Okano M, Nakanishi M, Taguchi Y, Sakiyama Y, Matsumoto S
Department of Pediatrics, Hokkaido University School of Medicine, Sapporo, Japan.
Acta Paediatr Jpn. 1992 Aug;34(4):385-92. doi: 10.1111/j.1442-200x.1992.tb00976.x.
Increased incidence of malignant disorders is noted in patients with both primary and acquired immunodeficiency diseases. The pathogenetic mechanism(s) for these disorders remain unclear. Defective immunosurveillance of these patients, however, is mainly postulated to be responsible for the increased risk of these malignant disorders. Of the malignant disorders, Epstein-Barr virus (EBV)-induced lymphoproliferative disorders (LPD) have been increasingly reported, possibly due to improved therapeutic management techniques such as bone marrow transplantation, which results in prolonged survival periods for the primary immunodeficiency; the dramatic development of immunosuppressive treatments for transplant recipients; and the growing numbers of acquired immunodeficiency syndrome (AIDS) patients. This review focuses on the primary immunodeficiency diseases and EBV-induced LPD, and discusses pathogenetic mechanism(s) for the increased incidence of these malignant disorders.
原发性和获得性免疫缺陷疾病患者中恶性疾病的发病率有所增加。这些疾病的发病机制仍不清楚。然而，这些患者免疫监视功能缺陷主要被认为是这些恶性疾病风险增加的原因。在恶性疾病中，爱泼斯坦-巴尔病毒（EBV）诱导的淋巴增殖性疾病（LPD）的报道越来越多，这可能是由于治疗管理技术的改进，如骨髓移植，从而使原发性免疫缺陷患者的生存期延长；移植受者免疫抑制治疗的迅速发展；以及获得性免疫缺陷综合征（AIDS）患者数量的增加。本综述重点关注原发性免疫缺陷疾病和EBV诱导的LPD，并讨论这些恶性疾病发病率增加的发病机制。
