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1、,原发胃肠结外淋巴瘤诊疗进展,刘艳艳 河南省肿瘤医院淋巴综合内科,P-MAR-2015.06-046 Valid Until 2017.06,专业资料，仅供医药卫生专业人士使用,声明,本幻灯片代表个人观点。处方请参考国家食品药品监督管理总局批准的药物说明书。,定义,来源于淋巴结外的淋巴组织 甚至来源于正常情况下不含淋巴组织的部位 当结内和结外病变同时存在时，定义较困难,发病率,占非霍奇金淋巴瘤的25%,淋巴瘤,淋巴结,淋巴结外,胃肠道,非胃肠道,胃 : B-cell MALT DLBCL H. Pylori,肠道 : T-cell Celiac disease,睾丸 脑 T/NK 鼻型,INT
2、ERNATIONAL EXTRANODAL LYMPHOMA STUDY GROUP,Extranodal Lymphoma Survival by histology and site in the IELSG series,少见 : 所有胃肠道肿瘤的3% 绝大多数胃肠道淋巴瘤来源于胃,原发胃肠道淋巴瘤,P Koch J Clin Oncol 2001,15%,3%,75%,Non Hodgkins Lymphoma Classification Project. Blood 1997;89:3909-18,Frequency % G Gastric I Intestin Nodal sit
3、e,1.4%G - 4%I,0%G - 25%I,0%G - 20%I,胃肠道淋巴瘤分类,Mantle cell L.,Diffuse large B cell lymphoma,T-cell lymphoma,Burkitt. L,MALT Lymphoma,1%G - 20% (colon),Follicular L.,38%G - 10%I,60%,系统检查分期,MALT lymphoma : ESMO GUIDELINES Dreyling M, Thieblemont C. et al. Ann Oncol 2012,Auto-antigens,- Thyroid Hashimoto
4、 thyroiditis - Salivary gland Myoepithelial sialoadenitis +/ - Sjgren S. - - Lung Lymphoid interstitial pneumopathy,MZL（边缘区淋巴瘤）: 与慢性抗原刺激相关,MALT Lymphomas,Site Infectious agents - Stomach Helicobacter pylori - Intestin Campylobacter jejuni - Ocular adnexa Chlamydia psittaci - skin Borrelia burgdorfer
5、i,Hepatitis C Virus,Microbial pathogens,1.,2.,+,Splenic MZL,Isaacson P, Wright DH. Cancer 1983,HELICOBACTER PYLORI in STOMACH,MZL: associated with a chronic antigenic stimulation,chronic Ag stimulation - chronic inflammation,MALT CONCEPT,MALT淋巴瘤常见的遗传损伤,NF-KB activation,Bertoni F. et al. Oncology 201
6、1,Normal stomach,Chronic gastritis MALT Lymphoma,+ additional factors: host, environment, genetic,HP,NFKB,t(11;18) API2-MALT1 t(1,14) BCL10 t(14;18) Ig-MALT1,E. De Kerviler Saint-Louis Hospital, Paris,胃MALT 淋巴瘤内镜,Pseudogastritis 30%,Nodular infiltration 25%,Ulcers 45%,JC Delchier Henri Mondor Hospit
7、al, Crteil,Normal stomach,Chronic gastric MALT Lymphoma,+ additional factors: host, environment, genetic,HP,NFKB,t(11;18) API2-MALT1 t(1,14) BCL10 t(14;18) Ig-MALT1,ATB,Hussel, Lancet 1993; Wootherspoon, Lancet 1993; Wndisch, JCO 2005,依赖H. Pylori的胃MALT 淋巴瘤的治疗,Hp. eradication,Complete response: 60% -
8、 100% Response: 3 to 28 months ! Resistance associated to t(11;18),Hussel, Lancet 1993; Wootherspoon, Lancet 1993; Wndisch, JCO 2005,Lymphoma,Reference n staging CR rate time to CR relapses procedure (%) (mos.) (n) Savio, 1996 12 CT 84 2-4 0 Pinotti, 1997 45 CT 67 3-18 2 Neubauer, 1997 50 CTEUS 80 1
