结节性淋巴细胞为主型霍奇金淋巴瘤七例

 目的　探讨结节性淋巴细胞为主型霍奇金淋巴瘤（NLPHL）临床病理特征及治疗。方法　经病理免疫组织化学证实为NLPHL患者7例。6例患者接受化疗和受累野放疗的综合治疗;1例仅接受单纯化疗。结果　3年局部控制率和总生存率分别为100 %和86 %。1例患者死于化疗后肺部感染。结论　NLPHL预后良好,综合治疗耐受好,不良反应少,但应注意治疗后不良反应的处理。     

结节性淋巴细胞为主型霍奇金淋巴瘤诊断及治疗进展

 结节性淋巴细胞为主型霍奇金淋巴瘤的形态特征、转化、免疫表型都有其特殊性,因此临床表现、诊断和治疗与其他经典类型霍奇金淋巴瘤有差异。文章综述了该病诊断及治疗的最新进展,尤其是近期常规治疗的疗效和利妥昔单抗的应用情况。     

结节性淋巴细胞为主型霍奇金淋巴瘤和富于T细胞和(或)组织细胞的B细胞淋巴瘤的鉴别诊断

目的 探讨结节性淋巴细胞为主型霍奇金淋巴瘤（NLPHL）和富于T细胞和（或）组织细胞的B细胞淋巴瘤（TCRBCL）的鉴别诊断。方法 按照WHO淋巴瘤新分类法,对15例NLPHL和16例TCRBCL的组织学和免疫表型进行分析,并分别对其中3例NLPHL和4例TCRBCL进行了EBERl／2原位杂交和IgH基因重排检测。结果 组织学上,NLPHL和TCRBCL均表现为小淋巴细胞背景中散在分布的肿瘤性大细胞。NLPHL的肿瘤性大细胞形态特征以L＆H细胞（即爆米花细胞）为主,TCRBCL的肿瘤性大细胞以中心母细胞类型为主,两者都可伴有其他变异形态。免疫表型上,NLPHL和TCRBCL的肿瘤性大细胞都呈CD20、CD79a、bcl-6和EMA阳性,CD3、CD45RO、CDl5和CD30阴性,背景小淋巴细胞以T淋巴细胞为主。但在NLPHL中,CD57阳性细胞明显多于TIA-1阳性细胞,小B淋巴细胞呈小灶状或弥漫散在分布;而在TCRBCL中,TIA-1阳性细胞明显多于CD57阳性细胞,小B淋巴细胞非常稀少。CD21检测显示,NLPHL的结节呈CD21阳性滤泡树突细胞（FDC）网架结构,而TCRBCL以及NLPHL的弥漫类型或弥漫区域中,FDC网架缺乏。NLPHL和TCRBCL都呈EBER1／2阴性,IgH基因重排可检测到80～120bp的单克隆条带。结论 NLPHL和TCRBCL有组织学和免疫表型特征的相似性,诊断和鉴别诊断必须结合形态学和瘤细胞、背景细胞的免疫表型特征。     

结节性淋巴细胞为主型霍奇金淋巴瘤向富含T细胞/组织细胞的大B细胞淋巴瘤样转化一例并文献复习

目的:探讨结节性淋巴细胞为主型霍奇金淋巴瘤（NLPHL）经治疗后复发并向富含T细胞/组织细胞的大B细胞淋巴瘤（THRLBCL）样转化患者的临床特点,提高对NLPHL和THRLBC鉴别诊断与治疗的认识。方法:回顾性分析复旦大学附属华东医院2019年10月收治的1例NLPHL治疗后复发并向THRLBCL样转化患者的治疗经过,并进行相关文献复习。结果:该患者经ABVD方案治疗后复发,再次经病理活组织及免疫组织化学检查诊断为THRLBCL样转化,经R-CHOP方案治疗后完全缓解。结论:NLPHL化疗效果不佳时需考虑是否存在向THRLBCL样转化的可能,需及时行活组织病理检查,调整治疗方案。     

淋巴细胞为主型霍奇金病的研究进展

淋巴细胞为主型霍奇金病(LPHD)或结节性淋巴细胞为主型霍奇金病(NLPHD)不同于结节硬化型、混合细胞型等经典型霍奇金病(CHD),是一种少见的、具有独特的病理和临床特征的B细胞淋巴瘤.临床特点为好发男性,病变主要限于体表淋巴结,进展缓慢,无全身症状.LPHD恶性细胞免疫表型CD20+,CD15-和CD30-是区别于CHD的重要病理学依据.LPHD传统上一线采用化疗、放疗综合治疗,虽然疗效显著,但多年后有复发倾向,加之,治疗所致远期毒性增多,因此,LPHD是否需要如此强烈的治疗遭到质疑.随着分子病理学的阐明以及靶向治疗药物的发展,其治疗可望得到改善.     

