Du Shouying, Scuderi Richard, Malicki Denise M, Willert Jennifer, Bastian John, Weidner Noel
Department of Pathology, University of California-San Diego, 200 West Arbor Drive, San Diego, CA 92103-8720, USA.
Pediatr Dev Pathol. 2011 Jan-Feb;14(1):64-70. doi: 10.2350/10-01-0787-CR.1. Epub 2010 Apr 29.
Approximately 13% of patients with Wiskott-Aldrich syndrome (WAS), a primary immune deficiency, develop malignant tumors, the predominant form being non-Hodgkin's lymphoma. Previously, only 4 cases of Hodgkin's lymphoma have been reported in WAS patients. Herein, we review the literature of WAS-related lymphomas and report 2 brothers with WAS who both developed lymphomas; one developed Epstein-Barr virus (EBV)-driven diffuse large B-cell lymphoma, and one developed EBV-negative classical Hodgkin's lymphoma. In contrast to many of the previously reported lymphomas in WAS patients, these lymphomas were extensively evaluated by means of molecular, flow cytometric, and immunohistochemical methods. Both brothers died shortly after diagnosis, despite aggressive therapy. The occurrence of 2 distinct forms of lymphomas in these brothers underscores the interplay between genetic susceptibility and environmental exposure in lymphoma pathogenesis.
威斯科特-奥尔德里奇综合征（WAS）是一种原发性免疫缺陷病，约13%的患者会发生恶性肿瘤，最常见的类型是非霍奇金淋巴瘤。此前，仅有4例WAS患者发生霍奇金淋巴瘤的报道。在此，我们回顾了与WAS相关淋巴瘤的文献，并报告了2例患WAS且均发生淋巴瘤的兄弟；其中1例发生了由EB病毒（EBV）驱动的弥漫性大B细胞淋巴瘤，另1例发生了EBV阴性的经典型霍奇金淋巴瘤。与之前报道的许多WAS患者淋巴瘤不同，这2例淋巴瘤均通过分子、流式细胞术和免疫组化方法进行了广泛评估。尽管接受了积极治疗，但两兄弟均在诊断后不久死亡。这两兄弟发生的2种不同类型淋巴瘤突出了淋巴瘤发病机制中遗传易感性与环境暴露之间的相互作用。
