淋巴结外淋巴瘤的误诊分析与对策

目的　对结外淋巴瘤的误诊原因分析并提出相应的防止对策。方法　自 1980年 1月至 2 0 0 0年 7月 ,对收治非霍奇氏淋巴瘤 85例患者进行回顾性分析。结果　 85例非霍奇氏淋巴瘤中结外淋巴瘤 5 0例 ( 5 8.5 %) ,误诊 30例 ,误诊率高达 6 0 %。 30例中发生于消化道者最多 ,共 11例 ( 36 .6 %) ,其次为鼻及咽部组织 ,共 7例 ( 2 3.3%) ,其它部位少见共12例 ( 4 0 %)。结论　结外淋巴瘤误诊发生于消化道、鼻及咽部组织为多。但可发生于任何器官组织 ,其临床表现无特征性 ,诊断困难 ,易发生误诊。临床医师必须具有多学科横向知识 ,加强对结外淋巴瘤的认识 ,并尽快进行病理学检查。     

原发性结外淋巴瘤42例临床及误诊分析

目的：探讨结外型恶性淋巴瘤临床特点、误诊原因及早期诊断和治疗。方法：对经病理确诊的结外非霍奇金淋巴瘤42例进行回顾性分析。结果：误诊率66．7％，发病部位以韦氏环、鼻腔最多见，其次为皮肤和胃肠道：29例行综合治疗，手术 化疗和／或放疗22例(5例联合生物治疗)，治疗 化疗7例；随访40例(95．2％)，尚有24例健在，生存5年以上者8例，Ⅰ、Ⅱ期占75．0％。结论：结外型淋巴瘤易误诊，临床表现缺乏特异性，韦氏环、鼻腔及皮肤最易受侵犯，病理检查是确诊的唯一方法；早期诊断、早期治疗预后较佳。     

原发性乳腺恶性淋巴瘤(附3例报告及文献复习)

原发性乳腺恶性淋巴瘤(Primary Breast Lymphoma，PBL)在临床上极为少见，献报告其发病率仅占原发性乳腺恶性肿瘤的0．04％～0.5％。占所有结外淋巴瘤的2．2％；绝大多数为非霍奇金淋巴瘤(NHL)。现将我院3例PBL患的临床特点、诊断及治疗报告如下，并结合献进行讨论。     

原发于淋巴结外恶性淋巴瘤44例临床分析

观察淋巴结外淋巴瘤的临床表现特点及误诊原因,方法复习近年来经病理检查确诊的１４２例恶性淋巴瘤,进行分析,结果确诊为结外淋巴瘤４４例,占３０．９％。４４例中发生于鼻及咽环淋巴组织者最多,共１７例,其次为消化道,共１３例,其它部位少见,共有１４例。     

原发性结外淋巴瘤46例临床分析

恶性淋巴瘤多指原发于淋巴结内，原发性结外恶性淋巴瘤（Primaryentranodallymphoma；PENL）是指其原发于一个结外器官或组织，可伴有或不伴有区域淋巴结浸润。1980年至1996年我院共收治成人结外恶性淋巴瘤46例，现就其发病情况、临床表现、病理特点及诊断等进行探讨。临床资料本组男36例，女10例；年龄16岁一72岁，平均43．2岁。首发症状为腹痛16例，肿块11例，消化道出血7例，咽痛7例，鼻出血2例，腰痛1例，发热1例，皮肤损害1例。从发病至确诊时间，最短10天，最长2年，平均5．1月。原发部位；胃17例，肠14例，鼻咽部6例，扁桃体3例，…     