淋巴细胞为主型霍奇金病的研究进展

淋巴细胞为主型霍奇金病(LPHD)或结节性淋巴细胞为主型霍奇金病(NLPHD)不同于结节硬化型、混合细胞型等经典型霍奇金病(CHD),是一种少见的、具有独特的病理和临床特征的B细胞淋巴瘤.临床特点为好发男性,病变主要限于体表淋巴结,进展缓慢,无全身症状.LPHD恶性细胞免疫表型CD20+,CD15-和CD30-是区别于CHD的重要病理学依据.LPHD传统上一线采用化疗、放疗综合治疗,虽然疗效显著,但多年后有复发倾向,加之,治疗所致远期毒性增多,因此,LPHD是否需要如此强烈的治疗遭到质疑.随着分子病理学的阐明以及靶向治疗药物的发展,其治疗可望得到改善.     

腮腺淋巴组织霍奇金淋巴瘤1例

患者男性，维吾尔族，年龄５８岁。左上颈肿物１年，伴疼痛２个月。１年前触及左腮腺部花生米大肿块，渐增大，近２个月增大明显伴持续钝痛，于２００１年２月２０日入本院五官科。查体营养良好，全身浅表淋巴结未触及，左腮腺触及１１．５cm×２．５cm哑铃状肿物，质中、触痛，对侧腮腺及双侧颌下腺正常，颈部未触及肿大淋巴结。临床诊断“腮腺淋巴瘤”。于３月７日行肿物摘除术送病检。病理检查：腮腺肿物标本６cm×４cm×４．５cm，切面灰白均细，包膜完整。镜检：在腮腺组织中可见弥漫分布的瘤细胞和背景细胞，肿瘤细胞大，呈异型性，可…     

儿童非霍奇金淋巴瘤53例临床分析

目的 总结53例儿童非霍奇金淋巴瘤的临床特点及治疗方法，方法 回顾性分析53例儿童非霍奇金淋巴瘤的临床症状，病理类型、诊断和治疗，结果 浅表淋巴结肿大为首发症状者36例，全组误诊率71.7%。病理类型主要为高度恶性（31/53），全组5年生存率为22.6%。结论 儿童非霍奇金淋巴瘤为儿童常见肿瘤，恶性度高，误诊较为常见，治疗应以化疗为主配合局部放疗。     

Familial nodular lymphocyte predominant Hodgkin lymphoma: Successful treatment with CHOP plus rituximab

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare tumor type distinct from classical Hodgkin lymphoma and its familial form is unusual. The two cases (mother at age 48 and son at age 30 years) of NLPHL in advanced clinical stage are described. The patients were successfully treated with an immunochemotherapy schedule consisting CHOP plus rituximab (CHOP-R). This chemotherapy was well tolerated and the patients reached complete remission. These remissions were for 34 and 40 months for mother and son, respectively. In patients with NLPHL, CHOP-R regimen should be used as an alternative treatment regimen to obtain a good long-lasting response without any adverse events.     

Diffuse large B-cell lymphoma arising in nodular lymphocyte predominant Hodgkin lymphoma: a report of 21 cases from the Nebraska Lymphoma Study Group

We sought to investigate the clinical characteristics and pathologic features and survival outcome of patients with diffuse large B-cell lymphoma (DLBCL) arising in nodular lymphocyte predominant Hodgkin lymphoma (NLPHL), since controversy regarding their prognosis exists in the literature. Twenty-one patients with DLBCL arising either concurrently with (n = 7) or subsequent to (n = 14) a diagnosis of NLPHL were identified in the Nebraska Lymphoma Study Group Registry. The clinical and pathologic features of the cases were evaluated, and survival analysis was performed from the time of diagnosis of DLBCL. The median time to the development of DLBCL in those with prior NLPHL was only one year (range, 0.5 - 24 years). The median age of the patients at the time of diagnosis of DLBCL was 46 years (range, 18 - 72 years) and the male to female ratio was 17 : 4. Ten patients presented with nodal DLBCL only, 6 patients presented with both nodal and extranodal involvement, and 5 patients presented with only extranodal DLBCL. Eleven patients had limited stage (I/II) disease and 10 had advanced stage (III/IV) disease. The median overall survival (OS) and failure-free survival (FFS) of the entire group was 35 months and 11 months, respectively, and the predicted 5-year OS and FFS was 31% and 18%, respectively. There were no significant differences in the survival outcomes between patients with DLBCL arising in NLPHL and age- and sex- matched patients with de novo DLBCL. Our findings suggest that patients with DLBCL arising in NLPHL have a prognosis similar to those with de novo DLBCL and should be treated aggressively.     

