{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,31]],"date-time":"2025-10-31T17:02:35Z","timestamp":1761930155744,"version":"3.37.3"},"reference-count":25,"publisher":"Springer Science and Business Media LLC","issue":"4","license":[{"start":{"date-parts":[[2021,1,3]],"date-time":"2021-01-03T00:00:00Z","timestamp":1609632000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2021,1,3]],"date-time":"2021-01-03T00:00:00Z","timestamp":1609632000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Int J Hematol"],"published-print":{"date-parts":[[2021,4]]},"DOI":"10.1007\/s12185-020-03046-x","type":"journal-article","created":{"date-parts":[[2021,1,3]],"date-time":"2021-01-03T06:03:08Z","timestamp":1609653788000},"page":"606-610","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["A case of JAK2V617F-positive essential thrombocythemia where allele burden was reduced by a PD-1 inhibitor"],"prefix":"10.1007","volume":"113","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0324-898X","authenticated-orcid":false,"given":"Ryujiro","family":"Hara","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroshi","family":"Kawada","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yara Yukie","family":"Kikuti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eri","family":"Kikkawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kaito","family":"Harada","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasuyuki","family":"Aoyama","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daisuke","family":"Ogiya","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masako","family":"Toyosaki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rikio","family":"Suzuki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sinichiro","family":"Machida","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ken","family":"Ohmachi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Makoto","family":"Onizuka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshiaki","family":"Ogawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryota","family":"Masuda","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masayuki","family":"Iwazaki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naoya","family":"Nakamura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kiyoshi","family":"Ando","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,1,3]]},"reference":[{"key":"3046_CR1","doi-asserted-by":"publisher","first-page":"2507","DOI":"10.1182\/blood-2014-05-579136","volume":"124","author":"A Tefferi","year":"2014","unstructured":"Tefferi A, Guglielmelli P, Larson DR, Finke C, Wassie EA, Pieri L, et al. Long-term survival and blast transformation in molecularly annotated essential thrombocythemia, polycythemia vera, and myelofibrosis. Blood. 2014;124:2507\u201313.","journal-title":"Blood"},{"key":"3046_CR2","doi-asserted-by":"publisher","first-page":"1627","DOI":"10.1056\/NEJMoa1507643","volume":"373","author":"H Borghaei","year":"2015","unstructured":"Borghaei H, Paz-Ares L, Horn L, Spigel DR, Steins M, Ready NE, et al. Nivolumab versus docetaxel in advanced nonsquamous non-small-cell lung cancer. N Engl J Med. 2015;373:1627\u201339.","journal-title":"N Engl J Med"},{"key":"3046_CR3","doi-asserted-by":"publisher","first-page":"1856","DOI":"10.1056\/NEJMoa1602252","volume":"375","author":"RL Ferris","year":"2016","unstructured":"Ferris RL, Blumenschein G Jr, Fayette J, Guigay J, Colevas AD, Licitra L, et al. Nivolumab for recurrent squamous-cell carcinoma of the head and neck. N Engl J Med. 2016;375:1856\u201367.","journal-title":"N Engl J Med"},{"key":"3046_CR4","doi-asserted-by":"publisher","first-page":"eaam7729","DOI":"10.1126\/scitranslmed.aam7729","volume":"10","author":"A Prestipino","year":"2018","unstructured":"Prestipino A, Emhardt AJ, Aumann K, O\u2019Sullivan D, Gorantla SP, Duquesne S, et al. Oncogenic JAK2(V617F) causes PD-L1 expression, mediating immune escape in myeloproliferative neoplasms. Sci Transl Med. 2018;10:eaam7729.","journal-title":"Sci Transl Med"},{"key":"3046_CR5","doi-asserted-by":"publisher","first-page":"1021","DOI":"10.3390\/cancers12041021","volume":"12","author":"E Hadzijusufovic","year":"2020","unstructured":"Hadzijusufovic E, Keller A, Berger D, Greiner G, Wingelhofer B, Witzeneder N, et al. STAT5 is expressed in CD34(+)\/CD38(-) stem cells and serves as a potential molecular target in ph-negative myeloproliferative neoplasms. Cancers (Basel). 2020;12:1021.","journal-title":"Cancers (Basel)"},{"key":"3046_CR6","doi-asserted-by":"publisher","first-page":"152","DOI":"10.31768\/2312-8852.2018.40(2):152-152","volume":"40","author":"E Atkinson","year":"2018","unstructured":"Atkinson E, Bakri M, Hayat A, Langabeer SE. Anagrelide and the CALR mutation allele burden in essential thrombocythemia. Exp Oncol. 