淋巴瘤应强调综合治疗,外科手术主要起协助诊断的作用,术后放疗与化疗是必要的。     

原发性肺淋巴瘤5例诊治体会

原发性肺淋巴瘤是指起源于结外,发生于肺内淋巴组织的肿瘤,临床少见,且较少特征性临床表现,易误诊。我们对1997年4月至2001年7月收治的5例原发性肺淋巴瘤进行回顾性分析,并复习相关文献探讨原发性肺淋巴瘤的临床特点、诊断治疗及预后。1临床资料与方法1.1一般资料本组5例患者均     

胃肠道原发性恶性淋巴瘤的诊断与治疗

胃肠道原发性恶性淋巴瘤(PMLGI)是最常见的结外淋巴瘤,是胃肠道肿瘤的一种特殊类型,术前诊断较困难,临床误诊率高,其疗效和预后与胃肠道其它恶性肿瘤不同.     

原发性卵巢非霍奇金淋巴瘤1例及相关文献复习

目的:探讨原发性卵巢弥漫性大B细胞淋巴瘤的病因、临床表现、诊断、治疗及预后。方法:报道1例原发性卵巢弥漫性大B细胞淋巴瘤的临床病理资料及术后免疫组化结果，并阅读及复习国内外相关文献。结果:该例患者为（右）卵巢弥漫性大B细胞淋巴瘤，积极治疗后现考虑肿瘤复发。结论:原发性卵巢弥漫性大B细胞淋巴瘤罕见，确诊依赖于术后病理及免疫组化，预后不佳，常采用以手术为主，辅以化疗和放疗的综合治疗。     

以原发性脾淋巴瘤为表现的弥漫性大B细胞淋巴瘤1例并文献复习

目的：探讨原发性脾淋巴瘤（PSL）的诊断以及原发性脾弥漫性大B细胞淋巴瘤的治疗。方法：报告1例以PSL为表现的弥漫性大B细胞淋巴瘤并结合文献进行复习。结果：患者表现为左上腹疼痛,无发热、盗汗,无浅表淋巴结肿大,CT证实脾脏内有占位性病变,脾脏病理示弥漫性大B细胞淋巴瘤,外周血和骨髓检查正常,脾切除术后PET/CT未见明显与淋巴瘤相关的18F-脱氧葡萄糖摄取异常增高表现,后给予联合化疗,随访8月余仍处于完全缓解期。结论：PSL是一少见肿瘤,采取脾切除后继以CHOP±利妥昔单抗治疗原发性脾弥漫性大B细胞淋巴瘤能够达到长期生存的目的。     

Successfully-treated mesenteric non-Hodgkin's lymphoma involving hepatic mass--a case report

A mesenteric diffuse large B-cell lymphoma which also involves the liver is very rare. We describe herein a mesenteric diffuse large B-cell lymphoma with hepatic involvement successfully treated by the combination of surgical resection and multiagent chemotherapy. A 77-year-old man was referred to our hospital because of a right lower abdominal tumor. Abdominal computed tomography showed a mass in the mesenterium at the ileocoecal region and multiple mass in the liver. Gallium scintigram showed focal hot uptake at the ileocoecal region and multiple areas of increased Gallium uptake in the liver. With the diagnosis of a mesenteric tumor with liver metastases, a laparotomy was performed. By an intraoperative pathological examination, non-Hodgkin's lymphoma was suggested. The mesenteric mass was completely resected, but additional operative procedures were not done to the liver. After the operation, the patient was determined to have Stage IVB diffuse large B-cell lymphoma, and chemotherapy based on the CHOP-like regimen was given. After the 8th course of such chemotherapy, he was confirmed to have achieved a complete remission by abdominal computed tomography and Gallium scintigram.The Stage IV mesenteric diffuse large B-cell lymphoma involving the liver seems to be an indication for combination therapy of surgical resection and multiagent chemotherapy.     

Diffuse large B-cell non-Hodgkin's lymphoma presenting as a vaginal mass in a patient with a history of intestinal mucosa-associated lymphoid tissue (MALT) lymphoma

Vaginal involvement in non-Hodgkin's lymphoma is uncommon and is often secondary to disseminated disease. Primary disease at this site is quite rare. We present here an unusual case of a patient who developed a diffuse large B-cell lymphoma presenting as a vaginal mass after having been treated for primary intestinal mucosa-associated lymphoid tissue (MALT) lymphoma 4 years earlier. Combined chemotherapy and radiation therapy for the intestinal MALT lymphoma produced complete remission that lasted for 2 years. Chemotherapy given for the diffuse large B-cell lymphoma with secondary vaginal involvement produced a second complete remission. The second remission was consolidated with high-dose chemotherapy and autologous bone marrow transplantation. Although the patient died from complications related to the transplant procedure, the disease was in complete remission at the time of her death. Given the rarity of primary intestinal MALT lymphoma and primary vaginal lymphoma, no standard treatment has been established. Treatment options have included chemotherapy, radiation therapy, or surgery, given alone or in combination.     

