淋巴组织肿瘤.ppt
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1、淋巴瘤 Lymphoma 基础医学院病理学系 同济医院病理研究所 敖启林,Dr John K.C.CHAN 陈国璋,4th Edition Published 20/9/2008,第一节 概述 一、定义 “来源于”成熟淋巴细胞及其前体细胞(淋巴母细胞)的恶性肿瘤,分为非霍奇金/白血病和霍奇金淋巴瘤两大类。 二、分类 (一)非霍奇金淋巴瘤(NHL):包括B,T和NK细胞(70%) 1、前体细胞: B/T/NK前体细胞(淋巴母细胞)淋巴瘤(2%) 2、成熟细胞:成熟细胞淋巴瘤(68%) B细胞(54%) T细胞(12%) NK/T或NK细胞(2%),(二)霍奇金淋巴瘤(HL):特殊类型成熟B细胞淋
2、巴瘤(30%) 结节性淋巴细胞为主型(1.5%) 经典型(28.5%) (缺陷性B细胞) 结节硬化型 淋巴细胞丰富型 混合细胞型 淋巴细胞消减型,第二节 淋巴结的免疫结构 (immunoarchitecture) 一、概念:通过免疫组织化学染色显示淋巴结的结构特征。包括肿瘤性或非肿瘤性细胞的成分(淋巴细胞,辅助性非淋巴细胞,淋巴窦,血管,网状纤维等)、数量、分布方式等特征。通过对比,掌握正常和异常免疫结构,对淋巴结病变进行诊断和鉴别诊断。高质量的组织学切片和合理的免疫标记物的选择是识别免疫结构的关键。,二、淋巴结的正常免疫结构 (一)淋巴结结构:皮质,髓质,窦,被膜和纤维小梁,(二)B淋巴细胞
3、分布: 1.主要在浅皮质区的初级和次级滤泡,髓索和副 皮质区也含一定量B细胞; 2.B细胞分化过程中的形态和免疫学特征成为非霍 奇金B细胞淋巴瘤分类的基础。,Pre pre B cell,Pre B cell,Immature B cell,Mature B cell,Activated B cell,Plasma cell,Precursor cells; antigen non-responsive,Virgin B cells; antigen responsive,Germinal center antigen encountered,TdT,CD20, CD22,CD19, PAX5
4、,CD79a,CD10,Bcl-6,CD138,Cytoplasmic CD22,Immunoarchitecture of normal lymph node,B cells (CD20+,CD10+,BCL-6+),T cells (CD3+); only small numbers of CD20+ cells,1、初级滤泡:处女型B细胞和成熟小淋巴细胞;,CD20,2、次级滤泡:由生发中心、套区和边缘区构成。,Organization of secondary lymphoid follicle,Marginal zone cells (IgM),Mantle zone cells (
5、IgM+D),Follicle center cells (IgM, IgG, IgA or IgE, not IgD),:约2:1,当:8-10:1或:3:1提示轻链限制表达,次级滤泡生发中心、套区和边缘区免疫标记: (1)生发中心(Germinal Center, GC): 1)中心细胞和中心母细胞:CD10+,BCL-6+,Bcl-2-, 2)滤泡树突细胞(Follicular dendritic cells, FDC) : CD21+,CD23+, CD35+ 3)T细胞:CD4+, CD10+, CXCL13/PD-1+,少量CD57+ 4)可染体巨噬细胞:CD68+,lysozym
6、e+,CD11c+ (2)套区:处女型B细胞和记忆细胞,表达IgM, IgD (3)边缘区:无特殊标记,绝大多数次级滤泡难以鉴别,腹腔 淋巴结易识别。,CD20,CD79a,CD21,CD10,Bcl-6,反应性滤泡增生的特点 对T细胞依赖性抗原的反应 滤泡增多,大小、形态不等,可弥漫分布,主要位于皮质区,无背靠背现象。 典型生发中心具有极性(Polarity):明区和暗区,套区明显,扁桃体组织更易识别; 星天现象,中心细胞和中心母细胞混杂, 免疫染色:生发中心细胞BCL-2-,ki-67指数较高。 滤泡间区一般无B细胞弥漫阳性,B细胞CD43和CD5阴性。,Dark zone,Light z
7、one,BCL-2,Ki67,Marginal zone cells,CD20,(三)T淋巴细胞分布:副皮质区(paracortex),T淋巴细胞为主 (CD4CD8): 主为小淋巴细胞,偶尔出现大细胞 (可以是活化的B细胞); 含有高内皮静脉 (High endothelial venules,HEV): 血液淋巴细胞入淋巴结的通道; 含有指状树突细胞(interdigitating dendritic cells,IDC): S100+ 的专职抗原递呈细胞;,Prothymocyte,Subcapsular thymocyte,Cortical thymocyte,Medullary th
8、ymocyte,Peripheral T cell,Precursor cells,Mature T cells,Cytoplasmic,Surface,CD4,CD8,CD1,CD4, CD8,CD3,CD2,CD7,TdT,IDC,CD3,副皮质区增生,常见病因: 病毒感染, 接种疫苗,药物,自身免疫疾病, 皮病性淋巴结炎,肿瘤转移等 形态特征:副皮质扩大,可形成结节; HEV增生; 混合小淋巴细胞和转化淋巴细胞,浆细胞,树突细胞,嗜酸性粒细胞等; 免疫染色:主为T细胞,或T/B细胞混合,一般无B细胞为主弥漫分布。,Immunoblast,Infectious mononucleosis,
