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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,INTER
2、NATIONAL 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 site,
3、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 t
4、hyroiditis - 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 burgdorferi,
5、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 2011,
6、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,Pseudogastritis30%,Nodular infiltration 25%,Ulcers45%,JC Delchier Henri Mondor Hospital,
7、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% - 100
8、% 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 0Pinotti, 1997 45 CT 67 3-18 2Neubauer, 1997 50 CTEUS 80 1-9 5No
9、bre Leitao, 1998 17 CT+EUS 100 1-12 1Steinbach, 1999 23 CTEUS 56 3-45 0 Montalban, 2001 19 CTEUS 95 2-19 0 Ruskone-Formestraux, 2001 24 CT+EUS 79 2-18 2LY03 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, Gut 56:168
10、5-7, 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 Gastric DLBCL
11、- 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 yrTsang, 2001 9 20-30 100% at 5 yrYahalom, 2002 51 30 median 89%
12、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, JCO 1995,(
13、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 Koch J Clin Onc
14、ol 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 : B-cell MALT
15、 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+,