间变性大细胞淋巴瘤

间变性大细胞淋巴瘤（ＡＬＣＬ）是形态学和免疫学上一种独特类型的非霍奇金淋巴瘤（ＮＨＬ）,它的发生与ｔ（２;５）（ｐ２３;ｑ３５）梁色体易位形成ＡＬＫ－ＮＰＭ融全基因有关。本文综述了ＡＬＣＬ在形态学、免疫学、遗传学和临床方面的特点及其诊断、治疗、预后。     

间变性淋巴瘤激酶阳性的间变性大细胞淋巴瘤研究进展

Stein于1985年首先报道间变性大细胞淋巴瘤(anaplas-tic large cell lymphoma,ALCL)是非霍奇金淋巴瘤(NHL)的一种独立类型[1]。最新的WHO分类中ALCL被归类于外周T细胞淋巴瘤,占同期NHL的2%~7%[2]。研究发现,约有半数患者产生致癌性的异常间变性淋巴瘤激酶(anaplasticlymphoma k     

间变性淋巴瘤激酶阳性和阴性间变性大细胞淋巴瘤分子遗传学研究

目的：研究间变性大细胞淋巴瘤（ALCL）的遗传学特征,探讨其发病机制,寻找有助于ALCL诊断、分类及预后评估的新分子靶标。方法收集ALCL病例石蜡包埋组织10例,其中4例间变性淋巴瘤激酶（ALK）阳性,6例ALK阴性。采用免疫组织化学染色及荧光原位杂交技术分别检测表型及2p23重排,利用OncoScan芯片在全基因组水平上扫描分析10例ALCL的拷贝数变异。结果10例ALCL均存在拷贝数变异,拷贝数获得者多于拷贝数缺失者。拷贝数获得主要累及17q11.2、Xp22.3、Xq28,拷贝数缺失主要累及3q26.1、14q11.2、22q11.23。 ALK阴性者拷贝数变异比ALK阳性者更为复杂：ALK阴性者拷贝数获得主要累及9q24.3-24.1、14q32.33,拷贝数缺失主要累及2p11.2、16p13.3,而ALK阳性者并无此变异。结论 ALCL存在复杂的遗传学不平衡,染色体片段获得多于缺失,ALK阴性ALCL遗传学不平衡更为复杂。 ALCL是异质性明显的一类肿瘤。     

间变性大细胞淋巴瘤

198 5年Stein等[1] 首次描述间变性大细胞淋巴瘤(anaplasticlargecelllymphoma ,ALCL) ,并确认为一种新的淋巴瘤类型。ALCL具有下列特征 :肿瘤细胞呈间变性 ,生长具黏结成团倾向 ,侵犯淋巴结窦 ,间变细胞CD30 (Ki 1)强阳性。ALCL也称之为Ki 1阳性大细胞淋巴瘤。虽然ALCL具有上述共同特征 ,但病理形态、基因表型和临床表现有广泛的异质性 (表 1)。B细胞来源ALCL在REAL和WHO分类中归于弥漫性大B细胞淋巴瘤[2 ,3 ] 。文中如未提到细胞来源 ,则特指T/null细胞ALCL。　　一、病理ALCL诊断性病理特点为淋巴结结构部分消失 ,肿瘤…     

间变性大细胞性淋巴瘤

目的　观察间变性大细胞性淋巴瘤的形态学特征 ,免疫表型和鉴别诊断。方法　用光镜、免疫组化对 14例病例进行观察。结果　肿瘤细胞有特征性的多形性的泡状核和明显的核仁 ,且大的瘤细胞呈Ki 1阳性 ,T、B细胞可表达或不表达 ,其中 1例ALK阳性。结论　间变性大细胞性淋巴瘤为非霍奇金氏淋巴瘤的特殊类型 ,需要与转移性癌、恶性黑色素瘤、霍奇金氏病等鉴别 ,应用免疫组化可确诊。     

