系统型间变性大细胞淋巴瘤30例临床分析

 目的　探讨系统型间变性大细胞淋巴瘤（S-ALCL）的临床特征和预后相关因素。方法　回顾性分析30例S-ALCL患者的临床资料。30例患者均以联合化疗为主,配合局部病灶野放疗8例。化疗方案主要为 CHOP、EPOCH、Hyper-CVAD,以CHOP方案为主。结果　30例S-ALCL患者中位年龄36岁,男女比例为1.5∶1,有B症状、Ⅲ～Ⅳ期和结外侵犯者分别占60.0 %（18／30）、73.3 %（22／30）和60.0 %（18／30）;乳酸脱氢酶（LDH）升高者占46.7 %（14／30）;间变性大细胞淋巴瘤激酶（ALK）+ 18例（60.0 %）,其发病年龄小于ALK- 者（u＝3.92,P＝0.001）。单因素分析显示ALK-及LDH升高是重要的预后不良因素。结论　S-ALCL患者发病年龄较轻,预后较好。但ALK-、LDH升高者预后不良。治疗以联合化疗为主,对于有不良预后因素的患者,大剂量治疗可能获益。     

原发性皮肤间变性大细胞淋巴瘤复发1例及文献复习

目的:探讨原发性皮肤间变性大细胞淋巴瘤(C-ALCL)的临床及组织病理学特征.方法:通过对1 例复发C-ALCL患者的皮损行组织病理学及免疫组化检查,并进行文献复习,观察C-ALCL的组织学特征及免疫表型的特点.结果:该例为复发病例,两次均表现为皮肤结节;镜下肿瘤细胞异型性明显,体积较大;大细胞均表达CD30,部分大细胞表达EMA,不表达ALK-1;用CHOP方案治疗,患者预后较好.结论:C-ALCL是一种少见的原发于皮肤的淋巴瘤,肿瘤细胞表达CD30,EMA可阳性,一般不表达ALK-1,预后较好.     

弥漫大B细胞淋巴瘤survivin表达与临床预后关系探讨

目的:探讨弥漫大B细胞淋巴瘤survivin表达与临床表现的关系.方法:收集本院自1997年至1999年的初治弥漫大B细胞淋巴瘤共63例,均接受治疗并进行随访.用免疫组化SP法检测其survivin表达情况.应用SPSS10.0软件行生存分析并对各临床指标与预后的关系进行单因素和多因素分析.结果:63例弥漫大B细胞淋巴瘤病例中有43例survivin表达阳性,阳性率为68.3%,其表达与性别、年龄、分期、结外病灶数目无明显相关关系,与PS、LDH、B症状、IPI显著相关(P<0.05).其5年总生存率为50.04%.survivin表达阳性的弥漫大B淋巴瘤患者的总生存率明显低于survivin表达阴性患者(P=0.000 1),二者的5年生存率分别为30.36%和90%.多因素分析显示,survivin表达是弥漫大B细胞淋巴瘤的独立预后指标.结论:survivin是弥漫大B细胞淋巴瘤的一个极有价值的预后指标,与IPI结合可于早期筛选出常规治疗预后不良的病例,有助于指导治疗及改善预后.     

原发系统型间变性大细胞淋巴瘤56例临床特征和预后相关因素分析

目的分析原发系统型间变性大细胞淋巴瘤（S-ALCL）的临床特点和预后相关因素。方法回顾性分析北京大学医学部病理学系淋巴瘤研究室确诊的56例S-ALCL的临床资料,采用免疫组织化学SP法检测间变淋巴瘤激酶（ALK）和bel-2蛋白的表达情况。结果56例S-ALCL患者中,中位年龄17岁,男女比例为1．67：1。预后分析可追访病例49例,死亡16例（32．65％）,均在2年内死亡,3年和5年生存率均为64．28％。56例患者均进行了ALK和bcl-2的检测,其阳性率分别为73．21％和17．86％。单因素预后分析显示不同临床分期、是否伴有或结外发病和ALK是否阳性对患者总体生存率的影响差异有统计学意义。临床分期是影响患者长期生存的独立预后因素。结论S-ALCL以40岁以下中青年男性发病为主,发病后第1年为死亡高发时段,对化疗敏感患者多数能达到完全缓解并获得长期生存。临床分期、伴有或结外发病、ALK对预测患者长期生存和指导治疗有重要意义。     