2018;40:152\u20133.","journal-title":"Exp Oncol"},{"key":"3046_CR7","first-page":"2687","volume":"9","author":"N Cascavilla","year":"2015","unstructured":"Cascavilla N, De Stefano V, Pane F, Pancrazzi A, Iurlo A, Gobbi M, et al. Impact of JAK2(V617F) mutation status on treatment response to anagrelide in essential thrombocythemia: an observational, hypothesis-generating study. Drug Des Devel Ther. 2015;9:2687\u201394.","journal-title":"Drug Des Devel Ther"},{"key":"3046_CR8","doi-asserted-by":"publisher","first-page":"e18","DOI":"10.3324\/haematol.2010.037846","volume":"96","author":"IR Zalcberg","year":"2011","unstructured":"Zalcberg IR, Ayres-Silva J, de Azevedo AM, Solza C, Daumas A, Bonamino M. Hydroxyurea dose impacts hematologic parameters in polycythemia vera and essential thrombocythemia but does not appreciably affect JAK2-V617F allele burden. Haematologica. 2011;96:e18-20.","journal-title":"Haematologica"},{"key":"3046_CR9","doi-asserted-by":"publisher","first-page":"1435","DOI":"10.3324\/haematol.2009.021444","volume":"95","author":"E Antonioli","year":"2010","unstructured":"Antonioli E, Carobbio A, Pieri L, Pancrazzi A, Guglielmelli P, Delaini F, et al. Hydroxyurea does not appreciably reduce JAK2 V617F allele burden in patients with polycythemia vera or essential thrombocythemia. Haematologica. 2010;95:1435\u20138.","journal-title":"Haematologica"},{"key":"3046_CR10","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1179\/102453309X385188","volume":"14","author":"TS Larsen","year":"2009","unstructured":"Larsen TS, Pallisgaard N, de Stricker K, M\u00f8ller MB, Hasselbalch HC. Limited efficacy of hydroxyurea in lowering of the JAK2 V617F allele burden. Hematology. 2009;14:11\u20135.","journal-title":"Hematology"},{"key":"3046_CR11","doi-asserted-by":"publisher","first-page":"360","DOI":"10.1186\/s12967-018-1729-7","volume":"16","author":"G Greenfield","year":"2018","unstructured":"Greenfield G, McPherson S, Mills K, McMullin MF. The ruxolitinib effect: understanding how molecular pathogenesis and epigenetic dysregulation impact therapeutic efficacy in myeloproliferative neoplasms. J Transl Med. 2018;16:360.","journal-title":"J Transl Med"},{"key":"3046_CR12","doi-asserted-by":"publisher","first-page":"639","DOI":"10.1111\/bjh.13535","volume":"172","author":"A Angona","year":"2016","unstructured":"Angona A, Alvarez-Larran A, Bellosillo B, Longaron R, Fernandez-Rodriguez C, Besses C. Dynamics of JAK2 V617F allele burden of CD34+ haematopoietic progenitor cells in patients treated with ruxolitinib. Br J Haematol. 2016;172:639\u201342.","journal-title":"Br J Haematol"},{"key":"3046_CR13","doi-asserted-by":"publisher","first-page":"1526","DOI":"10.1038\/bmt.2017.157","volume":"52","author":"C Wolschke","year":"2017","unstructured":"Wolschke C, Badbaran A, Zabelina T, Christopeit M, Ayuk F, Triviai I, et al. Impact of molecular residual disease post allografting in myelofibrosis patients. Bone Marrow Transplant. 2017;52:1526\u20139.","journal-title":"Bone Marrow Transplant"},{"key":"3046_CR14","doi-asserted-by":"publisher","first-page":"212","DOI":"10.3389\/fonc.2014.00212","volume":"4","author":"SE Langabeer","year":"2014","unstructured":"Langabeer SE, Haslam K, Conneally E. Monitoring residual disease in the ph-negative myeloproliferative neoplasms post-allogeneic stem cell transplantation: more mutations and more methodologies. Front Oncol. 2014;4:212.","journal-title":"Front Oncol"},{"key":"3046_CR15","doi-asserted-by":"publisher","first-page":"722","DOI":"10.3324\/haematol.2012.076901","volume":"98","author":"T Lange","year":"2013","unstructured":"Lange T, Edelmann A, Siebolts U, Krahl R, Nehring C, Jakel N, et al. JAK2 p.V617F allele burden in myeloproliferative neoplasms one month after allogeneic stem cell transplantation significantly predicts outcome and risk of relapse. Haematologica. 2013;98:722\u20138.","journal-title":"Haematologica"},{"key":"3046_CR16","doi-asserted-by":"publisher","first-page":"495","DOI":"10.1038\/leu.2016.290","volume":"31","author":"MO Holmstrom","year":"2017","unstructured":"Holmstrom MO, Hjortso MD, Ahmad SM, Met O, Martinenaite E, Riley C, et al. The JAK2V617F mutation is a target for specific T cells in the JAK2V617F-positive myeloproliferative neoplasms. Leukemia. 2017;31:495\u20138.","journal-title":"Leukemia"},{"key":"3046_CR17","doi-asserted-by":"publisher","first-page":"2264","DOI":"10.3389\/fimmu.2018.02264","volume":"9","author":"U Klausen","year":"2018","unstructured":"Klausen U, Holmberg S, Holmstr\u00f6m MO, J\u00f8rgensen NGD, Grauslund JH, Svane IM, et al. Novel strategies for peptide-based vaccines in hematological malignancies. Front Immunol. 