文献

J-GLOBAL ID：201802245931549767   整理番号：18A0154869

切断修復障害DNA二本鎖のための造血幹細胞移植の転帰【Powered by NICT】

Outcome of hematopoietic cell transplantation for DNA double-strand break repair disorders

著者 (55件)： Slack James (Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom) ,  Albert Michael H. (Dr. von Hauner University Children’s Hospital, Ludwig-Maximilians-Universitaet, Munich, Germany) ,  Balashov Dmitry (Immunology and Hematopoietic Stem Cell Transplantation Department, Federal Research Center for Pediatric Hematology, Oncology and Immunology, Moscow, Russia) ,  Belohradsky Bernd H. (Dr. von Hauner University Children’s Hospital, Ludwig-Maximilians-Universitaet, Munich, Germany) ,  Bertaina Alice (Stem Cell Transplant Unit, Department of Hematology and Oncology, Ospedale Pediatrico Bambino Gesu, Rome, Italy) ,  Bleesing Jack (Division of Bone Marrow Transplantation and Immune Deficiency, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio) ,  Booth Claire (Infection, Immunity, Inflammation, Molecular and Cellular Immunology Section, UCL Great Ormond Street Institute of Child Health, London, United Kingdom) ,  Buechner Jochen (Department of Pediatric Hematology and Oncology, Oslo University Hospital, Oslo, Norway) ,  Buckley Rebecca H. (Duke University School of Medicine, Departments of Pediatrics and Immunology, Duke University Medical Center, Durham, NC) ,  Ouachee-Chardin Marie (Paediatric BMT Unit, Robert Debre Hospital, Paris, France) ,  Deripapa Elena (Immunology and Hematopoietic Stem Cell Transplantation Department, Federal Research Center for Pediatric Hematology, Oncology and Immunology, Moscow, Russia) ,  Drabko Katarzyna (Department of Pediatric Hematology, Oncology and Bone Marrow Transplantation, Medical University of Lublin, Lublin, Poland) ,  Eapen Mary (Center for International Blood and Marrow Transplant Research, Division of Hematology/Oncology, Department of Medicine, Medical College of Wisconsin, Milwaukee, Wis) ,  Feuchtinger Tobias (Department of Haematology and Oncology, University Children’s Hospital Tuebingen, Tuebingen, Germany) ,  Finocchi Andrea (Department of Systems Medicine, University of Rome “Tor Vergata”, Unit of Immune and Infectious Diseases, Children’s Hospital Bambino Gesu, Rome, Itlay) ,  Gaspar H. Bobby (Infection, Immunity, Inflammation, Molecular and Cellular Immunology Section, UCL Great Ormond Street Institute of Child Health, London, United Kingdom) ,  Ghosh Sujal (Infection, Immunity, Inflammation, Molecular and Cellular Immunology Section, UCL Great Ormond Street Institute of Child Health, London, United Kingdom) ,  Ghosh Sujal (Division of Pediatric Stem Cell Therapy, Department of Pediatric Oncology, Hematology and Clinical Immunology, Medical Faculty, Center of Child and Adolescent Health, Heinrich-Heine-University, Duesseldorf, Duesseldorf, Germany) ,  Gillio Alfred (Joseph M. Sanzari Children’s Hospital, Hackensack University Medical Center, Hackensack, NJ) ,  Gonzalez-Granado Luis I. (Immunodeficiencies, Hematology & Oncology Unit, Pediatrics, Hospital 12 Octubre, and Universidad Complutense, Instituto de Investigacion Hospital 12 de Octubre (i+12), Madrid, Spain) ,  Grunebaum Eyal (Division of Immunology and Allergy, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada) ,  Guengor Tayfun (Division of Stem Cell Transplantation, University Children’s Hospital Zuerich, Zuerich, Switzerland) ,  Heilmann Carsten (BorneUngeKlinikken, Copenhagen, Denmark) ,  Helminen Merja (Paediatric Research Center, University Hospital of Tampere, Tampere, Finland) ,  Higuchi Kohei (Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan) ,  Imai Kohsuke (Department of Community Pediatrics, Perinatal and Maternal Medicine, Tokyo Medical and Dental University, Tokyo, Japan) ,  Kalwak Krzysztof (Department of Pediatric Hematology, Oncology