培美曲塞为主的化疗方案治疗中老年非霍奇金淋巴瘤回顾性分析CSCD

目的回顾性分析培美曲塞为主的化疗方案治疗中老年非霍奇金瘤患者的安全性及有效性。方法回顾性收集2016年11月至2018年10月中国医学科学院肿瘤医院收治的以培美曲塞为主的化疗方案治疗的非霍奇金淋巴瘤患者临床资料,并对相关文献进行系统性综述。结果共纳入10例非霍奇金淋巴瘤患者,7例为弥漫大B细胞淋巴瘤,2例为血管免疫母T细胞淋巴瘤,1例为结外NK/T细胞淋巴瘤。肿瘤复发或一线治疗进展后接受培美曲塞为主的化疗方案,弥漫大B细胞淋巴瘤有4例达到部分缓解,3例疾病进展,出现的不良反应主要为Ⅰ~Ⅱ度骨髓抑制及胃肠道反应。结论培美曲塞为主的化疗方案治疗弥漫大B细胞淋巴瘤患者,尤其是原发性中枢神经系统淋巴瘤,可能有一定的有效性,且安全性可控。     

Richter's syndrome presenting as a nasal lymphoma

Richter's syndrome (RS) is a high-grade large cell lymphoma arising in patients with B-cell chronic lymphocytic leukemia (CLL). The prognosis of RS is very poor and the development of RS is a serious complication of CLL. We present a case of a patient with a 4-year history of B-cell CLL who developed diffuse large B-cell nasal lymphoma. The patient exhibited good response to chemotherapy and irradiation and achieved complete remission. Five months later, the patient suffered leptomeningeal involvement and has been treated with repeated intrathecal chemotherapy. Leukemic cells of the patient expressed CD13. CD13+ CLLs have been reported to have an unfavorable prognosis and this case may support the view.     

Successful treatment of diffuse large B-cell non-hodgkin lymphoma with modified CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) chemotherapy and rituximab in a patient with Nijmegen syndrome

A 17-year-old Croatian boy with Nijmegen breakage syndrome (NBS) who developed diffuse large B-cell non-Hodgkin lymphoma is presented. The majority of the patients with this rare autosomal recessive disease are of Slavic origin and, in most of them, the disease is caused by NBS1 mutation 657del5, as was found in our patient. Nijmegen breakage syndrome is characterized by microcephaly, growth retardation, abnormal facial appearance, spontaneous chromosomal rearrangements, immunodeficiency, and a high predisposition to cancer development, predominantly lymphoma. Because of increased sensitivity to radiation therapy and chemotherapy, the treatment of malignancies in patients with NBS can be difficult. To our knowledge, our patient is the first with NBS reported in the literature who was successfully treated for diffuse large B-cell lymphoma with the anti-CD20 monoclonal antibody rituximab in addition to a modified dose of CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) chemotherapy. He has been in complete remission for 3 years after finishing the treatment.     

Diffuse large B-cell primary gastric lymphoma treated successfully with the CD20 monoclonal antibody (rituximab): a case with severe liver dysfunction due to liver cirrhosis and hepatocellular carcinoma

Primary gastric lymphoma is relatively rare in the scope of gastric malignancies. Here we report a case of diffuse large B-cell primary gastric lymphoma treated successfully with the CD20 monoclonal antibody, rituximab, alone. Because the patient had a complication of severe liver dysfunction due to hepatitis C virus induced-liver cirrhosis and hepatocellular carcinoma, it was difficult to treat the primary gastric lymphoma using standard therapy such as surgical resection and cocktail chemotherapy. Therefore, rituximab was administered to the patient, resulting in complete remission of the primary gastric lymphoma. This case indicates that monotherapy using only rituximab may be a promising option for the treatment of patients with diffuse large B-cell lymphoma accompanied by severe liver dysfunction.     

4例肝脏原发淋巴瘤的临床病理特点和治疗分析

背景与目的:肝脏原发淋巴瘤(primaryhepaticlymphoma,PHL)是临床上极为罕见的恶性肿瘤,国内外相关文献报道不多。本文旨在初步探讨PHL的临床病理特点和治疗。方法:对近十年来中山大学肿瘤防治中心收治的4例PHL患者的临床资料进行总结分析。结果:本组病例中,男性3例,女性1例,患者中位年龄53岁;单发病灶3例,多发病灶1例;4例均有HBV指标阳性;3例术前误诊,1例术前未能确诊;3例为非霍奇金淋巴瘤,且均为B细胞性,1例为霍奇金淋巴瘤;2例行手术切除,另2例仅行活检;术后均行全身辅助化疗。结论:PHL可能与HBV病毒感染有关;对于伴有B症状的肝内占位性病变要怀疑PHL的可能性;手术切除辅助CHOP方案化疗是目前较为理想的治疗方法。     

Evolving role of rituximab in the treatment of patients with non-Hodgkin's lymphoma

Rituximab is the first monoclonal antibody to have been registered for the treatment of B-cell lymphomas. Randomized studies have demonstrated its activity in follicular lymphoma, mantle cell lymphoma, and diffuse large B-cell lymphoma in untreated or relapsing patients. Rituximab has transformed the outcome of these patients because of its high activity and low toxicity. A combination of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone, has the highest efficacy ever described with any chemotherapy in diffuse large B-cell lymphoma and follicular lymphoma.