生前诊断原发性心脏淋巴瘤一例体会

<正> 原发性心脏淋巴瘤是指仅累及心脏及心包的淋巴瘤,极其罕见,而根据大量尸检报告显示恶性淋巴瘤的患者中有20%～25%的患者均有心脏累及,但大多数均不表现心脏相关症状.原发性心脏淋巴瘤的临床症状虽大多与心脏相关,却缺乏特异性,生前诊断者很少,本文所报道的这例则系曾误诊为心肌病,后诊为原发性心脏非何杰金氏淋巴瘤的一例患者.     

北京协和医院对18年间病理诊断肺淋巴瘤患者的回顾分析

目的回顾性总结分析肺淋巴瘤的分类、分型特征及临床特点。方法收集北京协和医院1999年-2016年近18年期间病理诊断为肺淋巴瘤的全部病例,通过光镜、免疫组织化学染色及基因重排方法进行病理分析。结果本组共有肺淋巴瘤病例142例,男女比例1︰1.33,平均年龄48岁。123例(86.6%)诊断为非霍奇金淋巴瘤,19例(13.4%)诊断为霍奇金淋巴瘤。101例(69.2%)诊断为B细胞性淋巴瘤,其中45.6%(46例)为黏膜相关淋巴组织淋巴瘤或边缘区淋巴瘤,18.8%(19例)为弥漫大B细胞性淋巴瘤,11.9%(12例)为淋巴瘤样肉芽肿病。22例诊断为T细胞性淋巴瘤,其中27.3%为外周T细胞淋巴瘤(6例),13.6%(3例)为NK/T细胞淋巴瘤,9.1%(2例)为间变大细胞淋巴瘤。121例患者临床资料完善,其中64例(52.9%)为原发性肺淋巴瘤,男女比例1︰1.37,平均年龄50岁;57例(47.1%)为继发性肺淋巴瘤,男女比例1︰1.19,平均年龄45岁。在原发性肺淋巴瘤中,96.9%为非霍奇金淋巴瘤,3.1%为霍奇金淋巴瘤;在继发性肺淋巴瘤中,78.9%为非霍奇金淋巴瘤,21.1%为霍奇金淋巴瘤。65例手术切除或胸腔镜下肺活检病例全部进行了病理分类及分型,77例穿刺活检病例中有22例(28.6%)进一步分型困难。结论肺淋巴瘤中最常见类型依次为黏膜相关淋巴组织淋巴瘤、霍奇金淋巴瘤、弥漫大B细胞性淋巴瘤和淋巴瘤样肉芽肿病,其中黏膜相关淋巴组织淋巴瘤和淋巴瘤样肉芽肿病是主要的原发性肺淋巴瘤类型,而弥漫大B细胞性淋巴瘤和霍奇金淋巴瘤是主要的继发性肺淋巴瘤类型。开胸手术或胸腔镜下肺活检的病例易于明确病理分型,而穿刺活检病例中有小部分病例进一步分型困难。     

27例鼻部淋巴瘤的诊断

鼻腔鼻窦非霍奇金淋巴瘤在亚洲发病率较高，仅次于胃肠道淋巴瘤，位居结外淋巴瘤的第二位。鼻部淋巴瘤的诊断有一定难度。2003年7月-2008年5月，本院收治鼻部淋巴瘤患者27例。现分析如下，以探讨本病的诊断方法。     

原发皮肤T细胞淋巴瘤治疗的最新进展

原发皮肤T细胞淋巴瘤(PCTCL)是指原发于皮肤并以T淋巴细胞单克隆扩增为特征的一组非霍奇金淋巴瘤,为原发性皮肤淋巴瘤(PCL)中最常见的类型,约占PCL的75%。PCTCL诊断标准为:诊断皮肤T细胞淋巴瘤6个月以内未发现皮肤以外组织的淋巴瘤。在早期,PCTCL主要指蕈样霉菌病     

不一致性淋巴瘤1例及病理分析

本文回顾了南京大学附属金陵医院干部一科1例不一致性淋巴瘤高龄患者的诊治。患者于1987年以左侧腮腺区淋巴结肿大为首发症状,1988年行左侧腮腺区淋巴结活检诊断为非霍奇金淋巴瘤、小B细胞性淋巴瘤,先后进行了50个疗程的"COP、OP"方案化疗及短期局部放疗。2014年出现咽喉部不适,行会厌部取检,诊断为间变性淋巴瘤酶(ALK)阴性的间变性大细胞淋巴瘤,最终诊断为不同时发生的不一致性淋巴瘤。不一致性B细胞和T细胞性淋巴瘤非常罕见,取每一个病变部位作活检有助于避免漏诊,从而选择正确的治疗方案。     