淋巴母细胞型淋巴瘤40例临床疗效及预后分析

目的：回顾性分析淋巴母细胞型淋巴瘤药物治疗为主的疗效和预后因素。方法：1978年4月至2001年7月间收治的淋巴母细胞型淋巴瘤共40例，以联合化疗为主，总结和分析临床疗效及预后因素。结果：全组完全缓解率47．5％，近期总有效率67．5％。分析显示外周血象正常者完全缓解率显著高于外周血白血病样变者，含蒽环类的诱导方案完全缓解率高于不含蒽环类的诱导方案。化疗后随访1-12年，失访4人，随访率90％。30例已相继死亡，按寿命表法统计全组1，2，3，4，5，6，8，12年生存率分别为54．4％，32．7％，27．2％，24．4％，24．4％，24．4％，19．5％，19．5％。COX回归多因素分析显示不良预后因素包括未达完全缓解，Ⅳ期病例，≥2个结外病变，骨髓侵犯，纵隔病变，中枢神经系统侵犯，外周血白血病样变者。结论：含蒽环类的方案能提高完全缓解率，而能否达到完全缓解，病情的早晚是影响预后的两个主要因素。以含蒽环类方案的足量化疗为主，达到完全缓解可能提高淋巴母细胞型淋巴瘤的长期生存率。     

原发性中枢神经系统淋巴瘤的临床与组织病理学特征

背景与目的：原发性中枢神经系统淋巴瘤是一种罕见的发生于中枢神经系统轴的非霍奇金淋巴瘤，病变局限于中枢神经系统内，不伴有其它系统的播散，近年来其发病率有逐年增高的趋势。本文探讨原发性中枢神经系统淋巴瘤的临床、影像学特点和组织病理学特征。方法：回顾性分析我院1997年1月至2002年10月间经手术及病理证实的35例原发性中枢神经系统淋巴瘤临床、影像学资料和组织病理学特征。35例组织病理标本中来源于手术切除的32例，经手术活检2例，立体定向活检1例。结果：本组病例男19例，女16例，男女比例为1．2：1，年龄26～72岁，平均年龄52岁。就诊时的临床症状主要表现为颅内压增高、肢体无力、瘫痪和神经精神症状，57％(20例)的患者病史短于4周，77％(27例)的患者病史短于8周。发病急，病情进展快。本组35例共52个肿瘤，其中16例(45．7％)为多发性肿瘤。肿瘤多位于额部(14例)、颞顶部(7例)和基底节区(5例)。29例(83％)患者的主体肿瘤位于小脑幕上。组织病理学检查发现B细胞来源34例，T细胞来源1例。结论：原发性中枢神经系统淋巴瘤是一组异质性肿瘤，侵袭性强，发病急，病史短，病情进展快。影像学特点为头颅CT平扫表现为等或高信号，注射造影剂后明显增强，肿瘤常发生于幕上大脑半球，易多发，极少发生出血、钙化或囊变。术前诊断困难，预后差。     

儿童间变性大细胞淋巴瘤19例临床研究

目的回顾性分析间变性大细胞淋巴瘤（ALCL）的临床特点、预后因素和治疗效果。方法对2003年1月至2007年2月间收治的19例ALCL患者的诊断和治疗情况进行回顾性分析。结果19例ALCL患者中,18例（94．74％）出现B组症状,14例（73．7％）早期出现结外侵犯。误诊率84．2％。化疗有效19例（100％）,6个疗程后完全缓解率95％,1年及2年的无病生存率均为88．2％。早期复发1例（5．3％）。结论ALCL患者B组症状多见,易早期出现结外侵犯,误诊率高。但对化疗敏感,治疗效果较好。     

NK/T细胞淋巴瘤

目的：了解和认识NK／T细胞淋巴瘤的临床病理和免疫学特点。方法：选用NK细胞特异性抗体CD56，T细胞特异性抗体CD3E，对117例T细胞淋巴瘤进行标记。结果：117例T细胞淋巴瘤中26例CD56（＋），CD3ε（＋），阳性表达率为22．2％，26例中结外淋巴瘤16例（61．5％），其中15例位于鼻部；淋巴结内10例（38．5％），26例中18例（69．2％）表现为细胞的多形性特点，12例（46．2％）有凝固性坏死。结论：NK／T细胞淋巴瘤免疫学特点是CD56与CD3ε共同表达，NK／T细胞淋巴瘤多发生在结外，以发生于鼻部者为多，少数发生在结内，肿瘤细胞的多形性和肿瘤组织的凝固性坏死是主要的病理学特点。     