MYC/BCL2双表达大B细胞淋巴瘤中PD-L1的表达及其临床意义CSCD

目的探讨MYC/BCL2双表达大B细胞淋巴瘤(DEL)与程序性细胞死亡受体-配体1(PD-L1)mRNA、蛋白表达的相关性及其临床意义。方法收集90例弥漫大B细胞淋巴瘤(DLBCL)病例,采用免疫组织化学染色检测MYC、BCL2蛋白并分组,双标记染色法检测各组病例的肿瘤细胞或微环境细胞中PD-L1表达;实时荧光PCR技术(Real-time PCR,qPCR)检测DEL组与non-DEL组PD-L1 mRNA相对表达量;收集临床病理资料并随访,对实验数据进行统计学分析。结果90例样本中28例为DEL,肿瘤细胞和微环境中PD-L1+分别为22例和26例。DEL组肿瘤细胞和微环境中PD-L1+分别为14例和9例;肿瘤细胞和微环境细胞PD-L1蛋白表达与DEL存在相关性(P<0.05);PD-L1 mRNA相对表达量在DEL与non-DEL组间存在显著差异(P=0.012);DEL组中PD-L1+与IPI评分和B症状的出现有关(P=0.007、0.021);Kaplan-Meier显示DEL中PD-L1+、肿瘤微环境PD-L1阳性(mPD-L1+)与患者预后相关(P=0.005、0.001)。结论DEL患者PD-L1 mRNA及蛋白表达都明显上调且与患者不良预后相关,PD-L1可作为DEL患者不良预后评估的危险因素。     

儿童间变性大细胞淋巴瘤临床及病理特征

目的:探讨儿童间变性大细胞淋巴瘤的临床表现、组织病理及免疫组化的特点。方法:对2001年—2006年收集的4例儿童间变性大细胞淋巴瘤进行临床资料分析、病理形态学观察及免疫表型检测。结果:4例患者临床表现主要为浅表淋巴结肿大,发病迅速且伴不规则发热及肝脾肿大;组织学特征是淋巴结结构破坏,上皮样大细胞浸润淋巴窦,瘤细胞较大,细胞核偏位,形态多样,类似马蹄形、肾形。患者的肿瘤细胞均表达ALK-1、EMA和K i-1。结论:对儿童间变性大细胞淋巴瘤中ALK-1、K i-1、EMA的表达情况检测有助于诊断和鉴别诊断。     

原发系统性间变性大细胞淋巴瘤临床病理特征及免疫表型分析

 目的　分析原发系统性间变性大细胞淋巴瘤（ALCL）的临床病理特征和免疫组织化学特点,提高诊治水平。方法　选取22例ALCL患者,均进行分期、国际预后指数（IPI）、乳酸脱氢酶（LDH）检测,应用免疫组织化学SP法检测间变性淋巴瘤激酶（ALK）、Ki-67、Caspase-3、CD30、EMA、Granzyme B等,回顾性分析患者临床、病理形态学资料、免疫表型及生物学特性,并进行预后分析。结果　22例均为原发系统性ALCL,ALK+ 15例（68.2 %）,ALK- 7例（31.8 %）;ALK+患者发病年龄、Ki-67增殖指数较ALK-患者低,Caspase-3表达率高,差异有统计学意义（χ2＝4.618,P＝0.032）;15例ALK+ALCL均表达CD30和EMA。ALCL中ALK的表达与Ki-67、Caspase-3的表达呈负相关（r＝-0.581,P＝0.006;r＝0.458,P＝0.032）。ALK+病例较ALK-病例Granzyme B（χ2＝0.11,P＝0.74）、 TIA-1（χ2＝0.01,P＝0.92）的表达率高,但差异无统计学意义（P＞0.05）。有效率为 54.5 ％（12／22）,其中完全缓解率为18.2 %（4／22）;全组中位生存期12个月,1年生存率为59.1 %（13／22）,2年生存率为50.0 ％（11／22）。Ann Arbor分期、LDH及IPI与疾病预后相关。结论　ALK+较ALK-ALCL患者核增殖低,恶性程度低,临床特征和免疫表型具有一定的特征性;ALK、Ki-67、Caspase-3、分期、血清LDH及IPI对预测ALCL患者的生存和指导治疗有帮助。     

