{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T20:08:49Z","timestamp":1791576529915,"version":"4.3.4"},"reference-count":38,"publisher":"Springer Science and Business Media LLC","issue":"8","license":[{"start":{"date-parts":[[2023,4,4]],"date-time":"2023-04-04T00:00:00Z","timestamp":1680566400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2023,4,4]],"date-time":"2023-04-04T00:00:00Z","timestamp":1680566400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100005677","name":"Jos\u00e9 Carreras Leuk\u00e4mie-Stiftung","doi-asserted-by":"publisher","award":["DJCLS 08R\/2020"],"award-info":[{"award-number":["DJCLS 08R\/2020"]}],"id":[{"id":"10.13039\/501100005677","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002428","name":"Austrian Science Fund","doi-asserted-by":"publisher","award":["SFB F4704-B20"],"award-info":[{"award-number":["SFB F4704-B20"]}],"id":[{"id":"10.13039\/501100002428","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Medizinische Fakult\u00e4t Mannheim der Universit\u00e4t Heidelberg"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Ann Hematol"],"published-print":{"date-parts":[[2023,8]]},"abstract":"<jats:title>Abstract\n<\/jats:title><jats:p>We sought to evaluate the efficacy of the purine analogue cladribine in 79 patients with advanced systemic mastocytosis (AdvSM) using data from the \u2018German Registry on Disorders of Eosinophils and Mast Cells (GREM)\u2019. The overall response rate according to modified Valent criteria (46 evaluable patients) for first- (1L) and second-line (2L) cladribine treatment was 41% (12\/29) and 35% (6\/17, <jats:italic>P<\/jats:italic>\u2009=\u20090.690), respectively, and the median overall survival (OS, all patients evaluable) was 1.9\u00a0years (<jats:italic>n<\/jats:italic>\u2009=\u200948) and 1.2\u00a0years (<jats:italic>n<\/jats:italic>\u2009=\u200931; <jats:italic>P<\/jats:italic>\u2009=\u20090.311). Univariate and multivariable analyses of baseline and on-treatment parameters identified diagnosis of mast cell leukemia (hazard ratio [HR] 3.5, 95% confidence interval [CI, 1.3\u20139.1], <jats:italic>P<\/jats:italic>\u2009=\u20090.012), eosinophilia\u2009\u2265\u20091.5\u2009\u00d7\u200910<jats:sup>9<\/jats:sup>\/L (HR 2.9 [CI 1.4\u20136.2], <jats:italic>P<\/jats:italic>\u2009=\u20090.006) and\u2009&lt;\u20093 cycles of cladribine (HR 0.4 [CI 0.2\u20130.8], <jats:italic>P<\/jats:italic>\u2009=\u20090.008) as independent adverse prognostic parameters for OS. There was no impact of other laboratory (anemia, thrombocytopenia, serum tryptase) or genetic markers (mutations in <jats:italic>SRSF2<\/jats:italic>, <jats:italic>ASXL1<\/jats:italic> or <jats:italic>RUNX1<\/jats:italic>) on OS. In consequence, none of the recently established prognostic scoring systems (MARS, IPSM, MAPS or GPSM) was predictive for OS. Modified Valent criteria were superior to a single factor-based response assessment (HR 2.9 [CI 1.3\u20136.6], <jats:italic>P<\/jats:italic>\u2009=\u20090.026). In conclusion, cladribine is effective in 1L and 2L treatment of AdvSM. Mast cell leukemia, eosinophilia, application of\u2009&lt;\u20093 cycles and a lack of response are adverse prognostic markers.<\/jats:p>","DOI":"10.1007\/s00277-023-05180-y","type":"journal-article","created":{"date-parts":[[2023,4,4]],"date-time":"2023-04-04T10:39:14Z","timestamp":1680604754000},"page":"2077-2085","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Response and resistance to cladribine in patients with advanced systemic mastocytosis: a registry-based