鼻腔非霍奇金淋巴瘤的预后及影响因素

目的：探讨鼻腔非霍奇金淋巴瘤(NHL)的预后及影响因素。方法：1985年2月～1996年7月间共收治143鼻腔NHL采用放疗、化疗和放、化疗综合治疗。结果：76例治疗成功占53．1％(76／143)。远处结外器官受侵是治疗失败的主要原因，其中以皮肤皮下最多见，淋巴结受侵和局部失败少见。治疗失败和临床分期相关，I期局部和远处复发为29．7％(30／101)，中晚期局部和远处复发分别为87．5％(28／32)、90．0％(9／10)。T细胞淋巴瘤与B细胞淋巴瘤远处器官受侵分别为54．2％(45／83)、35．9％(14／39)。在病变超出鼻腔侵犯邻近器官的I期患者中，综合治疗组和单纯放疗组远处失败基本相似。Ⅱ期综合治疗患者远处器官受侵明显低于单纯放疗患者。结论：有效的化疗方案能减少中晚期NHL患者远处器官受侵，提高生存率。复发多在2年内发生，复发患者的再治效果差。     

Treatment outcome of front-line systemic chemotherapy for localized extranodal NK/T cell lymphoma in nasal and upper aerodigestive tract

We analysed the treatment outcome of localized extranodal NK/T cell lymphoma initially treated with CEOP-B chemotherapy based on the primary site of involvement (nasal cavity vs. upper aerodigestive tract) and treatment modality (chemotherapy vs. chemotherapy followed by radiotherapy. Forty-three patients newly diagnosed as extranodal NK/T cell lymphoma were analysed: 29 cases from nasal cavity/nasopharynx and 14 from upper aerodigestive tract. Twenty-six patients were treated with chemotherapy alone, while adjuvant radiotherapy was given to 17 patients. Overall response rate to front-line CEOP-B chemotherapy was 67.4% (29/43) and the complete remission (CR) rate was 44.2% (19/43). Median overall and disease-free survival was 26.87 months [95% confidence interval (CI) = 8.71 - 45.03] and 15.27 months (95% CI = 2.92 - 27.62). The responders (CR or partial response) to initial CEOP-B chemotherapy showed longer overall survival than non-responders (P = 0.0026). Local relapse was observed to be higher in the chemotherapy alone group compared to the chemoradiotherapy group. Adjuvant radiotherapy failed to improve survival; thus, the median disease-free survival of the chemotherapy and chemoradiotherapy groups was not different (P = 0.9101). There may be a tendency for better overall survival in group of upper aerodigestive tract lymphoma than the nasal cavity/nasopharynx group (P = 0.0643). However, front-line CEOP-B chemotherapy has a limited role and adjuvant radiotherapy failed to improve survival in localized extranodal NK/T cell lymphoma.     

原发性结外非霍奇金淋巴瘤127例临床特征及误诊分析

目的分析淋巴结外非霍奇金淋巴瘤(NHL)的临床表现特点及误诊原因。方法回顾性分析127例原发结外非霍奇金淋巴瘤的发病情况、原发部位分布情况、首发症状及误诊情况等。结果原发于结外的非霍奇金淋巴瘤12／例，占同期非霍奇金淋巴瘤53．81％(127／236)；原发部位前5位依次为：胃肠道21．26％(27／127)，鼻腔18．9％(24／127)，Waldeyer环14．96％(19／127)，脾脏12．6％(16／127)，皮肤6．3％(8／127)；结外奇非霍金淋巴瘤误诊率为69．29％(88／127)；结外非霍奇金淋巴瘤首发症状因原发部位不同而临床表现各异，无特异性。结论结外NHL发生率较高，其临床表现缺乏特异性，早期易误诊。     