Bcl-6和CD10蛋白在弥漫大B细胞淋巴瘤中的表达及临床意义

目的 探讨Bcl-6和CD10蛋白在弥漫大B细胞淋巴瘤(DLBCL)中的表达及临床意义.方法 应用免疫组织化学方法 检测62例弥漫大B细胞淋巴瘤标本及20例淋巴结反应性增生(RLH)石蜡组织中Bcl-6、CD10蛋白的表达,结合随访资料进行临床预后分析.结果 Bcl-6、CD10在弥漫大B细胞淋巴瘤中均有表达,阳性率分别为82.26%(51/62)、38.71%(24/62),显著低于淋巴结反应性增生中的阳性率100%(52/52)、100%(52/52),差别有显著性(P<0.05);Bcl-6的表达与生存期相关,生存期大于5年组中Bcl-6的阳性率为87.50%(21/24),明显高于生存期小于5年组中Bcl-6阳性率50.00%(19/38)(P<0.05).Bcl-6的表达与弥漫大B细胞淋巴瘤患者的临床分期、B症状、结外浸润、治疗结果 差别无统计学意义(P>0.05);CD10的表达与弥漫大B细胞淋巴瘤患者的临床分期相关,临床分期越低,CD10阳性表达越高.生存期大于5年组中CD10的阳性率为75.00%(18/24),显著高于生存期小于5年组中CD10阳性率15.79%(6/38)(P<0.05).CD10的表达与弥漫大B细胞淋巴瘤患者的B症状、结外浸润、治疗结果 差别无统计学意义(P>0.05);CD10阳性表达者大部分同时表达Bcl-6,但在部分CD10表达阴性的病例中,Bcl-6也呈阳性表达,Bcl-6、CD10共表达组比非共表达组临床分期低,生存率高于非共表达组.结论 Bcl-6、CD10在弥漫大B细胞淋巴瘤中均与生存期成正相关,是预后良好的指标,二者联合检测有助于判断患者的预后和指导临床治疗.     

恶性组织细胞增生症瘤细胞属性及临床病理分析

目的:探讨恶性组织细胞增生症瘤细胞的属性,EB病毒感染的情况及其临床病理特点.方法:收集33例以往诊断为恶组的尸检病例及相关临床资料,并利用组织芯片技术将不同组织集成在一张切片上.免疫组化采用LsAB法.一抗选用CD4、CD8、CD20、CD45RO、CD56、CD3ε、CD30、CD68、TIA-1及Granzyme B.DNA-RNA原位杂交检测EB病毒编码的小分子mRNA.结果:33例恶组中,排除不符合传统恶组诊断标准的4例,选择其中29例作为研究对象.检测结果示29例中有28例属外周T细胞淋巴瘤,其中皮下脂膜炎样T细胞淋巴瘤3例、肠道T细胞淋巴瘤1例,间变性大细胞淋巴瘤1例;16/28例为EBER1/2阳性;余1例为恶组样B细胞性血管内淋巴瘤.结论:恶性组织细胞增生症是一组异质性淋巴细胞增生性疾病.绝大多数瘤细胞来源于细胞毒性,T细胞或NK细胞(28/29),少数来源于B淋巴细胞(1/29),未发现组织细胞源性的肿瘤.     

鼻腔NK/T细胞淋巴瘤中p53相关蛋白和潜伏膜蛋白1的表达及其临床意义

目的 探讨鼠双微体2(mdm2)、p53、p21基因和潜伏膜蛋白1(LMP-1)在鼻腔NK/T细胞淋巴瘤(NKTL)中的表达及相互作用关系,以及其与NKTL临床分期和预后的关系.方法 收集62例NKTL患者的临床病理资料,并进行随访.取62例NKTL组织构建组织芯片,采用免疫组化SP法检测mdm2、pS3、p21及LMP-1蛋白的表达情况;应用原位杂交法检测EBER1/2的表达.结果 mdm2、p53、p21和LMP-1蛋白在NKTL中的阳性表达率分别为61.3%、79.0%、58.1%和48.4%,EBER1/2的阳性表达率为90.3%.随着肿瘤临床分期的进展,mdm2、p53和p21蛋白的阳性表达率逐渐升高,并与临床分期相关(均P<0.05).mdm2、p53和p21蛋白的表达呈正相关(P<0.05),其阴性表达组患者的预后均好于阳性表达组(均P<0.05).LMP-1蛋白的表达与临床分期和预后无关(P>0.05).p53蛋白的表达水平是NKTL的独立预后因素.结论 mdm2、p53、p21的蛋白表达与NKTL的发生和发展密切相关,可作为评估NKTL生物学行为的良好指标.p53蛋白的表达水平是NKTL的独立预后因素.     