and BMT, Wroclaw Medical University, Wroclaw, Poland) ,  Kanazawa Nubuo (Department of Dermatology, Wakayama Medical University, Wakayama, Japan) ,  Karasu Guelsuen (Bahcesehir University Faculty of Medicine, Goztepe Medicalpark Hospital Pediatric Stem Cell Transplantation Unit, Istanbul, Turkey) ,  Kucuk Zeynep Y. (Division of Bone Marrow Transplantation and Immune Deficiency, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio) ,  Laberko Alexandra (Immunology and Hematopoietic Stem Cell Transplantation Department, Federal Research Center for Pediatric Hematology, Oncology and Immunology, Moscow, Russia) ,  Lange Andrzej (Hirszfeld Institute of Immunology and Experimental Therapy, Polish Academy of Sciences / Lower Silesian Center for Cellular Transplantation, Wroclaw, Poland) ,  Mahlaoui Nizar (French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hopitaux de Paris, Paris, France) ,  Mahlaoui Nizar (Immuno-Hematology Unit, Necker Children’s Hospital, Assistance Publique Hopitaux de Paris, Paris, France) ,  Meisel Roland (Division of Pediatric Stem Cell Therapy, Department of Pediatric Oncology, Hematology and Clinical Immunology, Medical Faculty, Center of Child and Adolescent Health, Heinrich-Heine-University, Duesseldorf, Duesseldorf, Germany) ,  Moshous D. (Immuno-Hematology Unit, Necker Children’s Hospital, Assistance Publique Hopitaux de Paris, Paris, France) ,  Muramatsu Hideki (Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan) ,  Parikh Suhag (Duke University School of Medicine, Pediatric Blood and Marrow Transplant Program, Duke University Medical Center, Durham, NC) ,  Pasic Srdjan (Mother and Child Health Institute, Medical Faculty, University of Belgrade, Belgrade, Serbia) ,  Schmid Irene (Dr. von Hauner University Children’s Hospital, Ludwig-Maximilians-Universitaet, Munich, Germany) ,  Schuetz Catharina (Department of Pediatrics and Adolescent Medicine, University Medical Center Ulm, Ulm, Germany) ,  Schulz Ansgar (Department of Pediatrics and Adolescent Medicine, University Medical Center Ulm, Ulm, Germany) ,  Schultz Kirk R. (Michael Cuccione Childhood Cancer Research Program, BC Children’s Hospital and Child and Family Research Institute, Vancouver, British Columbia, Canada) ,  Shaw Peter J. (Department of Oncology, Children’s Hospital at Westmead, Westmead, Australia) ,  Slatter Mary A. (Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom) ,  Slatter Mary A. (Primary Immunodeficiency Group, Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom) ,  Sykora Karl-Walter (Department of Pediatric Hematology Oncology, Hannover Medical, Hannover, Germany) ,  Tamura Shinobu (Department of Hematology and Oncology, Wakayama Medical University, Wakayama, Japan) ,  Taskinen Mervi (Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, Helsinki University Hospital, Helsinki, Finland) ,  Wawer Angela (Paediatric Haematology & Oncology, Kinderklinik der Technische Universitaet Muenchen, Krankenhaus Muenchen-Schwabing, Munich, Germany) ,  Wolska-Kusnierz Beata (Department of Immunology, Children’s Memorial Health Institute, Warsaw, Poland) ,  Cowan Morton J. (Allergy Immunology and Blood and Marrow Transplant Division, University of California San Francisco Benioff Children’s Hospital, San Francisco, Calif) ,  Fischer Alain (Immuno-Hematology Unit, Necker Children’s Hospital, Assistance Publique Hopitaux de Paris, Paris, France) ,  Gennery Andrew R. (Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom) ,  Gennery Andrew R. (Primary Immunodeficiency Group, Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom)
資料名： Journal of Allergy and Clinical Immunology  (Journal of Allergy and Clinical Immunology)
巻： 141  号： 1  ページ： 322-328.e10  発行年： 2018年 
JST資料番号： A0182B  ISSN： 0091-6749  資料種別： 逐次刊行物 (A)
記事区分： 原著論文  発行国： オランダ (NLD)  言語： 英語 (EN)