21例原发性肠道非霍奇金淋巴瘤误诊分析

胃肠道是原发性非霍奇金淋巴瘤最常见的结外累及部位。原发性肠道非霍奇金淋巴瘤临床表现无特异性,内镜活检诊断率较低。目的：回顾性分析原发性肠道非霍奇金淋巴瘤误诊病例,以期提高其诊断准确率。方法：收集上海仁济医院2003年1月-2011年10月所有经手术病理证实、符合Dawson标准的原发性肠道非霍奇金淋巴瘤病例,复习误诊病例,分析可能的误诊原因。结果：共入组误诊病例21例,其中弥漫性大B细胞淋巴瘤10例,原发性肠道T细胞淋巴瘤7例,套细胞淋巴瘤3例,黏膜相关淋巴样组织（MALT）淋巴瘤1例,大多数患者在诊断过程中曾行影像学和内镜检查。弥漫性大B细胞淋巴瘤多误诊为阑尾炎或胃肠炎,原发性肠道T细胞淋巴瘤常误诊为克罗恩病,套细胞淋巴瘤均误诊为结肠息肉,此外尚有误诊为肠道血管炎、淋巴细胞性胃肠炎以及未能明确消化道出血原因者。结论：不同病理类型原发性肠道非霍奇金淋巴瘤在误诊为其他肠道疾病时表现各有其特点,了解这些特点可能有助于正确诊断原发性肠道非霍奇金淋巴瘤。     

软组织原发性非霍奇金淋巴瘤1例并文献分析

非霍奇金淋巴瘤是一组高度异质性的淋巴系统恶性肿瘤,主要发生在淋巴组织及富于淋巴组织的器官,发生于结外淋巴组织器官者为结外淋巴瘤,原发结外部位有胃肠道、皮肤、中枢神经系统、头颈部、肝脏等,而原发于软组织的非霍奇金淋巴瘤十分少见[1-4],Travis报道其发病率仅占淋巴瘤的0.11%[4],组织学类型以非霍奇金淋巴瘤为主[5].本文分析我院收治的1例软组织原发性非霍奇金淋巴瘤,探讨该病的临床病理特征、诊断及治疗等情况.     

非霍奇金淋巴瘤18例误诊分析

非霍奇金淋巴瘤（NHL）没有特异的临床症状和体征。早期诊断困难，容易误诊，我院曾收治的25例非霍奇金淋巴瘤的住院患者，其中误诊18例，误诊时间最长为7个月，现报道如下。     

心脏淋巴瘤

尸检证实，淋巴瘤可累及心脏及／或心包。有淋巴瘤心脏转移及原发性心脏淋巴瘤二类。本病缺乏特征性表现，多数表现为充血性心力衰竭症候群，伴或不伴心包缩窄症状，预后差，病死率高，生前难以确诊。１病理与临床１．１淋巴瘤转移心脏：恶性肿瘤可伴有心脏转移，其中淋巴...     

104例非霍奇金淋巴瘤诊断回顾分析

目的 总结现今非霍奇金淋巴瘤确诊途径和方法及病理类型.方法 回顾我院1995～2006年104例非霍奇金淋巴瘤诊断方法和临床主要症状,并对其病理诊断类型加以总结.结果 104例淋巴瘤患者结内发病62例,其中颈部,锁骨上淋巴结确诊50例.结外发病42例,其中皮疹和皮下结节活检确诊9例,脾切除确诊9例,胃镜活检确诊6例,骨髓形态学诊断6例.病理分型弥漫大B细胞淋巴瘤占32例,外周T细胞淋巴瘤15例,滤泡型12例,MALT 8例.结论 颈部和锁骨上淋巴结活检是确诊淋巴瘤的主要途径,但由于淋巴瘤的结外发病,使其诊断相当困难,组织活检是确诊的金指标.病理类型诊断非常重要,其中弥漫大B细胞淋巴瘤是NHL的主要类型.     