Cytotoxic activity of T- and NK-cell lymphoma cells is not dependent on a mature cytotoxic phenotype

Cytotoxic T- and NK-cell neoplasms constitute a rare clinico-pathological entity associated with aggressive clinical behaviour and a poor prognosis. The entity comprises a heterogenous group of different diseases classified by histologic, immunologic as well as clinical features. Recently, expression patterns of "cytotoxicity-associated proteins" such as T-cell intracellular antigen (TIA), perforin and granzyme B have been applied to differentiate between an immature (TIA positive) and a mature (TIA and perforin and/or granzyme B positive) phenotype of these malignant cells. In particular, expression of perforin and granzyme B are considered to mediate cytotoxic activity. This study assesses histology/cytology, immunophenotype, expression of "cytotoxicity-associated proteins" and the actual exhibition of cytotoxic activity of lymphoma cells of 10 patients suffering from different T- and NK-cell neoplasms. As investigated by PKH67 labelling of the target cells 6 out of 10 samples exhibited cytotoxic activity. Thus, all samples of lymphoma cells with a mature phenotype exhibited cytotoxic activity. Nevertheless, the ability to induce cytotoxic cell lysis was neither restricted to mature lymphoma cells, nor to lymphoma cells expressing "cytotoxicity-associated proteins": two samples with an immature phenotype and one CD4 positive sample, completely lacking expression of "cytotoxic proteins" as well as NK cell-associated markers, destroyed target cells. Artificial activation of a mature cytotoxic phenotype by cell culture conditions or contact of lymphoma cells with target cells was excluded by demonstrating the absence of perforin expression after the incubation period in two exemplary cases. In conclusion, we demonstrate that the exhibition of cytotoxic activity is neither restricted to cells with a mature phenotype, nor does it depend on the expression of the "cytotoxicity-associated proteins" TIA, perforin or granzyme B.     

The significance of granzyme B expression in patients with angiocentric lymphoma of the head and neck.

BACKGROUND: The objectives of this study were to establish a correlation between granzyme B expression and the clinicopathologic characteristics of patients with angiocentric lymphomas of the head and neck and to determine whether the expression of granzyme B had any influence on the treatment outcomes of such patients. METHODS: Fifty-seven patients with angiocentric lymphoma of the head and neck who were treated between 1987 and 1996 were divided into two groups according to whether their tumors were immunoreactive for granzyme B: the granzyme B negative group (n = 22 patients) and the granzyme B positive group (n = 35 patients). The clinicopathologic features, immunohistochemical findings, patterns of disease failure, and survival data for the granzyme B positive group were compared with those for the granzyme B negative group. RESULTS: Greater than 60% of patients with angiocentric lymphoma of the head and neck were shown to have granzyme B positive tumors. All tumors that expressed granzyme B also consistently coexpressed CD56, indicating that they probably are the neoplastic equivalent of either natural killer (NK) cells or activated cytotoxic T cells. Although there were no significant differences in histopathologic features or expression of CD45RO and polyclonal CD3-epsilon between the groups, the Epstein-Barr virus genomes were detected more frequently in the granzyme B positive group compared with the granzyme B negative group. Despite a similar rate of complete remission after initial treatment, the locoregional recurrence rate of patients in the granzyme B positive group was much higher compared with patients in the granzyme B negative group. In addition, compared with patients in the granzyme B negative group, patients in the granzyme B positive group also had an increased risk of systemic disease recurrence and a decreased overall survival rate. CONCLUSIONS: The data indicate that the cytotoxic granule-associated protein, granzyme B, may be used as an additional marker for identifying NK/T-cell lymphoma and as a prognostic indicator for risk assessment in patients with angiocentric lymphoma of the head and neck.     