间变性大细胞淋巴瘤26例

 目的　探讨间变性大细胞淋巴瘤（ALCL）的临床特点。方法　回顾性分析26例ALCL的发病情况、临床分期、病理及免疫分型等临床和治疗的相关资料。结果　发病年龄3.5～68岁,<18岁11例,中位年龄22岁。原发淋巴结21例,结外起病5例,结外侵犯18例,浅表淋巴结肿大23例,肝脾大8例,20例有B症状。Ⅰ期5例、Ⅱ期1例、Ⅲ期12例、Ⅳ期8例。T、B免疫分型检查23例,T细胞型17例,非T非B细胞型5例,T B双表型1例。CHOP方案化疗11例,缓解9例,缓解后复发2例,死亡4例,失访2例;4例接受化疗的儿童患者缓解3例,死亡1例。结论　ALCL以儿童及青少年患者多见,结外侵犯较多,T细胞型多,中晚期及有B症状比例高,完成化疗疗程者和儿童患者预后较好。     

间变性大细胞淋巴瘤1例

间变性大细胞淋巴瘤(ALCL)临床较少见,约占非霍奇金氏淋巴瘤的2%～7%犤1犦,可累及淋巴结、皮肤、胃肠道等处,我科偶见1例,现报告如下。患者,女,34岁,因腹胀、右下肢水肿3个月于2002年6月3日入院。查体:双侧锁骨上、腹股沟、腋下均可触及肿大的淋巴结,质中,无压痛,与皮肤无粘连,最大约3cm×4cm,右腹股沟肿大淋巴结活动差,双腋下淋巴结相互融合,右侧乳房皮肤轻度水肿,双下肺呼吸音稍低,腹部膨隆,移动性浊音阳性,右下肢重度凹陷性水肿。查血常规:WBC25.1×109/L,N0.799,L0.076,M0.125,胸部正侧位片:两侧胸腔积液(少量)、右肺部感染,不除外…     

间变性大细胞淋巴瘤临床病理分析

 目的　研究间变性大细胞淋巴瘤（ALCL）的临床病理特征、免疫表型，以提高诊断水平。方法　回顾性分析19例ALCL的临床、病理形态学资料和免疫组织化学，CD30、ALK、CD43、CD45RO、EMA和TIA-1检测结果。结果　19例均为原发系统型ALCL，其中男性11例，女性8例，年龄2～71岁，平均26岁。16例（84.2 %）出现B症状，14例（73.7 %）结外侵犯。肿瘤细胞沿淋巴窦生长或散在分布，均可见标志性肿瘤细胞，CD+30 19例（100 %），ALK（+）15例（78.9 %），EMA（+）10例（52.6 %），TIA-1（+）14例（73.7 %），ALK、EMA和TIA-1同时阴性2例（10.5 %）。重新诊断17例（89.5 %）正确。结论　ALCL表现多样，大部分患者经临床、组织病理学和免疫标记特征综合判断可作出相应诊断，CD30、ALK、EMA、TIA-1对鉴别诊断有帮助。     

间变性大细胞淋巴瘤1例报告

介绍诊治的1例间变性大细胞淋巴瘤的临床特征、形态学、免疫表型及遗传学特征,并复习相关文献.     

Primary lymphoma of the ocular adnexa (orbital lymphoma) and primary intraocular lymphoma

Lymphomas of the orbit and eye are rare conditions that should be treated as separate entities due to the differences in presumed aetiology, investigations, management and outcomes. Orbital lymphoma is most often of low-grade histology; thyroid eye disease may predispose and chlamydial infection has been suggested as a trigger. Commonly, stage IE, in most cases, can be managed with radiotherapy alone using either a kilovoltage portal for conjunctival disease or a wedged pair of megavoltage beams for more infiltrative disease to a dose of 30 Gy in 15 fractions over 3 weeks. However, medical therapy is being investigated, including a rituximab-only approach for conjunctival-only presentations. The cure rate for stage IE disease is very high. In contrast, primary ocular lymphoma is often of high-grade histology, in particular diffuse large B-cell lymphoma, and can be regarded as one end of primary central nervous system lymphoma - both eyes and brain being at risk. Immunosuppression predisposes to the disease. Management consists of an initial high-dose chemotherapy regimen with methotrexate. In most cases, this should be followed by radiotherapy to the whole brain and globes to a dose of 30-36 Gy with a boost to bulk/presenting disease. Cure rates are rarely above 50%.     