Germinal center cell lymphomas: Prognostic significance of their histopathological differentiation

Summary Clinical data of 48 patients with centrocytic, 83 patients with centroblastic/centrocytic and 64 patients with centroblastic lymphoma who had entered a prospective multicenter study of the Kiel Lymphoma Study Group since October 1975 were compared. Advanced (stage IV) disease at time of diagnosis, predominantly due to bone marrow infiltration, was most frequent in centrocytic (69% of patients) and in centroblastic/centrocytic (51% of patients) lymphomas as compared to only 28% of patients with centroblastic lymphoma. High survival probability of patients with localized centrocytic and centroblastic/centrocytic lymphomas after radiotherapy, contrasting with a worse prognosis of corresponding patients with centroblastic lymphoma, is compatible with the classification of these lymphoma entities as neoplasias of low-grade malignancy. However, as shown by this prospective and previous retrospective trials overall survival probability of patients with advanced centrocytic lymphoma was inferior to that observed in corresponding patients with centroblastic/centrocytic lymphoma. These findings suggest the possibility that patients with advanced centrocytic lymphoma occupy an intermediate position between typical low-grade and typical high-grade malignant non-Hodgkin lymphomas.Supported by the Deutsche Krebshilfe e. V., BonnPresented in part at the 5th Meeting of the International Society of Haematology, European and African Division, Hamburg, August 26–31, 1979     

Primary gastric non-Hodgkin's lymphoma: a clinicopathological study of 41 patients

Pathological findings in 41 patients (male/female ratio: 1.3/1) with primary localized gastric non-Hodgkin's lymphoma (NHL) were retrospectively studied and correlated with survival. The median observation period after diagnosis was 32 (0–189) months. Nineteen patients were low-grade NHL, all but one B-cell lymphomas of the mucosa-associated lymphoid tissue (MALT) type. Twenty-two patients had primary (n-7) or secondary (n=15) high-grade lymphomas; Musshoff stage IE was found in 29 and II E in 12 cases. The median age at diagnosis was 61 years (range, 26–88 years), and proliferation, measured by the number of mitosis and Ki-67 antigen positivity (MIB-1), was high or moderately high in 24 cases and low in 17 cases. Follicular lymphatic hyperplasia could be found in 25 of 34 evaluable cases, more often in low-grade than in high-grade NHL. Most of the patients were treated by resective surgery and additional ratio- or chemotherapy. Thirteen patients (31%) died (median survival: 10 months), 5 of them within 3 months after surgery owing to postoperative complications. Survival was superior, though not statistically significant, in low-grade lymphomas. Our retrospective anlysis of heterogeneously treated gastric lymphomas reveals that gastric lymphomas, especially of the low-grade MALT type, often remain a localized disease with a good long-term prognosis. Our study confirms previous reports indicating that lymphomas of the MALT type represent a specific clinicopathological entity.     

胃原发性肠病相关型T细胞淋巴瘤1例报道

本文报道1例胃原发性肠病相关型T细胞淋巴瘤。患者为1例22岁年轻女性,初始临床分期为Ⅳ期,首先采取了CHOP、GDP、HD-MTX、IEO、DHAP方案序贯化疗,然后行远端胃切除术与淋巴结清扫术,为获得较长的生存期还进行了局部姑息性放疗。治疗取得了良好的疗效,患者的生存期已超过2年,并且生活质量较高。本病例强调了在高侵袭性结外淋巴瘤的治疗中外科手段的重要性。     

Clinical and prognostic heterogeneity of non-hodgkin lymphomas of high-grade malignancy

Summary Comparison of clinical data of 64 patients with centroblastic lymphoma, 55 patients with immunoblastic lymphoma and 31 patients with lymphoblastic lymphoma not only confirmed the original assumption of high-grade malignancy as proposed by the concept of the Kiel classification but also demonstrated distinct clinical differences, particularly between lymphoblastic lymphoma and the two other entities. Rapid lymph node enlargement as well as steep fall of survival curves within the first year after diagnosis were common characteristics. Bimodal age distribution, predominance of males and early generalization of disease were typical features of lymphoblastic lymphoma; elderly patients and patients with the unclassified subtypes of lymphoblastic lymphoma exhibited the worst prognosis. Whereas patients with centroblastic and immunoblastic lymphomas showed similar distribution of age, sex and initial stage of disease, patients with immunoblastic lymphoma presented more frequently with a reduced performance status and showed a poorer response to radio- and chemotherapy resulting in a worse prognosis discernible after the first year of follow-up. Generalization during course of the disease was significantly more frequent in immunoblastic than in centroblastic lymphoma.Supported by the Deutsche Krebshilfe e. V., BonnPresented in part at the 5th Meeting of the International Society of Haematology, European and African Division, Hamburg, August 26–31, 1979     

