{"created":"2023-06-19T10:29:33.860474+00:00","id":1670,"links":{},"metadata":{"_buckets":{"deposit":"a6662488-73ca-4e1b-b319-7048e80f81bf"},"_deposit":{"created_by":31,"id":"1670","owners":[31],"pid":{"revision_id":0,"type":"depid","value":"1670"},"status":"published"},"_oai":{"id":"oai:kwmed.repo.nii.ac.jp:00001670","sets":["1709617079800:35:317:562"]},"author_link":["110987","110988","110989","110990","110991","110992","110993","110994","110995","110996","110997","110998","110999","111000","111001","111002","111003","111004","111005","111006"],"item_1694495855422":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_version_type":"VoR"}]},"item_3_biblio_info_12":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2015","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"1","bibliographicPageEnd":"26","bibliographicPageStart":"19","bibliographicVolumeNumber":"41","bibliographic_titles":[{"bibliographic_title":"川崎医学会誌","bibliographic_titleLang":"ja"},{"bibliographic_title":"Kawasaki medical journal","bibliographic_titleLang":"en"}]}]},"item_3_description_8":{"attribute_name":"記事種別(日)","attribute_value_mlt":[{"subitem_description":"症例報告","subitem_description_language":"ja","subitem_description_type":"Other"}]},"item_3_description_9":{"attribute_name":"記事種別(英)","attribute_value_mlt":[{"subitem_description":"Case 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of Pathology1, Kawasaki medical school"},{"subitem_text_language":"en","subitem_text_value":"Department of Hematology, Kawasaki medical school"},{"subitem_text_language":"en","subitem_text_value":"Department of Hematology, Kawasaki medical school"},{"subitem_text_language":"en","subitem_text_value":"Department of Internal Medicine, Division of Hematology, Shinshu University School of Medicine"},{"subitem_text_language":"en","subitem_text_value":"Department of Internal Medicine, Division of Hematology, Shinshu University School of Medicine"},{"subitem_text_language":"en","subitem_text_value":"Department of Pathology1, Kawasaki medical school"},{"subitem_text_language":"en","subitem_text_value":"Department of Pathology1, Kawasaki medical school"},{"subitem_text_language":"en","subitem_text_value":"Department of Hematology, Kawasaki medical school"},{"subitem_text_language":"en","subitem_text_value":"Department of Hematology, Kawasaki medical school"},{"subitem_text_language":"en","subitem_text_value":"Department of Pathology1, Kawasaki medical school"}]},"item_3_textarea_10":{"attribute_name":"抄録(日)","attribute_value_mlt":[{"subitem_textarea_language":"ja","subitem_textarea_value":"T-cell large granular lymphocyte leukemia は長期(6か月以上)にわたる末梢血中の著明な大顆粒リンパ球(large granular lymphocyte;以下,LGL)のモノクローナルな増加によって特徴づけられる疾患で,しばしば赤芽球癆を伴うことが知られている.今回,我々はHCV陽性肝硬変患者に赤芽球癆を合併したT-LGLの1例を経験した.末梢血および骨髄塗抹標本では細胞質内に微細なアズール顆粒を有し,核異型を示すリンパ球の増加がみられ,末梢血および骨髄のフローサイトメトリーおよび骨髄吸引クロット標本の免疫組織化学で,CD3,CD8,CD57陽性リンパ球の増加が確認された.骨髄細胞のPCRではTCRβの再構成を認めず,TCRγおよびTCRδの再構成がみられた.またプレドニゾロン治療にてCD57陽性リンパ球の減少および赤芽球造血の回復が確認されたことから,赤芽球癆を合併したγδT-LGLと診断した.最近,T-LGLにはSTAT3あるいはSTAT5bのSHドメインの遺伝子変異が高頻度にみられることが報告されているが,本症例においては,これらの遺伝子変異は確認できなかった."}]},"item_3_textarea_11":{"attribute_name":"抄録(英)","attribute_value_mlt":[{"subitem_textarea_language":"en","subitem_textarea_value":"T-cell large granular lymphocytic leukemia (T-LGL) is characterized by marked increase of monoclonal large granular lymphocytes (LGL) in the peripheral blood over the long term (6 months or more). It has been reported about 20% cases of T-LGL cases are associated with pure red cell aplasia (PRCA). Here, we describe a case of T-LGL associated with PRCA. This case was characterized by increase in the number of CD3+,CD8+,CD57+, and granzyme B-positive lymphocytes with fine azurophilic cytoplasmic granules and nuclear atypia in peripheral blood and bone marrow. The patient was diagnosed havingγδT-LGL because T-cell receptor (TCR)γ and TCRδ gene but not TCRβ gene rearrangement was detected by the PCR of the bone marrow cells. Prednisolone administration decreased in number of the LGL cells, accompanying recover of erythropoiesis. Although somatic mutations in the Src homology 2 domain of STAT3 or STAT5b gene are reported in 70% percent of the T-LGL with PRCA, such STAT mutations could not be detected in this case."