Anaplastic Lymphoma Kinase (ALK) is not Expressed in Hodgkin's Disease: Results with ALK-11 Antibody in 327 Untreated Patients

The t(2;5)(p23;q35) or other rare chromosomal abnormalities involving 2p23 upregulate the ALK gene, which is not expressed in normal lymphocytes. Thus, detection of ALK protein is presumptive evidence of these 2p23 abnormalities. The t(2;S) and ALK immunoreactivity are common in anaplastic large cell lymphoma of T/null-cell lineage. However, a small subset of cases of Hodgkin's disease (HD) have been reported to either carry the t(2;5) or express ALK. In this study, we have immunohistochemically evaluated 327 cases of HD with the ALK-11 antibody. ALK-11 is a well characterized polyclonal antibody raised against an intracellular portion of the ALK protein. We detected ALK-11 immunoreactivity in 8 (2.4%) cases of HD. We further studied these positive cases with ALK-1 monoclonal antibody, which reacts with an intracellular portion of ALK, similar to ALK-11. All 8 ALK-11 positive cases were negative for ALK-1. These results indicate that rare cases of HD may react with ALK-11 antibody, similar to previous reports by others using different polyclonal anti-ALK antibodies. However, the absence of ALK-1 expression in these HD cases suggests that ALK protein is not truly present and that polyclonal anti-ALK antibodies may rarely yield non-specific cross reactivity. These results further support the use of anti-ALK antibodies in the differential diagnosis of HD from ALCL.     

Nasopharyngeal/nasal type T/NK lymphomas: analysis of 14 cases and review of the literature

AIMS AND BACKGROUND: Lymphoid malignancies expressing CD56 are rare and most occur in the nasal or nasopharyngeal region. They derive from natural killer cells or from a small subset of T cells that have granular cytoplasm containing molecules that mediate cytotoxic activity: TIA-1, granzyme B and perforin. Both types are closely associated with Epstein-Barr virus. METHODS: We report the pathologic, immunophenotypic and molecular findings in 14 cases of nasopharyngeal/nasal type T/NK lymphomas. RESULTS: Clinically, all patients had localized disease and also had symptoms limited to the nose. The neoplastic cells were frequently pleomorphic, and angiocentric growth was common. Combined immunophenotypic and gene rearrangement analyses demonstrated that most of the cases were true NK cell tumors and were either CD56+ and CD3- or CD56+ and CD3+. Immunohistochemical study showed TIA-1 and granzyme B expression in all cases. By in situ hybridization, most of the cases were associated to Epstein-Barr virus, harboring type 1 virus, and polymerase chain reaction amplification across the 30 bp deletion showed high frequency of latent membrane protein-1-deleted variants. CONCLUSIONS: The nasal type T/NK cell lymphoma shows distinctive clinicopathologic, immunophenotypic and molecular features. These results confirm the important role of Epstein-Barr virus as a local factor in their pathogenesis.     

Gastrointestinal T cell lymphoma: predominant cytotoxic phenotypes, including alpha/beta, gamma/delta T cell and natural killer cells

Gastrointestinal T cell lymphoma (TCL) is a rare subset of peripheral TCL, presenting with or without cytotoxic phenotype, a history of coeliac disease (CD) and enteropathy. However, CD is rare in Japan. Here, we describe the clinicopathological features of 18 Japanese cases. Lesions were found in the small intestine (n=13), stomach (n=3) and colon (n=2). Seven patients presented with enteropathy but none had a history of CD. Lymphomas appeared as ulceration (n=11), tumour formation (n=6), or polypoid growth (n=1). Histologically (REAL classification), neoplastic lesions were composed of intestinal type T cell lymphoma (ITCL, n=13, including one case with NK type), anaplastic large cell (ALCL, n=2), adult T cell leukaemia/lymphoma (ATLL, n=2), and lymphoblastic type (n=1). Epstein Barr virus infection was detected by EBER-1 in situ hybridization in 6 of 11 cases with ITCL but not in the other types. ALCL expressed CD30. CD56 was expressed in 3 of 11 cases of ITCL but not in other types. Among the 10 examined cases, 8 were alphabeta T cell type [CD2+, CD3+, T cell receptor (TCR)delta-1-, betaF1+], one was gammadelta T cell type [CD2+, CD3+, TCRdelta-1+, betaF1-], and the remaining case expressed natural killer (NK) cell type [CD2+, CD3-, CD56+, TCRdelta-1-, betaF1-]. Among the 8 examined cases, 3 expressed CD103 molecule, which was associated with extrathymic T cells of intraepithelial lymphocytes. All cases except ATLL expressed the cytotoxicity-associated molecule of TIA-1, and 11 of 14 TIA-1 positive cases expressed activated cytotoxic molecules of perforin, granzyme B, and/or Fas ligand. Despite the morphological, genetic and phenotypic heterogeneity, prognosis was poor, and 11 of 13 patients with small intestinal lesions died albeit appropriate treatment, but 3 of 4 patients with gastric or colonic lesions were still alive. The main cause of death was intestinal perforation. The latter might be due to the site specificity of small intestine and tumour cytotoxicity.     