Primary gastrointestinal non-Hodgkin's lymphoma in a population-based registry

In a population-based registry of 580 patients with non-Hodgkin's lymphoma (NHL) 54 patients had a primary gastric lymphoma, 42 an intestinal, 113 a primary extranodal lymphoma localised elsewhere than in the gastrointestinal tract and 371 a primary nodal NHL. Histological specimens were reviewed by a panel of pathologists and classified according to the Kiel classification and the International Working Formulation. The 4-year survival rates for primary gastric, intestinal, other extranodal and nodal NHL ranged from 50 to 60%; the 4-year recurrence-free survival rates were 50%, 35%, 19% and 19%, respectively. Among patients with localised intermediate-grade disease survival for those with gastric NHL was better than for those with intestinal lymphoma. Because it is population-based, our study cohort was not subjected to exclusion due to age, performance scale, etc. and therefore provides a more realistic picture of the occurrence and presentation of as well as prognosis for lymphoma in the population.     

The clinical study of extranodal natural killer cell lymphoma, nasal type

To analyze the clinical characteristics, treatment of extranodal NK/T-cell lymphoma, nasal type, the term "nasal type" describes in the nasal cavity and also in the extranasal sites. There were 82 patients with nasal NK/T lymphoma (group 1) and 11 patients with extranasal NK/T lymphoma (group 2). In group 1, 4 patients gave up treatment. Five patients received radiotherapy (RT) alone. Fifty-seven patients were treated with combination of chemotherapy and RT. Sixteen patients received chemotherapy alone. Most patients (82.9%) had stage I/II disease and a high frequency (about one-third) of B symptoms. The CR rate was 53.8%. The OS rate was 62.8% (49/78 cases). Three patients died in relation to L-asparaginase. Three patients with late relapses occurred at 10 and 17 years from CR, respectively. In group 2, except that one patient received chemoradiotherapy, 10 patients received chemotherapy. Seven patients died. The OS rate was 36.4%. Our study suggested that nasal and extranasal variants of extranodal NK/T lymphoma, nasal type represented different clinical behavior and prognosis. For comparison, extranasal NK/T lymphoma is more aggressive and higher mortality than nasal NK/T lymphoma.     

原发性结外淋巴瘤（附69例分析）

目的：探讨结外型恶性淋巴瘤的临床特点及其误诊情况。方法：对经病理确诊的恶性淋巴瘤２７２例进行回顾性分析。结果：误诊率为８４．１％,结外型淋巴瘤有６９例,占２５．４％,其中韦氏环、鼻腔占第一位,其次为腹腔、胃肠道,分别占２３．２％,１４．５％。其它部位少见。结论：结外型淋巴瘤易误诊,临床表现缺乏特异性,韦氏环、鼻腔最易受侵犯,病理检查是确诊的唯一方法。     

Prognostic Factors in Non-Hodgkin Lymphoma Stage I Treated with Radiotherapy

The results of treatment in 175 consecutive patients with non-Hodgkin lymphoma (NHL) clinical stage I treated between 1969 and 1984 were analysed according to different pretreatment prognostic variables. Treatment consisted of radiotherapy in 166 of the 175 patients. The estimated 5 and 10-year disease-free survival rates (DFS) were 63 % and 60% and the survival rates at 5 and 10 years 82% and 76% respectively. Lymphomas arising from gut-associated lymphoid tissue, i.e. Waldeyer's ring, the thyroid and the gastrointestinal tract had a more favourable clinical course (10-year projected DFS 83%) than nodal (50%) and other extranodal lymphomas. Although the number of patients with other extranodal sites was small, sites such as testis, nasal cavity, paranasal sinus and extradural space seemed to have a high relapse rate. Unfavourable clinical courses were also observed among nodal high-grade NHL if the lymph nodes were larger than 5 cm in diameter. Chemotherapy before radiotherapy may be recommended in NHL subgroups with a high relapse rate and which today are potentially curable with chemotherapy, i.e. high-grade NHL. This study indicates that large nodal lymphomas and some extranodal sites belong to this group.     