9、CD20,CD3,Large cells comprise mixture of B and T cells-Reactive process,CD30+ cells are scattered and staining is weak or moderate, which indicates the heterogeneity of the large cell population.,CD30 staining is diffusely strong positive, which indicates the monoclone entity.,Kappa,Lambda,Large B c
10、ells are polytypic,T细胞为主时支持良性的依据(谨慎!): (1)结构不同程度保留:淋巴窦和皮质淋巴滤泡; (2)大细胞群体分布不规则或呈地图样,不形成膨胀性团块; (3)无浸润现象:上皮,脂肪,血管,神经,纤维间质中单行排列; (4)细胞无明显异型:2.5倍小淋巴细胞;常无透明胞质;核形态 较规则,圆形或椭圆形,轻度扭曲或折叠; (5)免疫染色:常为CD4+为主, 少为CD8+为主(AIDS,骨髓移植等); 一般无CD4-CD8-或CD4+CD8+表型,PanT+(无缺失); 少见ALK,TdT,CD56, T cell receptor等表达,斑驳状,Autoimmune
11、 lymphoproliferative syndrome,ALPS,斑驳状,Reactive large lymphoid cell (immunoblast),Small lymphocytes,Nuclear size usually does not exceed 2.5 times that of small lymphocyte,Nucleus usually round or ovoid, and does not show irregular foldings,(四)髓索(medullary cord),富含浆细胞 可以清晰或不清晰 反应性B细胞的扩张延伸,如反应性淋巴滤泡增生
12、,浆细胞型 Castleman disease。,(五)淋巴窦(sinus),输入淋巴管 被膜下窦 间窦 髓窦 输出淋巴管; 被覆窦内皮 (D2-40),含组织细胞和淋巴样细胞; 腹腔内淋巴结窦常扩张,较明显; 深处窦的存在常提示良性病变。,(六)纤维结缔组织网 Connective tissue framework,纤维被膜, 延伸至结内的梁索 (可见梁索周窦); 正常时可能不清晰; 反应性病变常清晰,常有淋巴结周围炎。,第三节 淋巴瘤分类 WHO Classification 2008 非霍奇金淋巴瘤 non-Hodgkin lymphomas 霍奇金淋巴瘤 Hodgkin lymphom
13、as,非霍奇金淋巴瘤分类WHO Classification ofnon-Hodgkin lymphomas (2001),Precursor lymphoblastic lymphoma/leukemia (B or T cell type),Mature B-cell neoplasms Mature T-cell and NK-cell lymphomas,成熟B细胞肿瘤Mature B-cell neoplasms (WHO),B-cell CLL/SLL B-cell prolymphocytic leukemia Lymphoplasmacytic lymphoma Mantle
14、cell lymphoma Follicular lymphoma Extranodal marginal zone B-cell lymphoma of MALT type,Nodal marginal zone B-cell lymphoma Splenic marginal zone B cell lymphoma Hairy cell leukemia Diffuse large B-cell lymphoma (including mediastinal large B-cell lymphoma) Burkitt lymphoma Plasmacytoma, myeloma,成熟T
15、细胞或NK细胞肿瘤Mature T-cell occasionally waxing and waning Usually high stage (III/IV) at presentation; blood blood/marrow involvement uncommon (bad prognosis; not so bad if marrow involved by follicular lymphoma) Untreated, will kill in 1-2 years High proliferative fraction: potentially curable with che
16、motherapy +/- radiotherapy 80% CR; 2/3 no relapse,Survival curve of aggressive lymphomas,% Survival,100%,0%,Years,0,10,Treated,Untreated,Comparison of survival curves,% Survival,100%,0%,Years,0,10,Aggressive NHL,Indolent NHL,Paradoxically better outcome in the long run,高度侵袭性淋巴瘤Highly aggressive lymp
