Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States
Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States
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DOI：
10.1182/blood-2010-11-316745
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发表时间：
2011-06-02
期刊：
影响因子：
20.3
通讯作者：
Straus, Stephen E.
中科院分区：
文献类型：
--
作者：
Cohen, Jeffrey I.;Jaffe, Elaine S.;Straus, Stephen E.
慢性活动性EB病毒病（CAEBV）是一种淋巴组织增生性疾病，其特征是血液中EB病毒抗体或EB病毒DNA水平以及组织中淋巴细胞中EB病毒RNA或蛋白质水平显着升高。我们提出我们的经验，在过去的28年中，包括第一个8例在美国造血干细胞移植治疗的CAEBV。大多数CAEBV病例报告来自日本。与日本的CAEB病毒不同，日本的EB病毒几乎总是在组织中的T细胞或自然杀伤（NK）细胞中发现，而我们患者的组织中的B细胞中通常检测到EB病毒。大多数患者表现为淋巴结肿大和脾肿大;发热、肝炎和全血细胞减少是常见的。大多数患者死于感染或进行性淋巴细胞增生。与日本报告的病例不同，我们的患者经常表现为进行性B细胞丢失和低丙种球蛋白血症。虽然来自日本的CAEBV患者的NK细胞数量正常或增加，但我们的许多患者的NK细胞数量减少。虽然免疫抑制剂，利妥昔单抗，自体细胞毒性T细胞，或细胞毒性化疗往往导致短期缓解，他们是没有治愈。造血干细胞移植通常可以治愈CAEBV，即使是对化疗无反应的活动性淋巴组织增生性疾病患者。这些研究在www.example.com上注册为NCT 00032513（用于CAEBV）、NCT 00062868和NCT 00058812（用于EBV特异性T细胞研究）以及NCT 00578539（用于造血干细胞移植方案）。（血。2011; 117（22）：5835 - 5849）
Chronic active EBV disease (CAEBV) is a lymphoproliferative disorder characterized by markedly elevated levels of antibody to EBV or EBV DNA in the blood and EBV RNA or protein in lymphocytes in tissues. We present our experience with CAEBV during the last 28 years, including the first 8 cases treated with hematopoietic stem cell transplantation in the United States. Most cases of CAEBV have been reported from Japan. Unlike CAEBV in Japan, where EBV is nearly always found in T or natural killer (NK) cells in tissues, EBV was usually detected in B cells in tissues from our patients. Most patients presented with lymphadenopathy and splenomegaly; fever, hepatitis, and pancytopenia were common. Most patients died of infection or progressive lymphoproliferation. Unlike cases reported from Japan, our patients often showed a progressive loss of B cells and hypogammaglobulinemia. Although patients with CAEBV from Japan have normal or increased numbers of NK cells, many of our patients had reduced NK-cell numbers. Although immunosuppressive agents, rituximab, autologous cytotoxic T cells, or cytotoxic chemotherapy often resulted in short-term remissions, they were not curative. Hematopoietic stem cell transplantation was often curative for CAEBV, even in patients with active lymphoproliferative disease that was unresponsive to chemotherapy. These studies are registered at http://www.clinicaltrials.gov as NCT00032513 for CAEBV, NCT00062868 and NCT00058812 for EBV-specific T-cell studies, and NCT00578539 for the hematopoietic stem cell transplantation protocol. (Blood. 2011; 117(22): 5835-5849)