Cunningham-Rundles C, Cooper Dennis L, Duffy Thomas P, Strauchen James
Department of Medicine, Mount Sinai School of Medicine, New York City, New York 10029, USA.
Am J Hematol. 2002 Mar;69(3):171-8. doi: 10.1002/ajh.10050.
Common variable immunodeficiency (CVID) is a primary immunodeficiency disease characterized by low serum immunoglobulins IgG, IgA, and usually IgM. The central immune deficiency is impaired secretion of immunoglobulins and lack of antibody production; however, T cell dysfunction and a variety of inflammatory complications suggest global immune dysregulation. A number of reports have documented the association of primary immunodeficiency diseases with the development of non-Hodgkin's lymphoma (NHL). In CVID, the risk has been estimated to lie between 1.4% and 7%. As for NHL arising in other immunodeficiency states, the lymphomas in CVID are extranodal and are usually B cell in type. Of 22 B cell lymphomas that have appeared over a period of 25 years in a cohort of subjects with CVID, five lymphomas, appearing in more recently studied subjects, that arose in mucosal sites would be classified as mucosa-associated lymphoid tissue (MALT) lymphomas. MALT lymphomas are low-grade B cell lymphomas that result from a proliferation of neoplastic marginal-zone related cells of lymphoid tissue and tend to occur in organs that have acquired lymphoid tissue due to long-term infectious or autoimmune stimulation. Lymphomas of this kind have not been described in patients with congenital immunodeficiency, although chronic mucosal antigen stimulation is an integral part of these immune deficiency states.
普通可变免疫缺陷（CVID）是一种原发性免疫缺陷疾病，其特征为血清免疫球蛋白IgG、IgA降低，通常IgM也降低。核心免疫缺陷是免疫球蛋白分泌受损及抗体产生缺乏；然而，T细胞功能障碍和多种炎症并发症提示存在整体免疫失调。许多报告记录了原发性免疫缺陷疾病与非霍奇金淋巴瘤（NHL）发生之间的关联。在CVID中，该风险估计在1.4%至7%之间。至于在其他免疫缺陷状态下发生的NHL，CVID中的淋巴瘤为结外淋巴瘤，通常为B细胞型。在一组患有CVID的受试者中，25年间出现的22例B细胞淋巴瘤中，有5例出现在近期研究的受试者中，发生于黏膜部位，将被归类为黏膜相关淋巴组织（MALT）淋巴瘤。MALT淋巴瘤是低度B细胞淋巴瘤，由淋巴组织肿瘤性边缘区相关细胞增殖引起，倾向于发生在因长期感染或自身免疫刺激而获得淋巴组织的器官。尽管慢性黏膜抗原刺激是这些免疫缺陷状态的一个组成部分，但先天性免疫缺陷患者中尚未描述过这类淋巴瘤。
