（内科PPT课件）-淋巴瘤.ppt

1、 Definition:Lymphomas are a group of malignant tumors originating from lymph nodes or other lymphatic tissues.Lymphoma may occur in any tissue or organ around the body.Hodgkins lymphoma(HL)15%non-Hodgikins lymphoma(NHL)80-90%2015-9-15高仓健高仓健罗京罗京李开复李开复Male:1.39/100,000；Female:0.84/100,000NHL increasin

2、g over timeHD stable during the years在常见肿瘤中分别占第在常见肿瘤中分别占第9(9(男性男性),),第第10(10(女性女性)发达国家：发达国家：12-15/1012-15/10万；中国：万；中国：1.6-2.1/101.6-2.1/10万万淋巴瘤在淋巴瘤在欧美国家占恶性肿瘤欧美国家占恶性肿瘤死亡的第死亡的第6 6位，位，我国我国位于第位于第1010位。位。每年发病率平均增加每年发病率平均增加4 4,增长最迅速的恶性增长最迅速的恶性肿瘤肿瘤uDestruction of normal follicular structure by a great numb

3、er of abnormal lymphocytes or histiocytes;uInfiltration of tissues surrounding the capsule by the tumor cells;uDamage of capsule and the subcapsular sinuses.subcapsular sinuses,包膜下窦全身症状：发热、盗汗及体重减低（皮肤瘙痒）；淋巴结肿大；组织器官侵润特点特点临床起病：单个或多个淋巴结或结外组织和器官病变发生部位：身体各处 早期表现：无痛性进行性淋巴结肿大。PCRbcl-1/JH重组基因）正电子发射体层成像 1 2 3

4、 4体格检查体格检查一般状况一般状况B B症状症状查血查血查骨髓查骨髓查脑脊液查脑脊液X X线线CTCT超声超声PETPET镓镓6767心电图心电图内窥镜等内窥镜等形态学形态学免疫学检查免疫学检查遗传学检查遗传学检查分子生物学分子生物学 基因芯片基因突变FISHPCR定量Involvement of single lymph node region(I)or of a single extralymphatic organ or site(IE)Involvement of two or more lymph node regions on the same side of diaphragm

5、(II)or localized involvement of an extralymphatic organ or site and one or more lymph node regions on the same side of the diaphragm(IIE)Involvement of lymph node regions on both sides of the diaphragm(III),which may also be accompanied by involvement of the spleen(IIIS)or by localized involvement o

6、f an extralymphatic organ or site(IIIE)or both(IIISE)Diffuse or disseminated involvement of one or more extralymphatic organs or tissues,with or without associated lymphnode involvementThe absence or presence of fever,night sweats and/or unexplained loss of 10%or more body weight in the 6 months pre

7、ceding diagnosis are to be denoted in all cases by the suffix letters A or B,respectively.ML-Gross Reed-Sternberg cell初级滤泡初级滤泡次级滤泡次级滤泡初级滤泡和次级滤泡初级滤泡和次级滤泡B区区:CD20T区区:CD3副皮质区副皮质区滤泡间区滤泡间区CD3CD3和和PAX5PAX5CD20CD20CD3CD3多拷贝体多拷贝体The characteristic finding is the presence of Reed-Sternberg cells in the tumor

8、 structureinclude over 20 discrete entities with characteristic morphologic,immunophenotypic,genetic,and clinical features：85%of NHL are B-cell lymphomasB 淋巴母细胞白血病/淋巴瘤B 淋巴母细胞白血病/淋巴瘤，NOS B 淋巴母细胞白血病/淋巴瘤，具有重现性细胞遗传学异常B 淋巴母细胞白血病/淋巴瘤，具有t(9;22)(q34;q11.2);BCR-ABL1 B 淋巴母细胞白血病/淋巴瘤，具有t(v;11q23);MLL 重排 B淋巴母细胞白

9、血病/淋巴瘤，具有t(12;21)(p13;q22);TEL-AML1(ETV6-RUNX1)B 淋巴母细胞白血病/淋巴瘤，具有超二倍体 B 淋巴母细胞白血病/淋巴瘤，具有亚二倍体(亚二倍体ALL)B 淋巴母细胞白血病/淋巴瘤，具有t(5;14)(q31;q32);IL3-IGH B 淋巴母细胞白血病/淋巴瘤，具有t(1;19)(q23;p13.3);E2A-PBX1;(TCF3-PBX1)T淋巴母细胞白血病/淋巴瘤 慢性淋巴细胞白血病/小淋巴细胞淋巴瘤 前B细胞白血病 脾边缘区淋巴瘤 毛细胞白血病 脾淋巴瘤/白血病，未分类*脾弥漫红髓的小B细胞淋巴瘤 毛细胞白血病-变异型 淋巴浆细胞淋巴瘤

