アブストラクト
Japanese
| Title | 慢性活動性EBウイルス感染症 |
|---|---|
| Subtitle | 総説 |
| Authors | 木村宏 |
| Authors (kana) | |
| Organization | 名古屋大学大学院医学系研究科ウイルス学 |
| Journal | 日本造血細胞移植学会雑誌 |
| Volume | 10 |
| Number | 2 |
| Page | 87-93 |
| Year/Month | 2021 / |
| Article | 報告 |
| Publisher | 日本造血細胞移植学会 |
| Abstract | 慢性活動性EBウイルス感染症は, EBVがT細胞あるいはNK細胞に潜伏感染し, クローナリティを持って増殖, 臓器に浸潤し, 多彩な症状を呈する難治性疾患である. 予後は, 概して不良で, 無治療の場合には, 臓器合併症・急性転化により死に至ることが多い. 筆者らは, 慢性活動性EBウイルス感染症患者に対して大規模な網羅的遺伝子解析を行い, 1) 生殖細胞列の変異はほとんど認められないため原発性免疫不全症が潜んでいる可能性は低いこと, 2) EBV感染細胞では, DDX3X, KMT2Dなどのドライバー遺伝子変異が認められ, 経時的に観察できた例では, EBV感染細胞のクローン進化が確認されること, 3) 高率に欠失ウイルスが認められ発症に関与している可能性があること, を明らかにした. 本稿では, これら遺伝子解析によって解明されつつある慢性活動性EBウイルス感染症の発症病理と, 診断・治療に関わる最新の知見について概説する. |
| Practice | 臨床医学：内科系 |
| Keywords | Epstein-Barr virus, Chronic active EB virus infection, DDX3X, defective EBV |
English
| Title | Chronic active EB virus infection |
|---|---|
| Subtitle | |
| Authors | Hiroshi Kimura |
| Authors (kana) | |
| Organization | Department of Virology, Nagoya University Graduate School of Medicine |
| Journal | Journal of Hematopoietic Cell Transplantation |
| Volume | 10 |
| Number | 2 |
| Page | 87-93 |
| Year/Month | 2021 / |
| Article | Report |
| Publisher | The Japan Society for Hematopoietic Cell Transplantation |
| Abstract | [Abstract] Chronic active EB virus infection is an intractable disease. EBV latently infects T cells or NK cells, proliferates in a clonal manner, infiltrates organs, and triggers various symptoms. The prognosis is generally poor ; in the absence of treatment, organ complications and a blast crisis often cause death. Recently, we performed a large-scale, comprehensive genetic analysis of patients with chronic active EB virus infection, and found : 1) it is unlikely that any primary immunodeficiency is in play (because mutations in germ cell sequences were rarely observed) ; 2) EBV-infected cells contained mutations in driver genes such as DDX3X and KMT2D and, in cases that could be observed over time, clonal evolution of EBV-infected cells was evident ; and, 3) defective EBV was frequently observed and may be involved in disease onset. This review outlines the pathology of chronic active EB virus infection revealed by our genetic analyses, and the latest views on optimal diagnosis and treatment. |
| Practice | Clinical internal medicine |
| Keywords | Epstein-Barr virus, Chronic active EB virus infection, DDX3X, defective EBV |
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