Kersey J H, Shapiro R S, Filipovich A H
Bone Marrow Transplantation Program, University of Minnesota, Minneapolis.
Pediatr Infect Dis J. 1988 May;7(5 Suppl):S10-2.
Individuals with either primary or secondary immunodeficiencies are at high risk to develop not only infections but also malignancy (especially of the lymphoid system). The major focus of this paper is on malignancies that develop in immunodeficiency syndromes, particularly malignancies in naturally occurring immunodeficiencies and following bone marrow transplantation (BMT). As of August, 1986, 514 cases of naturally occurring immunodeficiencies have been registered at the Immunodeficiency Cancer Registry. Overall non-Hodgkin's lymphomas predominate in these patients, accounting for 48.6% of all cases. Non-Hodgkin's lymphoma is the predominant malignancy in ataxia-telangiectasia, common variable immunodeficiency, Wiskott-Aldrich syndrome (WAS) and severe combined immunodeficiency (SCID). The histopathology of the lymphomas differs somewhat in each of the disorders. In WAS, large cell "histiocytic" lymphoma predominates, with most cases having the features of B lymphocytes, including pleomorphic immunocytoma and immunoblastic lymphoma. Non-Hodgkin's lymphoma in SCID also generally has B cell features and in some cases multiple copies of Epstein-Barr virus (EBV) genomic DNA have been found in tumor tissue. In contrast to ataxia-telangiectasia, in which non-Hodgkin's lymphoma is also the predominant neoplasm, the morphology and cell marker characteristics are more similar to those seen in nonimmunodeficient children. The lymphomas in ataxia-telangiectasia are very heterogeneous with representation from all the major histologic subtypes. We have found no relationship between the degree of immunodeficiency and the development of malignancy. Immunodeficiency following BMT, as in naturally occurring immunodeficiencies, appears to predispose patients to the development of lymphoid malignancy, especially for recipients of partially mismatched bone marrow. In Minnesota 8 patients have developed B cell lympho-proliferative disorders (BLPD) following BMT.(ABSTRACT TRUNCATED AT 250 WORDS)
原发性或继发性免疫缺陷患者不仅极易发生感染，还易患恶性肿瘤（尤其是淋巴系统肿瘤）。本文主要关注免疫缺陷综合征中发生的恶性肿瘤，特别是自然发生的免疫缺陷以及骨髓移植（BMT）后发生的恶性肿瘤。截至1986年8月，免疫缺陷癌症登记处已登记514例自然发生的免疫缺陷病例。总体而言，非霍奇金淋巴瘤在这些患者中占主导地位，占所有病例的48.6%。非霍奇金淋巴瘤是共济失调毛细血管扩张症、常见变异型免疫缺陷、威斯科特-奥尔德里奇综合征（WAS）和严重联合免疫缺陷（SCID）中的主要恶性肿瘤。在每种疾病中，淋巴瘤的组织病理学略有不同。在WAS中，大细胞“组织细胞性”淋巴瘤占主导，大多数病例具有B淋巴细胞特征，包括多形性免疫细胞瘤和免疫母细胞淋巴瘤。SCID中的非霍奇金淋巴瘤通常也具有B细胞特征，在某些病例的肿瘤组织中发现了多个拷贝的爱泼斯坦-巴尔病毒（EBV）基因组DNA。与共济失调毛细血管扩张症不同，在共济失调毛细血管扩张症中，非霍奇金淋巴瘤也是主要的肿瘤，其形态和细胞标志物特征与非免疫缺陷儿童更为相似。共济失调毛细血管扩张症中的淋巴瘤非常异质性，涵盖所有主要组织学亚型。我们发现免疫缺陷程度与恶性肿瘤的发生之间没有关系。与自然发生的免疫缺陷一样，BMT后的免疫缺陷似乎使患者易患淋巴系统恶性肿瘤，尤其是接受部分不匹配骨髓的患者。在明尼苏达州，8名患者在BMT后发生了B细胞淋巴增殖性疾病（BLPD）。（摘要截短于250字）