Nodular lymphocyte-predominant Hodgkin lymphoma

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare subtype of Hodgkin lymphoma that differs from classic Hodgkin lymphoma (cHL) with respect to histologic and clinical presentation. Because the prognosis of NLPHL in early unfavorable and advanced stages is similar to that of cHL, treatment is similar. In contrast, early favorable-stage NLPHL has a better prognosis than cHL. Thus, NLPHL in early favorable stages might be treated with reduced-intensity programs without compromising cure rates. Because involved-field radiotherapy alone seems to be as effective as extended-field radiotherapy or combined modalities, it has been adopted by the German Hodgkin Study Group and the European Organisation for Research and Treatment of Cancer as the treatment of choice for stage IA NLPHL. Now that efficacy of the monoclonal antibody rituximab has been shown in relapsed NLPHL, its use in the first-line treatment of NLPHL is under investigation. for the German Hodgkin Study Group     

Resection alone in 58 children with limited stage, lymphocyte-predominant Hodgkin lymphoma-experience from the European network group on pediatric Hodgkin lymphoma

BACKGROUND: Lymphocyte-predominant Hodgkin lymphoma (LPHL) is a rare, CD20-positive, good prognostic lymphoma in children. Patients with early-stage LPHL who underwent successful surgical lymph node resection alone have been reported. To clarify the optimum treatment strategy in children, European study groups were asked to report their experience of surgery alone used in the treatment of pediatric LPHL. METHODS: Data from 58 patients were collected by the French Society for Pediatric Cancers, the German-Austrian Pediatric Study Group/German Society of Pediatric Oncology and Hematology (Germany), and the Children's Cancer and Leukaemia Group (United Kingdom). In total, there were 50 boys and 8 girls, and the median age was 11 years (age range, 4-17 years). Fifty-four patients had stage IA disease, 2 patients had stage IIA disease, and 2 patients had stage IIIA disease. RESULTS: With a median follow-up of 43 months (range, 2-202 months), the overall survival rate was 100%, and the progression-free survival (PFS) rate was 57%. Fifty-one of 58 patients achieved complete remission (CR) after surgery. In the CR group, the overall PFS rate was 67% (95% confidence interval, 51-82%). All seven patients who had residual disease after initial surgery developed recurrences (P = .003). Among 18 patients with stage IA LPHL who developed recurrent disease, 11 patients had local recurrences, and 7 patients recurred in stage IIA. One patient with stage IIIA disease presented with high-grade B-cell non-Hodgkin lymphoma at 10 years of follow-up. CONCLUSIONS: When complete resection was achieved, a substantial proportion of patients with surgically treated, early-stage LPHL experienced long-term remission and actually may have been cured.     

Diffuse large B-cell lymphoma arising in nodular lymphocyte predominant hodgkin lymphoma. A report of 21 cases from the Nebraska Lymphoma Study Group

We sought to investigate the clinical characteristics and pathologic features and survival outcome of patients with diffuse large B-cell lymphoma (DLBCL) arising in nodular lymphocyte predominant Hodgkin's disease (NLPHL), since controversy regarding their prognosis exists in the literature. Twenty-one patients with DLBCL arising either concurrently with (n=7) or subsequent to (n=14) a diagnosis of NLPHL were identified in the Nebraska Lymphoma Study Group Registry. The clinical and pathologic features of the cases were evaluated, and survival analysis was performed from the time of diagnosis of DLBCL. The median time to the development of DLBCL in those with prior NLPHL was only one year (range, 0.5-24 years). The median age of the patients at the time of diagnosis of DLBCL was 46 years (range, 18-72 years) and the male to female ratio was 17:4. Ten patients presented with nodal DLBCL only, six patients presented with both nodal and extranodal involvement, and five patients presented with only extranodal DLBCL. Eleven patients had limited stage (I/II) disease and 10 had advanced stage (III/IV) disease. The median overall survival (OS) and failure-free survival (FFS) of the entire group was 35 months and 11 months, respectively, and the predicted five-year OS and FFS was 31 and 18%, respectively. There were no significant differences in the survival outcomes between patients with DLBCL arising in NLPHL and age- and sex- matched patients with de novo DLBCL. In conclusion, our findings suggest that patients with DLBCL arising in NLPHL have a prognosis similar to those with de novo DLBCL and should be treated aggressively.