10、华氏巨球蛋白血症 重链病 Alpha重链病 Gamma重链病 Mu重链病 浆细胞瘤 骨的孤立性浆细胞瘤 髓外浆细胞瘤 粘膜相关淋巴组织结外边缘区淋巴瘤(MALT淋巴瘤)结内边缘区淋巴瘤 儿童结内边缘区淋巴瘤 滤泡性淋巴瘤 儿童滤泡性淋巴瘤 原发于皮肤的滤泡中心淋巴瘤 套细胞淋巴瘤 弥漫大B细胞淋巴瘤(DLBCL)，NOS 富含T/组织细胞的大B细胞淋巴瘤 原发于中枢神经系统的DLBCL 原发于皮肤的DLBCL，腿型 老年性EB病毒阳性的DLBCL 与慢性炎症相关的DLBCL 淋巴样肉芽肿病 原发于纵隔（胸腺）的大B细胞淋巴瘤 血管内大B细胞淋巴瘤 ALK阳性的大B细胞淋巴瘤 浆母细胞性淋巴瘤

11、HHV8相关的大B细胞淋巴瘤 多 中心Castleman 病 原发性渗出性淋巴瘤 伯基特淋巴瘤 B细胞淋巴瘤，不能分类型,，具有介于弥漫大B细胞淋巴瘤与伯基特淋巴瘤之间的特征 B细胞淋巴瘤，不能分类型，具有介于弥漫大B细胞淋巴瘤与典型霍奇金病之间的特征霍奇金淋巴瘤l结节淋巴细胞为主型霍奇金淋巴瘤 l典型霍奇金淋巴瘤l结节硬化型典型霍奇金淋巴瘤 l富含淋巴细胞的典型霍奇金淋巴瘤 l混合细胞性典型霍奇金淋巴瘤 l淋巴细胞消减型典型霍奇金淋巴瘤 移植后淋巴细胞增殖性疾病(PTLD)l早期损伤l浆细胞增生 l感染性单核细胞增多样PTLD l多形性 PTLD l单一形态的 PTLD(B-及T/NK-细胞

12、型)#l典型霍奇金淋巴瘤型PTLD#Lymphocyte richRareNodular infiltration predominantly with mature lymphocytesLocalized,good prognosis Nodular sclerosisAlways present lacunar typeInterlacing bands of collagen dividing the cellular infiltrate into islandsOccurs in young persons,stage I or II at the time of diagnosi

13、s,prognosis relatively favorableMixed cellularityManyFibrosis with localized necrosis,infiltration of neutrophils,eosinophils and plasma cells,lymphocytes and histiocytes Tendency to disseminate,prognosis relatively poorLymphocytedepletionVariable in numberPredominantly infiltration of hitiocytes,di

14、sseminated fibrosis and necrosis Occurs in old age stage III or IV at the time of diagnosis,prognosis usually poor结节性淋巴细胞为主型（NLPHL），和经典型（CHL）。病例形态、免疫表型、分子生物学、临床表现和生物行为均有差异。淋巴细胞为主型（LRHL），结节硬化型（NSHL），混合细胞型（MCHL），淋巴细胞消减型（LDHL）Low GradeSmall lymphocytic(SLL)Follicular,small cleaved cell(FSCL)Follicular,

15、mixed small cleaved and large cell(FMC)B cell,rarely T cellB cellB cellIntermediate GradeFollicular,large cell(FLCL)Diffuse,small cleaved cell(DSCL)Diffuse,mixed small cleaved and large cell(DML)Diffuse,large cell(cleaved and non-cleaved)(DLCL)B cellB cell,occasionally TB cell,T cellB cell,T cellHig

16、h GradeLarge cell immunoblastic(IBL)Lymphoblastic(convoluted and non-convoluted)(LL)Small non-cleaved cell(Burkitt and non-Burkitt)(SNCL)B cell,T cellThymic T cellB cellWHO将HNL分为：B细胞、T细胞和NK T细胞，以及灰区NHL。结外发病多结外发病多分期较晚多分期较晚多HL少少滤泡淋巴瘤少滤泡淋巴瘤少发病少发病少好治的少好治的少年轻的少年轻的少NHL多多弥漫型多弥漫型多总数多总数多难治的多难治的多男的多男的多总数多总数多难

17、治的多难治的多男的多男的多T T细胞淋巴瘤占细胞淋巴瘤占20-30%20-30%，远多于欧美国家，远多于欧美国家NHLIndolentYearsGenerally not curableGenerally defer Watch&WaitRx/asymptomaticAggressiveMonthsCurable in someTreatVery aggressiveWeeksCurable in someTreatAll typesVariable months to yearsCurable in mostTreatHL-ABVD,MOPP，-NHL-CHOP,CHOPE,DICE,ESH

18、AP，-CD20CD22CD25CD30CD40CD52CD80Blood,2011 Feb;117(5):1453-62First-line:Very aggressive,CR or PR 此外缓解时间和生存时间也是判断化疗疗效的重要指标,除去客观的指标外，一般状况的改善可做为治疗有效的指标 Pathological featureImmunophenotypeClinical stageSystemic manifestationsAgeMass diameterInfiltration sites and organsLDH and B2MP levelsSensitivity to chemotherapyProliferation rate of lymphoma cell 穿穿 刺刺临床医师临床医师CD5CD38活检活检病理医病理医师师CD3MPO检验科检验科 病理科病理科检验科检验科 血液科血液科检验科检验科 病理科病理科