17、homas:Medium-sized cell lymphoma,Lymphoblastic lymphoma Burkitt lymphoma,Highly aggressive lymphomas,Children, young adults Very rapidly growing: oncologic emergency Often high stage; marrow involvement common; CNS involvement may occur Untreated, will kill in weeks to months Very high proliferative
18、 fraction: highly responsive to treatment. Standard chemotherapy: no good; very aggressive chemotherapy: many cured,Survival curve of highly aggressive lymphomas,% Survival,100%,0%,Years,0,10,Treated,Untreated,局限性惰性淋巴瘤Localized indolent lymphomas,Extranodal marginal zone B-cell lymphoma of mucosa-as
19、sociated lymphoid tissue (MALT) type Primary cutaneous anaplastic large cell lymphoma,Localized indolent lymphomas,Indolent tumor Can occur in any age Often localized at presentation Many patients can apparently be cured (there is a suggestion of plateau in survival curve), although late relapses ar
20、e possible Can undergo regression spontaneous for primary cutaneous ALCL with anti-Helicobacter therapy for gastric extranodal MZL,各型淋巴瘤的临床病理特征,淋巴母细胞淋巴瘤,Rapidly growing tumor Lymphoma: T B Leukemia (ALL): B T Untreated: rapidly fatal Aggressive therapy: good survival Cannot predict lineage from morp
21、hology Defining marker: TdT+,CD99,CD20,CD79a,B-LBL,T-LBL,滤泡性淋巴瘤FOLLICULAR LYMPHOMA,Usually involving lymph nodes High-stage disease at presentation Pathology: Recapitulates the organization and cytology of the normal germinal centers Key immunophenotype: B marker+, CD10+, Bcl6+, Bcl2+ Follicular lym
22、phoma involving some extranodal sites (such as skin, duodenum, testis) show distinctive clinical and biologic features Genetics:Ig gene rearrangement; t(14;18)(q32;q21) bcl-2 gene rearrangement (due to BCL2/IGH translocation),Follicular lymphoma vsreactive follicular hyperplasia,Major criterion Clos
23、ely packed follicles throughout, with scanty interfollicular tissues,1 major criterion or 3 minor criteria. But confirm by ancillary tests if uncertain,滤泡性淋巴瘤主要诊断标准示意图,Major criterion fulfilled in 80% of cases of follicular lymphoma,Just go on to high magnification to confirm follicle center cell mo
24、rphology,Centrocytes often have triangular shaped nuclei,滤泡性淋巴瘤的次要诊断标准,In the remaining 20% of cases (non-crowded follicles), a combination of minor criteria (3) have to be considered, and supplemented by ancillary studies as required,Minor criteria No tingible-body macrophages Cellular monotony: ce
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