原发结外弥漫大B细胞淋巴瘤的临床特点分析

发布时间：2018-07-11 18:13

本文选题：扁桃体 + 弥漫大B细胞淋巴瘤　；参考：《南京医科大学》2017年博士论文

【摘要】：第一部分原发扁桃体弥漫大B细胞淋巴瘤29例临床特点分析目的扁桃体是头颈部非霍奇金淋巴瘤(NHL)好发部位,弥漫大B细胞淋巴瘤(DLBCL)是其最常见病理类型,本研究目的为总结原发扁桃体弥漫大B细胞淋巴瘤(PT-DLBCL)临床特征、免疫表型及预后因素。方法回顾性分析2009年10月至2017年1月于我院就诊的29例PT-DLBCL患者临床资料。结果29例患者中,男性17例(58.6%),女性12例(41.4%),中位年龄58(16～86)岁。所有患者均因咽部不适,异物感(89.7%)或咽部疼痛(27.6%)就诊。能明确细胞起源的28例患者中,生发中心B细胞(GCB)型占75%,非生发中心 B 细胞(non-GCB)型占 25%。Ann Arbor 分期 Ⅰ 期占 41.4%,Ⅱ期占 58.6%,无Ⅲ、Ⅳ期患者。中位随访时间39(3～89)个月,1例患者(3.4%)失访。中位治疗6(3～8)个疗程后对28例患者进行评估,其中20例(71.4%)达完全缓解(CR),7例(25%)达部分缓解(PR),1例(3.6%)患者疾病稳定(SD)。3年及5年无进展生存(PFS)均为82.5%,3年及5年总生存(OS)均为95.5%。年龄70岁(P=0.034)与短的PFS相关;IPI2分(P=0.034)、LDH增高(P0.0001)、治疗方案中未使用利妥昔单抗(P=0.034)与短的OS相关结论PT-DLBCL分期早,大多数为GCB型,常见于老年。患者对放化疗敏感,预后较好,大多数患者治疗后可获得长期生存。年龄70岁、LDH增高的患者预后相对较差,联合免疫化疗效果优于单纯化疗。第二部分原发中枢神经系统弥漫大B细胞淋巴瘤42例临床特点分析目的分析原发中枢神经系统弥漫大B细胞淋巴瘤(PCNS-DLBCL)的发病情况、临床表现、治疗效果及预后。方法收集2008年5月至2017年1月经我院诊治并有明确病理确诊的42例PCNS-DLBCL患者的资料,对一般情况、临床表现、中枢病变累及部位、病理免疫组化、实验室检查、治疗方案、疗效及预后进行分析。结果42例患者中,男性22例(52.4%),女性20例(47.6%),中位年龄61(13～77)岁。主要临床表现为头晕(33.3%)或肢体乏力或轻偏瘫(35.7%)。病理类型均为弥漫大B细胞淋巴瘤(DLBCL)。可进行细胞起源分析的35例患者中,11例(31.4%)为生发中心B细胞(GCB)型,24例(68.6%)为非生发中心B细胞(non-GCB)型。后续未接受放化疗的患者3例(7.1%),单纯放疗患者5例(11.9%),单纯化疗患者9例(21.4%)及化放疗联合治疗患者25例(59.6%),4例(9.5%)患者接受自体造血干细胞。中位随访时间24(2-65)个月,中位总生存(OS)34(2～65)个月,预估5年OS为31.9%。年龄70岁是无进展生存(PFS)及OS的不良预后因素(P0.0001,P=0.001),深部脑组织浸润与OS负相关(P=0.008),除手术外,使用联合放化疗的患者OS优于未行放化疗的患者(P=0.035)。结论PCNS-DLBCL总体预后差。大于70岁,有深部脑组织浸润的患者预后相对更差。化疗联合放疗或移植治疗患者的治疗效果优于单纯化疗患者。第三部分原发乳腺弥漫大B细胞淋巴瘤18例临床特点分析目的分析原发乳腺弥漫大B细胞淋巴瘤(PB-DLBCL)的临床特点。方法收集我院2007年1月至2017年1月收治的18例PB-DLBCL患者资料,对患者症状、体征、免疫表型、预后因素及治疗方案的选择进行分析。结果18例患者均因自觉乳腺肿块就诊,经手术切除或肿块穿刺后病理检查明确诊断。15例(83.3%)免疫组织化学资料完全的患者中,生发中心B细胞(GCB)型4例(26.7%),非生发中心B细胞(non-GCB)型11例(73.3%)。18例患者中Ann Arbor分期Ⅰ期占44.4%,Ⅱ期占55.6%。1例患者(5.6%)至外院就诊,失访。余17例患者中位随访时间66(13～112)个月。2例患者(11.8%)死亡,均死于本病。5年无进展生存(PFS)88.2%,5年总生存(OS)86.7%。年龄≥60岁(P=0.021)、分期调整国际预后指数(IPI)≥2分(P=0.006)以及β2-微球蛋白(β2-MG)增高(P=0.037)与PFS负相关;年龄≥60岁(P=0.034)和分期调整IPI≥2分(P=0.012)与OS负相关。手术后未经放化疗治疗的患者在确诊16个月后死亡,接受放化疗的患者至随访结束仅1例死亡。结论PB-DLBCL与其它乳腺肿瘤相比,无特异性的临床表现,诊断依靠病理。手术后未行后续放化疗治疗的患者生存期短,手术后行放化疗治疗的患者可延长生存。
[Abstract]:Part 1: clinical characteristics of 29 cases of primary tonsillar diffuse large B cell lymphoma. Tonsillar is a good location of non Hodgkin's lymphoma (NHL) in the head and neck. Diffuse large B cell lymphoma (DLBCL) is the most common pathological type. The purpose of this study is to summarize the clinical features of primary tonsil diffuse B cell lymphoma (PT-DLBCL) and the immune form. Methods and prognostic factors. Methods a retrospective analysis of the clinical data of 29 PT-DLBCL patients in our hospital from October 2009 to January 2017. Results of 29 cases, 17 men (58.6%), 12 women (41.4%), middle age 58 (16~86) years old. All patients were diagnosed with pharynx discomfort, foreign body sensation (89.7%) or pharynx pain (27.6%). The origin of cell origin could be clearly defined. Of the 28 patients, the primary B cell (GCB) cells accounted for 75%, the non germinal center B cell (non-GCB) accounted for 41.4%, 58.6%, no III, IV, 39 (3~89) months, 1 patients (3.4%). After middle treatment 6 (3~8) courses, 28 patients were evaluated, 20 cases (71.4) %) reached complete remission (CR), 7 (25%) reached partial remission (PR), 1 (3.6%) patients with disease stability (SD) were 82.5% for.3 and 5 years of progression free survival (PFS), 3 and 5 years (OS) were all 95.5%. age 70 years (P=0.034) associated with short PFS; IPI2 points (P =0.034) increased, and the treatment regimen did not use rituximab and short PT-DLBCL staging is early, most of them are GCB type and common in old age. Patients are sensitive to radiotherapy and chemotherapy, and the prognosis is better. Most patients can obtain long-term survival after treatment. The age of 70 years old, the prognosis of