9、-9 5 Nobre Leitao, 1998 17 CT+EUS 100 1-12 1 Steinbach, 1999 23 CTEUS 56 3-45 0 Montalban, 2001 19 CTEUS 95 2-19 0 Ruskone-Formestraux, 2001 24 CT+EUS 79 2-18 2 LY03 interim analysis, 2000 190 CT 62 3-24 15,抗生素和质子泵治疗stage I 胃 MALT 淋巴瘤,After 5 years = 71%,Median follow-up = 7 years,Fischbach et al, G
10、ut 56:1685-7, 2007 Pinotti et al, 10-ICML Abstract # 361 Stathis A et al, Ann Oncol 2009,n = 120 patients,抗生素治疗后的缓解期,Normal stomach,Chronic gastric MALT Lymphoma,HP,t(11;18) API2-MALT1 t(1,14) BCL10 t(14;18) Ig-MALT1,DLBCL,p53 deletion, p16 deletion,Gastric DLBCL Dependant to Hp. ?,10 pts with Gastr
11、ic DLBCL - Stage IE or IIE PPI-amoxicillin-clarithromycin for 7 days,JC Delchier et al. IELSG 2011,Biomarkers associated with antigen dependance,RT in localized gastric MALT lymphoma,Author n RT dose (Gy) FFP Schechter, 1998 17 28-43 100% at 2 yr Tsang, 2001 9 20-30 100% at 5 yr Yahalom, 2002 51 30
12、median 89% at 4 yr Hitchcock, 2002 9 34 median 78% (100% local) Goda JS, 2010 25 25-30 79% at 5 yr,烷化剂单药治疗,24 例患者, 17 例stage I ，7 例stage IV Cyclophosphamide or Chlorambucil for 8-24 mos. 100% ORR (75% CR) 5-year EFS: 50% 5-year OS: 75% 5 relapses at initial sites (1 with transformation),Hammel et al
13、, JCO 1995,(cyclophosphamide or chlorambucil),Lymphoma,Nodal,Extranodal,Gastro-intestinal,Non Gastro-intestinal,Gastric : B-cell MALT DLBCL H. Pylori,Intestinal : T-cell Celiac disease,Testis Brain T/NK nasal Type,胃肠道弥漫大B细胞淋巴瘤,60% of primary GI lymphoma,GI DLBCL,临床表现,侵袭性 B 症状 大肿块 坏死 穿孔风险: 10%!,P Koc
14、h J Clin Oncol 2001:19:3861,GI DLBCL,治疗目的,One Goal,To cure the patient with the first line of treatment,GI DLBCL,TREATMENT,No surgery Biomarkers are needed to detect the Hp. - dependant gastric DLBCL Standard R-CHOP,GI DLBCL,Lymphoma,Nodal,Extranodal,Gastro-intestinal,Non Gastro-intestinal,Gastric :
15、 B-cell MALT DLBCL H. Pylori,Intestinal : T-cell Celiac disease,Testis Brain T/NK nasal Type,EATL,肠病型小肠T细胞淋巴瘤(EATL),通常有谷蛋白敏感性肠病既往史 腹腔疾病患者: Relative risk of EATL x 50-100 临床表现: 多发空肠溃疡 Extension: GI tract : estomac, colon Extra-intestinal : blood, skin, lung,EATL,EATL,粘膜内T淋巴细胞增殖 Phenotype T CD3+CD4- CD8-/+ TCR- CD103+ Phenotype T cytotoxic TIA1+ granzyme B+,Granzyme B,CD103,组织学表现,治疗,不含谷蛋白膳食 No good chemotherapy OS poor 1 year = 35% 5 years = 20%,EATL,结论,结外淋巴瘤具有独特的临床病理特征 胃肠道淋巴瘤最常见 多数与慢性抗原刺激相关 治疗应采取个体化,Thanks for your attention
