Vorechovsky I, Litzman J, Lokaj J, Hausner P, Poch T
Paediatric Research Institute, Brno, Czechoslovakia.
Cancer Immunol Immunother. 1990;31(4):250-4. doi: 10.1007/BF01789177.
Two patients with common variable immunodeficiency (CVID) and malignant tumours are reported. The first patient developed myelogenous leukaemia soon after the myelodysplastic syndrome has been diagnosed. The undifferentiated gastric lymphoma found in the second patient suggests that an increased risk of gastrointestinal malignancies in CVID could partly be due to lymphomas. We hypothesize that the tissue- or site-specific risk of lymphomas and gastrointestinal cancer can be explained by an increased chromosomal or genomic instability with a higher mutation rate and genomic disorganization, and that this instability could be related to viral carcinogenesis. The primary immunodeficiency per se may not be responsible for the cancer susceptibility in CVID patients.
报告了两名患有常见可变免疫缺陷(CVID)和恶性肿瘤的患者。第一名患者在骨髓增生异常综合征被诊断后不久就发展为骨髓性白血病。第二名患者发现的未分化胃淋巴瘤表明,CVID患者胃肠道恶性肿瘤风险增加可能部分归因于淋巴瘤。我们推测,淋巴瘤和胃肠道癌的组织或部位特异性风险可以通过染色体或基因组不稳定性增加、更高的突变率和基因组紊乱来解释,并且这种不稳定性可能与病毒致癌作用有关。原发性免疫缺陷本身可能不是CVID患者癌症易感性的原因。