2018;9:2264.","journal-title":"Front Immunol"},{"key":"3046_CR18","doi-asserted-by":"publisher","first-page":"1000","DOI":"10.1038\/leu.2017.6","volume":"31","author":"G Nishanth","year":"2017","unstructured":"Nishanth G, Wolleschak D, Fahldieck C, Fischer T, Mullally A, Perner F, et al. Gain of function in Jak 2(V617F)-positive T-cells. Leukemia. 2017;31:1000\u20133.","journal-title":"Leukemia"},{"key":"3046_CR19","doi-asserted-by":"publisher","first-page":"1559","DOI":"10.3390\/cells9061559","volume":"9","author":"LM Braun","year":"2020","unstructured":"Braun LM, Zeiser R. Immunotherapy in myeloproliferative diseases. Cells. 2020;9:1559.","journal-title":"Cells"},{"key":"3046_CR20","doi-asserted-by":"publisher","first-page":"310","DOI":"10.1007\/s11899-019-00527-7","volume":"14","author":"L Masarova","year":"2019","unstructured":"Masarova L, Bose P, Verstovsek S. The rationale for immunotherapy in myeloproliferative neoplasms. Curr Hematol Malig Rep. 2019;14:310\u201327.","journal-title":"Curr Hematol Malig Rep"},{"key":"3046_CR21","doi-asserted-by":"publisher","first-page":"52","DOI":"10.1016\/j.leukres.2019.02.010","volume":"79","author":"JC Wang","year":"2019","unstructured":"Wang JC, Chen C, Kundra A, Kodali S, Pandey A, Wong C, et al. Programmed cell death receptor (PD-1) ligand (PD-L1) expression in Philadelphia chromosome-negative myeloproliferative neoplasms. Leuk Res. 2019;79:52\u20139.","journal-title":"Leuk Res"},{"key":"3046_CR22","doi-asserted-by":"publisher","first-page":"1192","DOI":"10.1158\/2159-8290.CD-18-1356","volume":"9","author":"C Cimen Bozkus","year":"2019","unstructured":"Cimen Bozkus C, Roudko V, Finnigan JP, Mascarenhas J, Hoffman R, Iancu-Rubin C, et al. Immune checkpoint blockade enhances shared neoantigen-induced t-cell immunity directed against mutated calreticulin in myeloproliferative neoplasms. Cancer Discov. 2019;9:1192\u2013207.","journal-title":"Cancer Discov"},{"key":"3046_CR23","doi-asserted-by":"publisher","first-page":"143","DOI":"10.1056\/NEJMoa1601202","volume":"375","author":"MS Davids","year":"2016","unstructured":"Davids MS, Kim HT, Bachireddy P, Costello C, Liguori R, Savell A, et al. Ipilimumab for patients with relapse after allogeneic transplantation. N Engl J Med. 2016;375:143\u201353.","journal-title":"N Engl J Med"},{"key":"3046_CR24","doi-asserted-by":"publisher","first-page":"1763","DOI":"10.3390\/cancers12071763","volume":"12","author":"MO Holmstr\u00f6m","year":"2020","unstructured":"Holmstr\u00f6m MO, Hasselbalch HC, Andersen MH. Cancer immune therapy for Philadelphia chromosome-negative chronic myeloproliferative neoplasms. Cancers (Basel). 2020;12:1763.","journal-title":"Cancers (Basel)"},{"key":"3046_CR25","doi-asserted-by":"publisher","first-page":"3784","DOI":"10.1182\/blood-2013-07-515676","volume":"122","author":"K Takahashi","year":"2013","unstructured":"Takahashi K, Patel KP, Kantarjian H, Luthra R, Pierce S, Cortes J, et al. JAK2 p.V617F detection and allele burden measurement in peripheral blood and bone marrow aspirates in patients with myeloproliferative neoplasms. Blood. 2013;122:3784\u20136.","journal-title":"Blood"}],"container-title":["International Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s12185-020-03046-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s12185-020-03046-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s12185-020-03046-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,3,22]],"date-time":"2021-03-22T18:28:12Z","timestamp":1616437692000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s12185-020-03046-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1,3]]},"references-count":25,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2021,4]]}},"alternative-id":["3046"],"URL":"https:\/\/doi.org\/10.1007\/s12185-020-03046-x","relation":{},"ISSN":["0925-5710","1865-3774"],"issn-type":[{"type":"print","value":"0925-5710"},{"type":"electronic","value":"1865-3774"}],"subject":[],"published":{"date-parts":[[2021,1,3]]},"assertion":[{"value":"26 August 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"14 November 2020","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"17 November 2020","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"3 January 2021","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with ethical standards"}},{"value":"The authors declare that they have no conflicts of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"This case report was carried out following the Code of Ethics of the World Medical Association (Declaration of Helsinki).","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Human subjects"}}]}}