Watkins Casey, Sahni Ryan, Holla Nikhil, Litchfield John, Youngberg George, Krishnaswamy Guha
Quillen College of Medicine - Johnson City, Tennessee, USA.
Cardiovasc Hematol Disord Drug Targets. 2012 Sep;12(1):21-7. doi: 10.2174/187152912801823084.
Patients can develop malignancies due to various reasons including genetic factors, chemical carcinogens, radiation, and defects in their immune system. The immune system is postulated to carry out routine surveillance for malignancy. Patients who have defective immune responses may be susceptible to malignancies due to complicated underlying mechanisms. These include defective immune response to cancer-causing bacteria, transforming viruses, and concomitant molecular, cellular and immunoregulatory defects. Common variable immune deficiency (CVID) is characterized by hypogammaglobulinemia, impaired antibody responses and an increased susceptibility to infections. A disorderly immune response, or immune dysregulation, may also lead to autoimmune complications and possibly to malignancy. The treatment of CVID involves infusion of replacement doses of immunoglobulin, either intravenously (IGIV) or subcutaneously (SCIG). However, it is unclear whether adequate replacement of immunoglobulins is sufficient to prevent the increased risk of malignancy seen in this disease. We present two cases of unusual solid tumors complicating CVID treated with adequate doses of intravenous immunoglobulins. In this study we review the occurrence of malignancy in patients with CVID and postulate mechanisms that may be involved indigent to this disease. We will also review the role of replacement immunoglobulin and discuss cancer screening in these high risk individuals.
患者可能由于多种原因发生恶性肿瘤，包括遗传因素、化学致癌物、辐射以及免疫系统缺陷。据推测，免疫系统会对恶性肿瘤进行常规监测。免疫反应有缺陷的患者可能由于复杂的潜在机制而易患恶性肿瘤。这些机制包括对致癌细菌、转化病毒的免疫反应缺陷，以及伴随的分子、细胞和免疫调节缺陷。常见可变免疫缺陷（CVID）的特征是低丙种球蛋白血症、抗体反应受损以及感染易感性增加。免疫反应紊乱或免疫失调也可能导致自身免疫并发症，并可能导致恶性肿瘤。CVID的治疗包括静脉注射（IGIV）或皮下注射（SCIG）替代剂量的免疫球蛋白。然而，尚不清楚免疫球蛋白的充分替代是否足以预防该疾病中出现的恶性肿瘤风险增加。我们报告了两例接受足量静脉注射免疫球蛋白治疗的CVID并发罕见实体瘤的病例。在本研究中，我们回顾了CVID患者中恶性肿瘤的发生情况，并推测可能与该疾病相关的机制。我们还将回顾替代免疫球蛋白的作用，并讨论这些高危个体的癌症筛查。
