UTILIZATION OF THE IMMUNODEFICIENCY CANCER RESEARCH

收集自然发生的罕见癌症患者病例 来自世界各地的免疫缺陷已经允许研究人员在 免疫缺陷癌症登记处（ICR）描述比例过剩 淋巴瘤和胃癌的发病率。 是 现在公认淋巴瘤代表了一个主要的 危及生命的自然并发症，以及获得性T淋巴细胞 免疫缺陷（例如，与慢性免疫抑制治疗相关 在实体器官同种异体移植、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 淋巴瘤在假定非免疫缺陷的人，淋巴瘤在 免疫缺陷患者更可能1）发生频率更高 在较年轻的年龄或开始后的短潜伏期 免疫抑制，2）表达弥漫性和高度恶性组织学， 3)发展于结节部位，如中枢神经系统，4） 与病毒转化有关，特别是涉及爱泼斯坦巴尔 病毒，和5）对常规治疗模式无反应。 在 在接下来的24个月里，ICR的目标是：1）开始研究 一种特殊免疫缺陷的淋巴瘤，Wiskott Aldrich综合征，2） 继续进行现有的合作，并积极寻求新的合作 将利用国际民事代表处提供的独特资源，以及3） 继续发展一个详细、有效的计算机化数据库， 作为未来研究通用资源的ICR案例。

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.