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% 24% 27%36%44%37% 41%48%29%0%100%淋巴瘤淋巴结 淋巴结外胃肠道 非胃肠道胃 : B-cellMALTDLBCLH. Pylori肠道 : T-cell Celiac disease睾
2、丸脑T/NK 鼻型INTERNATIONAL 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 200115%3%75%follicular22%mantle cell6%DLBCL35%MZL, MALT8%MZL3% others6%anaplastic T/null2%PTCL7%lymphoblastic2%Burki
3、tt1%small ly8%Non Hodgkins Lymphoma Classification Project. Blood 1997;89:3909-18Frequency %G Gastric I IntestinNodal site1.4%G - 4%I0%G - 25%I0%G - 20%I胃肠道淋巴瘤分类Mantle cell L. Diffuse large B cell lymphomaT-cell lymphomaBurkitt. LMALT Lymphoma1%G - 20% (colon)Follicular L. 38%G - 10%I60%系统检查分期MALT l
4、ymphoma : ESMO GUIDELINES Dreyling M, Thieblemont C. et al. Ann Oncol 2012Lymphoma GI lymphomaMandatory physical exam complete blood counts basic biochemical studies (renal and liver function, LDH and 2MG, serum protein immunofixation) HIV, HCV and HBV serology CT of the chest, abdomen and pelvis- G
5、ASTRIC : Gastroduodenal endoscopy with multiple biopsies taken from each region of the stomach, duodenum, gastro-esophageal junction and from any abnormal-appearing site; H. pylori status must be evaluated in gastric L.- SMALL INSTESTINE (IPSID Immuno-Proliferative Small Intestinal Disease): Campylo
6、bacter Jejuni search in the tumor biopsy by PCR, immunohistochemistry or in situ hybridization may be performed.- LARGE INTESTINE : colonoscopyRecommended bone marrow aspirate and biopsy If clinically indicated, head & neck MRI studies and other imaging are to be realizedAuto-antigens- Thyroid Hashi
7、moto 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 LymphomasSite Infectious agents- Stomach Helicobacter pylori - Intestin Campylobacter jejuni- Ocular adnexa Chlamydia psittaci- skin Borrelia burgdorferi Hepatitis C VirusMicrobial pathogens1.2.+Splenic MZLIsaacson P, Wright DH. Cancer 1983