}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"藤原, 英世","creatorNameLang":"ja"},{"creatorName":"フジワラ, ヒデヨ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"薄, 聖子","creatorNameLang":"ja"},{"creatorName":"ススキ, セイコ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"近藤, 敏範","creatorNameLang":"ja"},{"creatorName":"コンドウ, トシノリ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"関口, 和","creatorNameLang":"ja"},{"creatorName":"セキグチ, ノドカ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"石田, 文宏","creatorNameLang":"ja"},{"creatorName":"イシダ, フミヒロ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"岡, 大五","creatorNameLang":"ja"},{"creatorName":"オカ, ダイゴ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"是澤, 里紗","creatorNameLang":"ja"},{"creatorName":"コレサワ, リサ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"和田, 秀穂","creatorNameLang":"ja"},{"creatorName":"ワダ, ヒデホ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"杉原, 尚","creatorNameLang":"ja"},{"creatorName":"スギハラ, タカシ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"定平, 吉都","creatorNameLang":"ja"},{"creatorName":"サダヒラ, ヨシト","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Fujiwara, Hideyo","creatorNameLang":"en"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Susuki, Seiko","creatorNameLang":"en"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Kondo, Toshinori","creatorNameLang":"en"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Sekiguchi, Nodoka","creatorNameLang":"en"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Ishida, Fumihiro","creatorNameLang":"en"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Oka, Daigo","creatorNameLang":"en"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Koresawa, Risa","creatorNameLang":"en"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Wada, Hideho","creatorNameLang":"en"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Sugihara, Takashi","creatorNameLang":"en"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Sadahira, Yoshito","creatorNameLang":"en"}],"nameIdentifiers":[{}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2017-01-23"}],"displaytype":"detail","filename":"KJ00009881119.pdf","filesize":[{"value":"1.3 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"KJ00009881119.pdf","objectType":"fulltext","url":"https://kwmed.repo.nii.ac.jp/record/1670/files/KJ00009881119.pdf"},"version_id":"280c4de7-2e61-4cfe-86a2-f25b1ab083af"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"大顆粒リンパ球性白血病","subitem_subject_language":"ja","subitem_subject_scheme":"Other"},{"subitem_subject":"赤芽球癆","subitem_subject_language":"ja","subitem_subject_scheme":"Other"},{"subitem_subject":"STAT3変異","subitem_subject_language":"ja","subitem_subject_scheme":"Other"},{"subitem_subject":"Large granular lymphocyte","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"Leukemia","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"Pure red cell aplasia","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"STAT3 mutation","subitem_subject_language":"en","subitem_subject_scheme":"Other"}]},"item_language":{"attribute_name":"言語","attribute_value_mlt":[{"subitem_language":"jpn"}]},"item_resource_type":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"resourcetype":"journal article","resourceuri":"http://purl.org/coar/resource_type/c_6501"}]},"item_title":"赤芽球癆を合併したT-cell large granular lymphocyte leukemia の一例","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"赤芽球癆を合併したT-cell large granular lymphocyte leukemia の一例","subitem_title_language":"ja"},{"subitem_title":"T-cell large granular lymphocyte leukemia with pure red cell aplasia","subitem_title_language":"en"}]},"item_type_id":"3","owner":"31","path":["562"],"pubdate":{"attribute_name":"PubDate","attribute_value":"2017-01-23"},"publish_date":"2017-01-23","publish_status":"0","recid":"1670","relation_version_is_last":true,"title":["赤芽球癆を合併したT-cell large granular lymphocyte leukemia の一例"],"weko_creator_id":"31","weko_shared_id":-1},"updated":"2023-10-11T01:35:54.677999+00:00"}