How we diagnose and treat vitreoretinal lymphoma

The eye is a rare site for the development of malignant lymphoma. Based on cell type and involved intraocular structures, which as a whole represent an immune‐privileged site, several subtypes of primary intraocular lymphoma need to be discerned. Primary vitreoretinal lymphoma (PVRL), the most common form, is an aggressive B‐cell malignancy and considered a subtype of primary central nervous system (CNS) lymphoma. Ocular symptoms are non‐specific and often mimic uveitis, frequently resulting in delayed diagnosis. Bilateral ocular involvement and dissemination/relapse in the CNS are common. Diagnosis of PVRL is usually based on the analysis of vitreous biopsy material. In addition to cytological and immunocytochemical examination, measurements of cytokine levels and molecular determination of B‐cell clonality and recurrent mutations increase the diagnostic yield. Both systemic chemotherapy and exclusively local treatment, including ocular radiotherapy and intravitreal chemotherapy, are successful approaches for the management of PVRL, although it is currently not predictable which patients require systemic treatment in order to avoid cerebral dissemination, a complication associated with a considerably worse prognosis.     

Composite and sequential lymphoma between classical Hodgkin lymphoma and primary mediastinal lymphoma/diffuse large B-cell lymphoma,a clinico-pathological series of 25 cases

Composite and sequential lymphomas involving both classical Hodgkin lymphoma (CHL) and primary mediastinal B-cell lymphoma (PMBCL) are rare phenomena. Beyond the relevant biological interest raised by these cases, treatments and outcome data are poorly covered in the recent literature. This retrospective analysis describes the pathological and clinical characteristics of 10 composite and 15 sequential cases included after a central pathological review. At diagnosis, 70% of the composite lymphomas presented a disseminated and extranodal disease. Among the 15 sequential lymphomas, 12 were CHL at first occurrence and three were PMBCL. Based on their clinical evolution, these sequential lymphomas could be divided into early (i.e., diagnosis of second lymphoma within a year) and late [(i.e., a second lymphoma occurrence occurring after a long period of complete remission]). All composite cases were alive in complete remission after a median follow-up of 34 months. If the early sequential lymphoma presented a particularly poor outcome with a median overall survival shorter than one year, the late cases were efficiently salvaged. Further molecular studies are needed to describe the underlying biology of these rare diseases, possibly representing the extreme of tumour cell plasticity found in grey-zone lymphoma.     

Vitreoretinal lymphoma occurring after systemic chemotherapy for primary conjunctival diffuse large B cell lymphoma: A case report

Introduction:Ocular adnexal lymphoma and vitreoretinal lymphoma are rare forms of non-Hodgkin lymphoma. They are regarded as distinct disease entities due to the differences in molecular mechanism, management, and outcome. We present a rare case of conjunctival diffuse large B cell lymphoma (DLBCL) that developed to vitreoretinal lymphoma after systemic chemotherapy.Patient concerns:A 60-year-old man presented with a left salmon-colored conjunctival mass.Diagnosis:A biopsy was performed, and histopathologic examination showed DLBCL. Immunohistochemical staining was positive for CD20 with increased κ to λ light chain ratio.Interventions:Bone marrow biopsy also revealed DLBCL. Gallium-67 scintigraphy showed abnormal uptake only in the left orbital lesion. Ann Arbor stage was estimated as IV. The patient underwent systemic combination chemotherapy and immunotherapy.Outcomes:Four months after the last course of chemotherapy, primary conjunctival DLBCL relapsed, manifesting vitreous opacity. Diagnostic vitrectomy confirmed a diagnosis of vitreoretinal lymphoma.Lessons:Conjunctival DLBCL and vitreoretinal lymphoma are both DLBCL. After systemic chemotherapy for conjunctival DLBCL, the lymphoma may relapse in intraocular sites as secondary vitreoretinal lymphoma.     

Diagnosis and management of follicular lymphoma: A comprehensive review

Follicular Lymphoma (FL) is an indolent lymphoma and may have various clinical courses. Worldwide, FL is the second most common non‐Hodgkin lymphoma (NHL) type after diffuse large B‐cell lymphoma. In this review article, the author is discussing relevant diagnostic tools, prognostic factors, and updated study results on the management of patients with newly diagnosed and relapsed/refractory FL. Controversies in the treatment, maintenance therapy, stem cell transplantation, and novel treatment approaches will be comprehensively discussed.