MALT淋巴瘤约占所有 B 细胞淋巴瘤的 7%-8%,被视为非霍奇金淋巴瘤中第三常见的类型。[4]Cohen SM, Petryk M, Varma M, et al. Non-Hodgkin's lymphoma of mucosa-associated lymphoid tissue. Oncologist. 2006;11:1100-1117.http://www.ncbi.nlm.nih.gov/pubmed/17110630?tool=bestpractice.com[5]The Non-Hodgkin's Lymphoma Classification Project. A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. Blood. 1997;89:3909-3918.http://bloodjournal.hematologylibrary.org/cgi/content/full/89/11/3909http://www.ncbi.nlm.nih.gov/pubmed/9166827?tool=bestpractice.com 此外,它是最常见的原发性结外淋巴瘤类型,在原发性胃部淋巴瘤中占的比例高达 50%。[1]Jaffe ES, Harris NL, Stein H, et al, eds. World Health Organization classification of tumors. Pathology and genetics: tumors of haemopoietic and lymphoid tissues. Lyon, France: IARC Press; 2008.[4]Cohen SM, Petryk M, Varma M, et al. Non-Hodgkin's lymphoma of mucosa-associated lymphoid tissue. Oncologist. 2006;11:1100-1117.http://www.ncbi.nlm.nih.gov/pubmed/17110630?tool=bestpractice.com 就诊时的整体平均年龄为 61 岁,女性稍占多数(男性与女性比率为 1:1.12)。[6]An SK, Han JK, Kim YH, et al. Gastric mucosa-associated lymphoid tissue lymphoma: spectrum of findings at double-contrast gastrointestinal examination with pathologic correlation. Radiographics. 2001;21:1491-1502.http://radiographics.rsna.org/content/21/6/1491.longhttp://www.ncbi.nlm.nih.gov/pubmed/11706219?tool=bestpractice.com胃部 MALT 淋巴瘤约占所有 MALT 淋巴瘤的 30%,患者平均年龄为 57 岁,无性别差异。[7]Kahl B, Yang D. Marginal zone lymphomas: management of nodal, splenic, and MALT NHL. Haematology Am Soc Hematol Educ Program. 2008;359-364.http://asheducationbook.hematologylibrary.org/cgi/content/full/2008/1/359http://www.ncbi.nlm.nih.gov/pubmed/19074110?tool=bestpractice.com[8]Isaacson PG. Gastric MALT lymphoma: from concept to cure. Ann Oncol. 1999;10:637-645.http://annonc.oxfordjournals.org/cgi/reprint/10/6/637http://www.ncbi.nlm.nih.gov/pubmed/10442184?tool=bestpractice.com 意大利东北部的 MALT 淋巴瘤的发病率更高。[9]Doglioni C, Wotherspoon AC, Moschini A, et al. High incidence of primary gastric lymphoma in northeastern Italy. Lancet. 1992;339:834-835.http://www.ncbi.nlm.nih.gov/pubmed/1347858?tool=bestpractice.com一种特殊亚型,免疫增生性小肠病(之前称为 α 重链病)在中东地区和南非开普敦地区的发病率增加。[10]Pinkel D. Differentiating juvenile myelomonocytic leukemia from infectious disease. Blood. 1998;91:365-367.http://bloodjournal.hematologylibrary.org/cgi/content/full/91/1/365http://www.ncbi.nlm.nih.gov/pubmed/9414312?tool=bestpractice.com[11]Price SK. Immunoproliferative small intestinal disease: a study of 13 cases with alpha heavy-chain disease. Histopathology. 1990;17:7-17.http://www.ncbi.nlm.nih.gov/pubmed/2227833?tool=bestpractice.com约 70% 的 MALT 淋巴瘤为非胃部淋巴瘤,患者平均年龄为 60 岁。[7]Kahl B, Yang D. Marginal zone lymphomas: management of nodal, splenic, and MALT NHL. Haematology Am Soc Hematol Educ Program. 2008;359-364.http://asheducationbook.hematologylibrary.org/cgi/content/full/2008/1/359http://www.ncbi.nlm.nih.gov/pubmed/19074110?tool=bestpractice.com[12]Zucca E, Conconi A, Roggero E, et al. Non-gastric MALT lymphomas: a survey of 369 European patients. The International Extranodal Lymphoma Study Group. Ann Oncol. 2000;11:99. 累及的部位包括头颈部 (30%)、眼附属器 (24%)、肺部 (12%),皮肤 (12%)、肠道 (8%)、甲状腺 (7%)、乳房 (2%) 以及生殖泌尿道 (1%)。[12]Zucca E, Conconi A, Roggero E, et al. Non-gastric MALT lymphomas: a survey of 369 European patients. The International Extranodal Lymphoma Study Group. Ann Oncol. 2000;11:99.硬脑膜、泪腺及眼眶也可能受累。