The X-linked lymphoproliferative disease: from autopsy toward cloning the gene 1975-1990.
The X-linked lymphoproliferative disease: from autopsy toward cloning the gene 1975-1990.
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DOI：
10.3109/15513819109065466
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发表时间：
1991-09
期刊：
影响因子：
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通讯作者：
D. Purtilo;H. Grierson;Jack R. Davis;M. Okano
中科院分区：
文献类型：
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作者：
D. Purtilo;H. Grierson;Jack R. Davis;M. Okano
虽然x连锁淋巴细胞增生性疾病（XLP）是罕见的（每1 × 10(6) 1-2名男性），但它可以作为识别由eb病毒（EBV）引起的各种疾病的模型，从无球蛋白血症到感染病毒后致命的传染性单核细胞增多症。对XLP患者的研究也为理解EBV如何在患有原发性免疫缺陷疾病、器官移植接受者和获得性免疫缺陷综合征的儿童中诱发疾病铺平了道路。这篇综述是为了纪念医学博士Gordon Vawter，他慷慨地提供了免疫缺陷和淋巴增生性疾病发病机理的见解。
Although X-linked lymphoproliferative disease (XLP) is rare (1-2 males per 1 x 10(6)), it serves as a model for discerning diverse diseases caused by Epstein-Barr virus (EBV) ranging from agammaglobulinemia to fatal infectious mononucleosis following infection with the virus. The study of patients with XLP has also paved the way to understanding how EBV induce diseases in children with primary immunodeficiency diseases, organ transplant recipients, and those with acquired immunodeficiency syndrome. This review is dedicated to the memory of Gordon Vawter, M.D., who generously provided insights into the causes of pathogenesis of immune deficiency and lymphoproliferative disorders.