patients with increased LDH is relatively poor and the effect of combined immuno chemotherapy is better than that of chemotherapy alone. The second part of the primary central nervous system diffuse large B cell lymph nodes. Analysis of the clinical characteristics of 42 cases of tumor to analyze the incidence, clinical manifestation, therapeutic effect and prognosis of the primary central nervous system diffuse large B cell lymphoma (PCNS-DLBCL). Methods the data of 42 cases of PCNS-DLBCL patients with pathological diagnosis were collected from May 2008 to 2017 in 1 menstruation, and the general, clinical and central diseases were analyzed. In the 42 patients, 22 (52.4%), 20 women (47.6%) and 61 (13~77) age were found in 42 patients. The main clinical manifestations were dizziness (33.3%) or limb fatigue or hemiplegia (35.7%). The pathological types were diffuse large B cell lymphoma (DLBCL). Of the 35 cases of cell origin analysis, 11 (31.4%) were type of germinal center B cell (GCB), 24 (68.6%) was non germinal B cell (non-GCB), 3 (7.1%), 5 (11.9%), 9 (21.4%) patients with simple radiotherapy, 25 cases (59.6%) in combination therapy and radiotherapy. The patients received autologous hematopoietic stem cells. The median follow-up time was 24 (2-65) months, the median total survival (OS) was 34 (2~65) months, and the estimated 5 year OS was 70 years old for 31.9%. age (P0.0001, P=0.001), and the deep brain tissue infiltration and OS negative correlation (P=0.008). Except for the operation, the OS of the patients who used combined radiotherapy and chemotherapy was better than the failure. Patients with chemotherapy (P=0.035). Conclusion the overall prognosis of PCNS-DLBCL is poor. More than 70 years old, the patient with deep brain tissue infiltration is worse. The treatment effect of chemotherapy combined with radiotherapy or transplantation is better than that of patients with simple chemotherapy. The third part of primary breast diffuse large B cell neenoma is analyzed in 18 cases of clinical analysis of primary milk The clinical features of the adenoid diffuse large B cell lymphoma (PB-DLBCL). Methods 18 cases of PB-DLBCL patients were collected from January 2007 to January 2017 in our hospital. The symptoms, signs, immunophenotypes, prognostic factors and the choice of treatment options were analyzed. Results 18 patients were treated by self aware breast lumps and underwent surgical resection or mass puncture. Pathological examination clearly diagnosed.15 (83.3%) patients with complete immuno histochemical data, 4 cases (26.7%) of germinal center B cell (GCB), 11 cases of non germinal center B cell (non-GCB) type (73.3%).18 cases, Ann Arbor stage I accounted for 44.4%, phase II accounted for 55.6%.1 cases (5.6%) to external hospital and lost visit. The median follow-up time of the remaining 17 patients was 66 (3). 13~112).2 patients (11.8%) died, all died of non progressive survival (PFS) 88.2%, 5 years of total survival (OS) 86.7%. age over 60 years (P=0.021). The international prognostic index (P=0.006) and beta 2- microglobulin (P=0.006) and beta 2- microglobulin (P=0.037) were associated with PFS negative correlation in 5 years. 12) negative correlation with OS. Patients who did not undergo chemotherapy after surgery died after 16 months of diagnosis, and only 1 patients died at the end of the follow-up. Conclusion PB-DLBCL had no specific clinical manifestations compared with other breast tumors, and the diagnosis depended on the pathology. The patients who did not follow the follow-up chemotherapy after surgery were short of survival, after surgery. Patients treated with chemotherapy can prolong their survival.
【学位授予单位】：南京医科大学
【学位级别】：博士
【学位授予年份】：2017
【分类号】：R733.1

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