Tumorigenicity of T lymphoma/T lymphoma hybrids and T lymphoma/normal cell hybrids

Stable hybrids formed between clones of established murine T-cell lymphoma lines, and between lymphoma clones and normal spleen or thymus cells were examined for their tumorigenic properties by intravenous (i.v.) and intradermal (i.d.) inoculation into syngeneic AKR mice. Fusion parents consisted of T lymphoma clones of high and low tumorigenicity derived from the SL 12 cell line. In addition, normal spleen cells and thymocytes were fused with poorly tumorigenic T-lymphoma clones. Hybrids tested by i.v. inoculation of 10(6) cells to syngeneic hosts showed that fusion between the lymphoma cells resulted in hybrids which displayed the phenotype of the highly tumorigenic parent. Also, it was shown that fusion of poorly tumorigenic lymphoma cells with normal spleen cells resulted in hybrids with enhanced tumorigenicity. Fusion of poorly tumorigenic lymphoma cells with normal thymocytes resulted in hybrids with the highest tumorigenic potential. The pattern of spread for the tumor/tumor hybrid was that of the highly tumorigenic parent. Tumor spread patterns for the spleen/tumor hybrids were different from those of the thymocyte/tumor hybrids. Intradermal inoculation of 10(5) cells from tumor/spleen or tumor/thymocyte hybrids revealed differences in latent periods between parental and hybrid cells, the tumor/thymocyte hybrids having the shortest latent period. Surface marker studies and T-cell antigen receptor mRNA determinations in the tumor cell/normal cell hybrids indicated that the normal parent was a cell of immature phenotype. Therefore, high tumorigenicity is a dominant characteristic, and poorly tumorigenic but "immortal" T lymphoma cells can derive characteristics which increase their in vivo growth capacity from the putative immature normal cells with which they selectively fuse.     

介于弥漫大B 细胞淋巴瘤和伯基特淋巴瘤之间的未分类淋巴瘤

目的:分析介于弥漫大B 细胞淋巴瘤和伯基特淋巴瘤之间的未分类的B 细胞淋巴瘤（B-cell lymphoma ,unclassifiable,with features intermediate between DLBCL and Burkitt lymphoma,DLBCL/BL）的临床特点、治疗与预后,增加对该病的认识。方法:收集郑州大学第一附属医院2013年1 月至2014年12月收治的13例DLBCL/BL患者临床病理资料,采用Kaplan-Meier 法进行生存分析,采用Logrank 检验对临床分期、年龄、LDH 水平、IPI 评分、初治化疗方案等进行单因素分析。结果:13例患者中12例存在结外侵犯,13例患者的中位OS为10个月,中位PFS 为6 个月。单因素分析显示IPI 评分、LDH 水平与预后有统计学相关性,行CHOP、CHOP 样与高强度化疗方案患者之间生存差异具有统计学意义（P = 0.054）。 结论:DLBCL/BL恶性程度高,生存期短,结外侵犯多见,对CHOP 及CHOP 样方案治疗反应差,高强度化疗可能改善预后,IPI 评分≥ 3 分及 LDH 升高是其不良预后因素。      

Evaluation and management of the “New” lymphoma entities: Mantle cell lymphoma, lymphoma of mucosa-associated lymphoid tissue, anaplastic large-cell lymphoma, and primary mediastinal B-cell lymphoma