analysis"],"prefix":"10.1007","volume":"102","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0767-1504","authenticated-orcid":false,"given":"Johannes","family":"L\u00fcbke","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Naumann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georgia","family":"Metzgeroth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastian","family":"Kreil","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timo","family":"Brand","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans-Peter","family":"Horny","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karl","family":"Sotlar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicholas C. P.","family":"Cross","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alice","family":"Fabarius","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Valent","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wolf-Karsten","family":"Hofmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas","family":"Reiter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juliana","family":"Schwaab","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,4,4]]},"reference":[{"issue":"11","key":"5180_CR1","doi-asserted-by":"publisher","first-page":"1420","DOI":"10.1182\/blood-2016-09-731893","volume":"129","author":"P Valent","year":"2017","unstructured":"Valent P, Akin C, Metcalfe DD (2017) Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts. Blood 129(11):1420\u20131427","journal-title":"Blood"},{"issue":"6","key":"5180_CR2","doi-asserted-by":"publisher","first-page":"435","DOI":"10.1111\/j.1365-2362.2007.01807.x","volume":"37","author":"P Valent","year":"2007","unstructured":"Valent P, Akin C, Escribano L et al (2007) Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria. Eur J Clin Invest 37(6):435\u2013453","journal-title":"Eur J Clin Invest"},{"issue":"5","key":"5180_CR3","doi-asserted-by":"publisher","first-page":"695","DOI":"10.1046\/j.1365-2141.2003.04575.x","volume":"122","author":"P Valent","year":"2003","unstructured":"Valent P, Akin C, Sperr WR et al (2003) Diagnosis and treatment of systemic mastocytosis: state of the art. Br J Haematol 122(5):695\u2013717","journal-title":"Br J Haematol"},{"issue":"4","key":"5180_CR4","doi-asserted-by":"publisher","first-page":"508","DOI":"10.1002\/ajh.26118","volume":"96","author":"A Pardanani","year":"2021","unstructured":"Pardanani A (2021) Systemic mastocytosis in adults: 2021 Update on diagnosis, risk stratification and management. Am J Hematol 96(4):508\u2013525","journal-title":"Am J Hematol"},{"issue":"21","key":"5180_CR5","doi-asserted-by":"publisher","first-page":"18250","DOI":"10.18632\/oncotarget.4213","volume":"6","author":"A Chatterjee","year":"2015","unstructured":"Chatterjee A, Ghosh J, Kapur R (2015) Mastocytosis: a mutated KIT receptor induced myeloproliferative disorder. Oncotarget 6(21):18250\u201318264","journal-title":"Oncotarget"},{"issue":"5","key":"5180_CR6","doi-asserted-by":"publisher","first-page":"1115","DOI":"10.1038\/leu.2015.4","volume":"29","author":"M Jawhar","year":"2015","unstructured":"Jawhar M, Schwaab J, Schnittger S et al (2015) Molecular profiling of myeloid progenitor cells in multi-mutated advanced systemic mastocytosis identifies KIT D816V as a distinct and late event. Leukemia 29(5):1115\u20131122","journal-title":"Leukemia"},{"issue":"31","key":"5180_CR7","doi-asserted-by":"publisher","first-page":"2846","DOI":"10.1200\/JCO.19.00640","volume":"37","author":"M Jawhar","year":"2019","unstructured":"Jawhar M, Schwaab J, Alvarez-Twose I et al (2019) MARS: mutation-adjusted risk score for advanced systemic mastocytosis. J Clin Oncol 37(31):2846\u20132856","journal-title":"J Clin Oncol"},{"issue":"1","key":"5180_CR8","doi-asserted-by":"publisher","first-page":"136","DOI":"10.1038\/leu.2015.284","volume":"30","author":"M Jawhar","year":"2016","unstructured":"Jawhar M, Schwaab J, Schnittger S et al (2016) Additional mutations in SRSF2, ASXL1 and\/or RUNX1 identify a high-risk group