Filipovich A H, Mathur A, Kamat D, Kersey J H, Shapiro R S
Immunodeficiency Cancer Registry, University of Minnesota, Minneapolis.
Immunodeficiency. 1994;5(2):91-112.
Lymphoproliferative disorders and selected carcinomas which occur as complications of primary or secondary immunodeficiencies are frequently fatal. The incidence rates of these cancers vary from 1% to as high as 25% among specific groups of persons with primary (genetically-determined) immunodeficiencies as well as acquired immunodeficiencies, including immunosuppressed organ transplant recipients and individuals infected with HIV. Lymphoproliferative disorders including Epstein Barr virus (EBV) associated B cell lymphoproliferative disease (BLPD) and Hodgkin's disease represent the predominant category of tumors in both primary and acquired immunodeficiencies. EBV is an important cofactor common to many, but not all, B cell "lymphomas." Immunodeficient individuals who are at risk for developing EBV BLPD may demonstrate both inadequate immune responses to the virus as well as generalized immunoregulatory dysfunction reflected as imbalances in cytokine production favoring the proliferation of transformed B lymphocytes. Historically, the success of treatment of lymphoproliferative disorders in immunodeficiencies with conventional multi agent chemotherapies and/or radiation has been limited by unfavorable tumor response rates and high morbidity and mortality related to intercurrent opportunistic infections. With improvements in supportive care and the use of recombinant biologic response modifiers such as alpha interferon and/or other immunotherapies to treat EBV BLPD, survival of immunodeficient hosts following tumor diagnosis may improve. In addition to lymphoproliferative disorders, patients with congenital immunodeficiencies associated with IgA deficiency (including ataxia telangiectasia and Common Variable Immunodeficiency) are at increased risk for gastrointestinal carcinomas. Early detection and surgical excision of such tumors can result in prolonged survival in such patients.
作为原发性或继发性免疫缺陷并发症出现的淋巴增殖性疾病和某些癌症通常是致命的。在患有原发性（基因决定的）免疫缺陷以及获得性免疫缺陷的特定人群中，包括免疫抑制的器官移植受者和感染HIV的个体，这些癌症的发病率从1%到高达25%不等。淋巴增殖性疾病包括与爱泼斯坦-巴尔病毒（EBV）相关的B细胞淋巴增殖性疾病（BLPD）和霍奇金病，是原发性和获得性免疫缺陷中主要的肿瘤类型。EBV是许多（但不是所有）B细胞“淋巴瘤”共有的重要辅助因子。有发生EBV BLPD风险的免疫缺陷个体可能表现出对该病毒的免疫反应不足以及普遍的免疫调节功能障碍，表现为细胞因子产生失衡，有利于转化B淋巴细胞的增殖。从历史上看，用传统的多药化疗和/或放疗治疗免疫缺陷中的淋巴增殖性疾病的成功率受到不利的肿瘤反应率以及与并发机会性感染相关的高发病率和死亡率的限制。随着支持治疗的改善以及使用重组生物反应调节剂如α干扰素和/或其他免疫疗法来治疗EBV BLPD，肿瘤诊断后免疫缺陷宿主的生存率可能会提高。除了淋巴增殖性疾病外，与IgA缺乏相关的先天性免疫缺陷患者（包括共济失调毛细血管扩张症和常见可变免疫缺陷）患胃肠道癌的风险增加。早期发现并手术切除此类肿瘤可使这些患者延长生存期。