抄録/ポイント： 稀なDNA切断修復障害は感染とリンパ網内系悪性腫瘍の素因となる。造血細胞移植(HCT)が治癒が,同時投与化学療法または放射線療法は全身放射線感受性のために損傷した。Nijmegen染色体不安定症候群,DNAリガーゼIV欠乏,Cernunnos XRCC4様因子(Cernunnos-XLF)欠損症,毛細血管拡張性運動失調症(AT)用HCT転帰データを収集した。,適応,ドナー,移植前処置,移植片対宿主病,および転帰を含む世界の38中心のデータを解析した。アルキルないおよび/または150mg~2フルダラビン以下及び40mg/kgシクロホスファミド以下を使用したかどうかは放射線療法またはアルキル化剤と骨髄非破壊的前処置(RIC)を含んでいたならば,移植前処置は骨髄破壊的コンディショニング(MAC)として分類した。五十五新しい14を更新し,18以前に公表された患者を分析した。HCT時の年齢の中央値は48か月であった(範囲,1.5 552か月)。二十九名の患者は感染症に対する移植を受け,21名は悪性度を有しており,13名は骨髄機能不全を有し,13名は予防的移植を受け,5名が複数の適応を呈し,6人は情報を持っていなかった。MAC,59受信RIC,および4を二十二は注入した;情報は2名の患者には利用できなかった。DNAリガーゼIV欠乏,Cernunnos-XLF欠損,またはNijmegen染色体不安定症候群の77人の患者中七十三人はコンディショニングを受けた。生存は77例中53例(69%)であり,RIC(P=.006)を受けている患者よりもMACを受けている患者の悪化した。ほとんどの死亡では移植後の初期に起こり,条件付けの低い耐性を示唆した。AT患者の生存率は25%であった。83例中四十一例(49%)は急性GvHD,MAC(26/56 [46%] vs 12/21 [57%], P=.45)を受けている患者と比較して,RICの投与を受けた患者で頻度が低かったを経験した。追跡期間中央値は35か月であった(範囲,2 168か月)。二次悪性腫瘍はフォローアップの15年間で報告されていない。成長と発達遅延は,HCT後に残った免疫介在性合併症は消失した。RIC HCTはDNA修復を障害関連免疫不全。二次悪性腫瘍サーベイランスに必要である長期フォローアップ。ATのためのルーチンHCTは推奨されない。Copyright 2018 Elsevier B.V., Amsterdam. All rights reserved. Translated from English into Japanese by JST.【Powered by NICT】

シソーラス用語： *除去修復 ,  骨髄 ,  放射線療法 ,  *DNA【核酸】 ,  免疫不全症候群 ,  *造血幹細胞移植 ,  移植片対宿主病 ,  薬物療法 ,  患者 ,  生存率
準シソーラス用語： 二次癌 ,  DNAリガーゼIV ,  Nijmegen染色体不安定症候群 ,  悪性腫瘍 ,  *転帰 , 【Automatic Indexing@JST】

著者キーワード (6件)： 毛細血管拡張性運動失調症 ,  Cernunnos XLF欠損症 ,  DNA修復障害 ,  DNAリガーゼIV欠乏 ,  造血幹細胞移植 ,  Nijmegenはbreakage症候群

分類 (4件)： 血液の腫よう  (GK04000M) ,  血液疾患の治療一般  (GK05010E) ,  腫ようの外科療法  (GE03033T) ,  免疫療法薬・血液製剤の臨床への応用  (GW22020O)

タイトルに関連する用語 (5件)： 切断修復 ,  障害 ,  DNA ,  造血幹細胞移植 ,  転帰