Expression of cytotoxic proteins in peripheral T-cell and natural killer-cell (NK) lymphomas: association with extranodal site, NK or Tgammadelta phenotype, anaplastic morphology and CD30 expression

Most peripheral T-cell lymphomas (PTCL) express the alphabeta T-cell receptor (TCR) whereas rare PTCL express the gammadelta TCR. Most if not all gammadelta PTCL are extranodal lymphomas and among them, hepatosplenic gammadelta PTCL constitute a distinct clinicopathological entity. Besides alphabeta and gammadelta PTCL, there is a recently recognized group of extranodal, mainly nasal tumours, which display, in most instances, phenotypic and genotypic features of Natural-Killer cell non-Hodgkin's lymphomas (NK-NHL). Cytotoxic cells, including NK cells and cytotoxic alphabeta and gammadelta T lymphocytes may induce lysis of the target by using granule-associated cytotoxic proteins such as the T-cell intracellular antigen-1 (TIA-1), perforin and granzyme B. Expression of TIA-1 can be detected in all cytotoxic cells whereas granzyme B and perforin expression can be detected in high levels only in activated cytotoxic cells. Recently, several studies showed that the expression of these cytotoxic proteins in tumour cells of PTCL and NK-NHL is associated with a) extranodal site of clinicopathological presentation b) NK or Tgammadelta-cell phenotype c) CD30 expression in cutaneous T-cell lymphoproliferations and d) anaplastic morphology in nodal PTCL. This latter finding contrasts with the data that only rare Hodgkin lymphomas (HL) express cytotoxic proteins in Hodgkin and Reed-Sternberg cells. Altogether the data of the literature indicate that most extranodal T and NK-NHL are activated cytotoxic lymphomas with the notable exception of hepatosplenic gammadelta PTCL which represent tumours of non-activated cytotoxic cells. On this basis, it is suggested that the expression of cytotoxic proteins may be useful for the identification and classification of extranodal T and NK-cell lymphomas and, to some extent, for the differential diagnosis between HL and CD30+ anaplastic large cell lymphomas. Cytotoxic lymphomas are preferentially localized in extranodal sites such as skin, lung, upper respiratory and gastrointestinal tracts, which are continuously exposed to various antigens. Since cytotoxic T and NK cells are regarded as first line of defense in these sites, and some cytotoxic tumours such as nasal lymphomas and enteropathy-type intestinal lymphomas are associated with EBV and gliadin, respectively, it is likely that chronic antigen exposure may play a role in the pathogenesis of cytotoxic lymphomas occurring in mucosa and/or skin. Besides chronic antigenic stimulation, chronic immunosuppression may also have pathogenetic significance in cytotoxic lymphomas in view of their increased incidence in immunocompromised patients.     

ALK-1-negative anaplastic large cell lymphoma associated with breast implants: a new clinical entity

