UTILIZATION OF THE IMMUNODEFICIENCY CANCER RESEARCH

自然发生的癌症患者罕见病例的收集 来自世界各地的免疫缺陷使研究人员能够 描述比例过剩的免疫缺陷癌症登记(ICR) 淋巴瘤和胃癌在这组人中的比例。它是 现在公认的是淋巴瘤是主要的 自然和获得性T淋巴细胞的危及生命的并发症 免疫缺陷(例如，与慢性免疫抑制治疗有关 同种异体实体器官移植后，骨髓移植后 免疫缺陷或获得性免疫缺陷综合征，艾滋病)。相比较 对推测为非免疫缺陷的人的淋巴瘤， 免疫缺陷患者更可能1)以更高的频率发生 年龄较小或开始后潜伏期较短 免疫抑制，2)表现为弥漫性和高度恶性组织学， 3)发生在结外部位，如中枢神经系统，4)BE 与病毒转化有关，特别是涉及爱泼斯坦·巴尔 病毒；5)对常规治疗方式无反应。在 在接下来的24个月里，ICR的目标是1)启动对糖尿病病因的研究 一种特殊免疫缺陷的淋巴瘤，Wiskott Aldrich综合征，2) 继续进行持续的协作并积极征集新的协作 这将利用通过ICR提供的独特资源，以及3) 继续开发详细、有效的计算机化数据库 ICR案例作为未来研究的普遍资源。

The collection of rare cases of cancer in patients with naturally-occurring immunodeficiencies from around the world has permitted investigators at the Immunodeficiency Cancer Registry (ICR) to describe the proportional excess of lymphomas and gastric carcinomas among this group of individuals. It is now well-recognized that lymphomas represent one of the major life-threatening complications of natural, as well as acquired T lymphocyte immunodeficiencies (e.g. associated with chronic immunosuppressive therapy following solid organ allografting, post marrow transplant immunodeficiency, or acquired immunodeficiency syndrome, AIDS). Compared to lymphomas in presumably nonimmunodeficient persons, lymphomas in immunodeficient patients are more likely to 1) occur with higher frequency at younger ages or with short latency following initiation of immunosuppression, 2) express diffuse and high grade malignant histology, 3) develop in extranodal sites such as the central nervous syste, 4) be associated with viral transformation, particularly involving Epstein Barr virus, and 5) be unresponsive to conventional treatment modalites. In the next 24 months the ICR aims to 1) initiate studies of the etiology of lymphomas in one particular immunodeficiency, Wiskott Aldrich syndrome, 2) continue ongoing collaborations and actively solicit new collaborations which will utilize the unique resources available through the ICR, and 3) continue the development of a detailed, validated computerized data base of ICR cases as a universal resource for future studies.