High endothelial venules in extranodal lymphomas with emphasis on their prognostic significance.

In the present study, the frequency of occurrence of high endothelial venules (HEVs) in extranodal lymphomas at various sites was examined, and its prognostic implication subsequently estimated. The presence of HEVs was tested for by an immunohistochemical method using the monoclonal antibody, HECA-452, known to be specific for HEVs. The number of cases at each extranodal site were 13 in the thyroid, three in the salivary gland, 15 in the gastrointestinal(GI) tract, 20 in the brain, six in the lung, 12 in the pleura, nine in the testis, 10 in the bone, 19 in the skin and seven in the breast. Histologically, 26 of the 114 cases (23%) were of high grade malignancy according to the Working Formulation. Immunohistochemistry revealed 89 cases (78%) to be of B-cell type, eight (7%) T-cell type and 17 (15%) others. HEVs were observed frequently among the cases of lymphoma of the salivary gland (100%), thyroid (77%), GI tract (53%), lung (50%) and breast (43%). Evident were a preponderance of female patients (P less than 0.02) and those with histologically favorable lymphoma types (P less than 0.001), and a more favorable prognosis (P less than 0.001) for cases with HEVs over those without. There were no significant differences in age and immunologic type distributions between cases with and without HEVs. The findings suggest that the presence of HEVs could be used as an indicator for a favorable prognosis in cases of extranodal lymphoma.     

Clinicopathologic features and prognostic factors of primary extranodal non-Hodgkin's lymphomas in Turkey

Clinical, histopathologic, and prognostic features of 114 patients with primary extranodal non-Hodgkin's lymphoma were evaluated. Median age of the patients was 48 (range, 15-76) and the ratio of male/female was 55/59. Thirty-seven patients had stage 1, 55 patients stage II, 6 patients stage III, and 16 patients stage IV. The most common sites of primary extranodal non-Hodgkin's lymphoma were the gastrointestinal (GI) tract and head-neck region. Stomach (66%) and tonsils (33%) were the most frequently involved organ in GI tract and head-neck region, respectively. Eighty percent of patients had intermediate or high-grade lymphomas, 20% had low-grade subtypes. Complete remission was achieved in 83% of all patients with chemotherapy +/- radiotherapy +/- surgery. Overall and disease-free survival at 5 years were 63% and 59%, respectively. In conclusion, clinical and histopathologic characteristics and prognosis of our cases with primary extranodal non-Hodgkin's lymphoma were usually similar to those of the cases in Western countries with some differences in the incidence of some specific primary extranodal non-Hodgkin's lymphomas and in the histopathologic subtypes.     

Non-Hodgkin's lymphoma confined to the nasal cavity: its relationship to the polymorphic reticulosis and results of radiation therapy

From 1975 through 1988, nine patients with locally confined nasal non-Hodgkin's lymphoma (NHL) were treated with radiation therapy in the Department of Radiology, Chiba University Hospital. Immunohistochemical study disclosed that all NHL's have T-lineage. Additionally, unique histological pictures of polymorphism, angiodestruction, and necrosis were seen in most of cases. These three findings are the histological features of polymorphic reticulosis (PMR), which is the main cause of lethal midline granuloma and has recently been shown to belong to T-cell malignancy. Therefore, it is concluded that the nasal T-cell NHL and PMR are really a single disease entity. The predominance of the T-cell lymphoma in the nasal cavity as well as its histological distinctness clearly indicate that the head and neck extranodal NHL cannot be discussed together. Although the disorder was considered to be locally limited at presentation, only 3 of the 9 patients with nasal NHL could be induced into long-term remission with involved field radiotherapy. The distant extranodal spread was the primary cause of failure. Multimodality treatment using intensive chemotherapy might improve the prognosis of nasal NHL.