Non-Hodgkin's lymphomas (NHL) represent a major health problem worldwide, and incidence has been on the rise continuously for the last few decades. It is estimated that approximately 55,000 new cases of NHL will be diagnosed in the United States in 1998 and that slightly fewer than 25,000 patients will die of treatment failure or recurrent disease. The rising incidence of NHL is related not only to the acquired immunodeficiency syndrome epidemic but to also a steady increase in the number of cases diagnosed in older patients without immunosuppression. The new pathologic classification of NHL (revised European-American lymphoma classification, REAL) developed by the International Lymphoma Study Group (ILSG) is already resulting in more accurate disease-specific epidemiologic and clinical investigations. These studies have brought a new awareness of the existence and the relative prevalence of discrete NHL subtypes that appear to predominate among patients in different populations according to age, sex, geographic distribution, and predisposing conditions. This developing database has also the potential to result in the discovery of specific environmental causes, predisposing genetic factors, and therapeutic approaches. Some of the entities defined in the REAL classification, such as follicular lymphomas, diffuse B large-cell lymphomas, and T-cell lymphoblastic lymphomas, were already well described in the older classification systems (Kiel and Working Formulation). Others, such as mantle cell lymphoma, (MCL) anaplastic large-cell lymphoma (ALCL), lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), and primary mediastinal B-cell lymphoma (PMBCL) are relatively new members of the family, and accurate data on their clinicopathologic features and natural histories have only recently begun to emerge.This review presents in detail the most recent data on the clinical presentation of, diagnostic evaluation of, and treatment options for the most common of the new NHL entities: MCL, MALT lymphoma, CD30⁺ (Ki-1⁺) ALCL, and PMBCL. These four entities combined represent approximately 20% of all cases of NHL and exemplify well the broad clinicopathologic spectrum of NHL and the diagnostic and therapeutic challenges facing those who care for patients affected by these conditions.     

Immunodeficiencies and lymphoma

Primary immunodeficiencies (ID) lead to a high incidence of cancer, particularly of lymphomas. The risk excess varies according to the type of ID. Cellular IDs carry a very high risk, tumoral IDs have an intermediate risk while deficiencies of non-specific immunity bear no risk. Acquired IDs are also associated with an increased risk of lymphomas. The physiopathogeny of ID-associated lymphomas remains unknown, although several hypotheses have been proposed, including a defective immunological surveillance, repeated viral and antigenic stimulations and DNA repair anomalies.     

A clinicopathologic study of node-based, low-grade, peripheral T-cell lymphoma. Angioimmunoblastic lymphoma, T-zone lymphoma, and lymphoepithelioid lymphoma.

Postthymic (peripheral) T-cell malignancy shows marked diversity in histopathologic appearances as well as in clinical and prognostic aspects. Histologic findings and clinical behavior of 110 cases of the three specific types of low-grade, peripheral T-cell lymphomas, i.e., lymphoepithelioid (LeL), angioimmunoblastic (AILD), and T-zone (TzL) lymphomas, were studied. There were 74 men and 36 women (age range, 24 to 90 years; median, 58). Histologic study of LeL, AILD, and TzL showed prominent reactive features which are distinct from those of high-grade, T-cell lymphomas (pleomorphic/immunoblastic types). Corresponding to the differences in the histologic pictures of each type, there were differences in the clinical pictures and prognosis. Hypergammablobulinemia (greater than 4 g/dl) was more common in AILD than in the others. However, these three types exhibited a widely variegated, sometimes overlapping spectrum of histologic appearances, and it was extremely difficult to distinguish one from the other on several occasions. The same was true of their clinical and laboratory findings, and they had a relatively favorable prognosis as compared with pleomorphic/immunoblastic lymphomas. Although the conventional phenotypic analysis showed the prominent mixture of helper/inducer and cytotoxic/suppressor T-cells with a varying degree of B-cells and histiocytes, the double immunohistochemical study revealed that the neoplastic cells consisted predominantly of helper/inducer cells. Furthermore, five cases (5%) showed the morphologic transition among the three types or development into pleomorphic/immunoblastic lymphoma. They seemed to constitute a comprehensive and yet distinct group of T-cell lymphomas. Based on morphologic findings and clinical data, the authors demonstrated the distinct character of the node-based, low-grade, T-cell lymphomas and also the relationship among the three types in this group. The results of phenotypic and genotypic analyses also support the concept proposed here.     

自身免疫与淋巴瘤

 自身免疫和淋巴瘤之间有双重相关性,即自身免疫性疾病患者淋巴瘤发生率增高,同时淋巴瘤患者易合并自身免疫现象。现对自身免疫性疾病患者发生淋巴瘤和淋巴瘤患者合并自身免疫现象的流行病学情况、临床特点以及内在机制等方面的新进展进行综述。