Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada; Division of Immunology and Allergy, The Hospital for Sick Children, Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada.
Clin Immunol. 2018 Sep;194:46-59. doi: 10.1016/j.clim.2018.06.011. Epub 2018 Jul 4.
Primary Immunodeficiency disorders (PID) have been increasingly recognized in association with hematologic malignancies. To better appreciate this association, a systemic search of the Ovid MEDLINE database was performed with terms related of hematologic malignancies and all PID described in the 2017 International Union of Immunological Societies Expert Committee for Primary Immunodeficiency. More than 60 PID distinct PID, caused by cell-intrinsic and extrinsic mechanisms were associated with diverse hematologic malignancies. These occurred among all subgroups of PID, including syndromic and non-syndromic combined PID affecting cellular and humoral immunity, predominantly antibody deficiencies and defects of immune regulation. In addition, defects in phagocyte numbers or functions, or in innate immunity were associated with hematologic malignancies. Increased awareness and vigilance for the possibility of malignancy is required when caring for patients with PID.
原发性免疫缺陷病（PID）与血液系统恶性肿瘤的关系日益受到关注。为了更好地了解这种关联，我们对 Ovid MEDLINE 数据库进行了系统检索，使用了与血液系统恶性肿瘤和 2017 年国际免疫学协会专家委员会描述的所有 PID 相关的术语。超过 60 种不同的 PID，由内在和外在的细胞机制引起，与各种血液系统恶性肿瘤有关。这些发生在 PID 的所有亚组中，包括影响细胞和体液免疫的综合征和非综合征联合 PID，主要是抗体缺陷和免疫调节缺陷。此外，吞噬细胞数量或功能的缺陷，或固有免疫缺陷与血液系统恶性肿瘤有关。在照顾 PID 患者时，需要提高对恶性肿瘤可能性的认识和警惕。
