Isaacson P G
Semin Diagn Pathol. 1985 Aug;2(3):210-23.
A high incidence of intestinal lymphoma has been noted in the Middle East since the early 1960's. Initially thought to be a single entity, Middle Eastern intestinal lymphoma is now perceived as at least 3 distinct types. Mediterranean lymphoma (MTL), also known as alpha chain disease and immunoproliferative small intestinal disease (IPSID), occurs in the proximal small intestine of young adults who present with malabsorption. Histologically a phase of plasma cell infiltration is followed by invasive lymphoma. In approximately 50% of cases an alpha 1 heavy chain paraproteinaemia is present. Intestinal lymphoma of so called Western type (WTL) occurs in older patients without malabsorption or a paraproteinaemia. The lymphoma is of follicular center cell type and there is no preceding plasma cell infiltrate. Recent work suggests that both MTL and WTL belong to a distinctive group of lymphomas that specifically arise from mucosa associated lymphoid tissue. The third type of Middle Eastern intestinal lymphoma occurs predominantly in the ileo-caecal region of children and is histologically indistinguishable from Burkitts lymphoma.
自20世纪60年代初以来，中东地区肠道淋巴瘤的发病率一直很高。中东肠道淋巴瘤最初被认为是一种单一疾病，现在被认为至少有3种不同类型。地中海淋巴瘤（MTL），也称为α链病和免疫增殖性小肠疾病（IPSID），发生在出现吸收不良的年轻成年人的近端小肠。组织学上，先是浆细胞浸润阶段，随后是侵袭性淋巴瘤。在大约50%的病例中，存在α1重链副蛋白血症。所谓的西方型肠道淋巴瘤（WTL）发生在无吸收不良或副蛋白血症的老年患者中。该淋巴瘤为滤泡中心细胞型，且无先前的浆细胞浸润。最近的研究表明，MTL和WTL都属于一组独特的淋巴瘤，它们特别起源于黏膜相关淋巴组织。中东肠道淋巴瘤的第三种类型主要发生在儿童的回盲部区域，组织学上与伯基特淋巴瘤无法区分。