Concerns have been raised recently regarding the increasing number of reports of non-Hodgkin lymphoma (NHL) that developed in close proximity to silicone or saline breast implants. In particular, an increased risk of anaplastic large cell lymphoma (ALCL) in patients with breast prostheses has been proposed. We reviewed clinical and pathologic findings in 40 women who received a diagnosis of breast NHL arising in association with breast implants and of 27 patients who had a diagnosis of ALCL with breast involvement reported in the published literature. Among the 40 reported cases of prosthesis-associated breast lymphomas, 28 were anaplastic lymphoma kinase-1–negative (ALK-1^–) ALCLs, whereas of 27 ALCLs in patients without implants found in the literature, only 10 were ALK-1^–. The finding of 28 cases of breast ALK-1^– ALCL occurring in patients with implants compared with 10 cases in women without implants is in favor of an association between silicone breast prostheses and ALK-1^– ALCL. Although the incidence of this type of lymphoma remains remarkably low given that breast prostheses have been widely used for decades, clinical and pathologic evidence for a causative role is becoming dramatically strong. The histologic, phenomenologic, and clinical similarities of the majority of implant-related ALK-1^– ALCLs suggest a common mechanism, especially when compared with the counterpart of patients without implants in which very few and highly dishomogeneous cases of the same malignancy were detected. There is convincing evidence that primary implant-related ALK-1^– ALCL represents a distinct clinicopathologic entity that has been inappropriately fitted into the category of systemic ALK-1^– ALCL. Thus it should be recognized as a separate category and classified on its own.     

细胞毒分子在原发结内的外周Ｔ细胞淋巴瘤非特指型中的表达及意义

【摘　要】　目的：通过对外周Ｔ细胞淋巴瘤 非特指型（ＰＴＣＬ-Ｕ）患者病理组织蜡块细胞毒分子（ＣＭ）Ｔ细胞内抗原（ＴＩＡ-１）和颗粒酶Ｂ（ＧＢ）的免疫组化检测,探讨ＣＭ在ＰＴＣＬ-Ｕ进一步分型中的作用及意义。方法：应用免疫组化的方法检测２００５年１月～２００８年１月５１例经病理确诊的ＰＴＣＬ-Ｕ患者的ＴＩＡ-１和ＧＢ的表达情况,并分析其与Ｋｉ-６７、临床特征和治疗意义以及预后的关系。结果：本组５１例患者中有３３３％（１７／５１）表达至少１个细胞毒分子。在ＣＭ阳性与阴性表达中,Ｋｉ-６７高表达比例为８８.２％ ｖｓ．５０.０％（Ｐ＝０.００７８）,ＬＤＨ升高比例为８２.４％ ｖｓ．５２.９％（Ｐ＝０.０４０６）,出现Ｂ症状比例为７０.６％ｖｓ．３５.３％（Ｐ＝０.０１７３）,一般行为状态差（ＰＳ＞２分）比例为６４.７％ ｖｓ．３２.４％（Ｐ＝０.０２７９）;总的生存率经Ｌｏｇ-ｒａｎｋ检验ＣＭ阳性显著低于阴性（Ｐ＝０.００１５）。多因素生存分析显示,ＣＭ、一般行为状态差（ＰＳ＞２分）和Ｋｉ-６７高表达可以做为ＰＴＣＬ-Ｕ的独立预后因素。结论：细胞毒分子的表达不仅和ＰＴＣＬ-Ｕ预后差相关,而且作为细胞毒细胞的特异性标记在原发于结内的ＰＴＣＬ-Ｕ进一步分型中具有一定的作用。     

系统性间变性大细胞淋巴瘤骨髓累及的临床病理学研究

 目的　探讨系统性间变性大细胞淋巴瘤（S-ALCL）骨髓累及的临床病理学特点、免疫学表型及临床生物学行为。方法　回顾性分析34例S-ALCL病例资料,进行骨髓活检（19例）或涂片（15例）。其中ALK（+）24例,ALK（-）10例。HE染色、免疫组织化学染色观察病理形态及免疫表型,原位杂交法检测EB病毒。结果　6例（17.6 %）S-ALCL存在骨髓累及,均经骨髓活检标本确定,15例患者骨髓涂片中均未见肿瘤累及。ALK（+）ALCL和ALK（-）ALCL骨髓累及的发生率分别为16.7 %（4／24）和20.0 %（2／10）,差异无统计学意义（P＝0.3555）。与无骨髓累及病例比较,骨髓累及病例的年龄、性别分布差异无统计学意义（P值分别为0.8089和0.3085）。骨髓累及者肿瘤细胞以间质性分布为主[83.3 %（5／6）]。生存分析统计提示伴有骨髓累及的患者预后明显差于无骨髓累及者（P＝0.0407）。结论　S-ALCL骨髓累及发生率低,与患者的发病年龄、性别及ALK 蛋白的表达无相关性。伴有骨髓累及的S-ALCL患者临床预后差,骨髓活检在判断S-ALCL预后中有重要意义。