of patients with KIT D816V(+) advanced systemic mastocytosis. Leukemia 30(1):136\u2013143","journal-title":"Leukemia"},{"issue":"12","key":"5180_CR9","doi-asserted-by":"publisher","first-page":"2342","DOI":"10.1038\/leu.2016.190","volume":"30","author":"M Jawhar","year":"2016","unstructured":"Jawhar M, Schwaab J, Hausmann D et al (2016) Splenomegaly, elevated alkaline phosphatase and mutations in the SRSF2\/ASXL1\/RUNX1 gene panel are strong adverse prognostic markers in patients with systemic mastocytosis. Leukemia 30(12):2342\u20132350","journal-title":"Leukemia"},{"issue":"14","key":"5180_CR10","doi-asserted-by":"publisher","first-page":"2460","DOI":"10.1182\/blood-2013-04-496448","volume":"122","author":"J Schwaab","year":"2013","unstructured":"Schwaab J, Schnittger S, Sotlar K et al (2013) Comprehensive mutational profiling in advanced systemic mastocytosis. Blood 122(14):2460\u20132466","journal-title":"Blood"},{"issue":"3","key":"5180_CR11","doi-asserted-by":"publisher","first-page":"e194","DOI":"10.1016\/S2352-3026(20)30400-2","volume":"8","author":"JI Mu\u00f1oz-Gonz\u00e1lez","year":"2021","unstructured":"Mu\u00f1oz-Gonz\u00e1lez JI, \u00c1lvarez-Twose I, Jara-Acevedo M et al (2021) Proposed global prognostic score for systemic mastocytosis: a retrospective prognostic modelling study. Lancet Haematol 8(3):e194\u2013e204","journal-title":"Lancet Haematol"},{"issue":"21","key":"5180_CR12","doi-asserted-by":"publisher","first-page":"2964","DOI":"10.1182\/bloodadvances.2018026245","volume":"2","author":"A Pardanani","year":"2018","unstructured":"Pardanani A, Shah S, Mannelli F et al (2018) Mayo alliance prognostic system for mastocytosis: clinical and hybrid clinical-molecular models. Blood Adv 2(21):2964\u20132972","journal-title":"Blood Adv"},{"issue":"2","key":"5180_CR13","doi-asserted-by":"publisher","first-page":"470","DOI":"10.1038\/leu.2017.234","volume":"32","author":"DJ DeAngelo","year":"2018","unstructured":"DeAngelo DJ, George TI, Linder A et al (2018) Efficacy and safety of midostaurin in patients with advanced systemic mastocytosis: 10-year median follow-up of a phase II trial. Leukemia 32(2):470\u2013478","journal-title":"Leukemia"},{"issue":"26","key":"5180_CR14","doi-asserted-by":"publisher","first-page":"2530","DOI":"10.1056\/NEJMoa1513098","volume":"374","author":"J Gotlib","year":"2016","unstructured":"Gotlib J, Kluin-Nelemans HC, George TI et al (2016) Efficacy and safety of midostaurin in advanced systemic mastocytosis. N Engl J Med 374(26):2530\u20132541","journal-title":"N Engl J Med"},{"issue":"26","key":"5180_CR15","doi-asserted-by":"publisher","first-page":"2605","DOI":"10.1056\/NEJMc1515403","volume":"374","author":"MO Chandesris","year":"2016","unstructured":"Chandesris MO, Damaj G, Canioni D et al (2016) Midostaurin in advanced systemic mastocytosis. N Engl J Med 374(26):2605\u20132607","journal-title":"N Engl J Med"},{"issue":"12","key":"5180_CR16","doi-asserted-by":"publisher","first-page":"2183","DOI":"10.1038\/s41591-021-01538-9","volume":"27","author":"DJ DeAngelo","year":"2021","unstructured":"DeAngelo DJ, Radia DH, George TI et al (2021) Safety and efficacy of avapritinib in advanced systemic mastocytosis: the phase 1 EXPLORER trial. Nat Med 27(12):2183\u20132191","journal-title":"Nat Med"},{"issue":"12","key":"5180_CR17","doi-asserted-by":"publisher","first-page":"2192","DOI":"10.1038\/s41591-021-01539-8","volume":"27","author":"J Gotlib","year":"2021","unstructured":"Gotlib J, Reiter A, Radia DH et al (2021) Efficacy and safety of avapritinib in advanced systemic mastocytosis: interim analysis of the phase 2 PATHFINDER trial. Nat Med 27(12):2192\u20132199","journal-title":"Nat Med"},{"issue":"4","key":"5180_CR18","doi-asserted-by":"publisher","first-page":"975","DOI":"10.1111\/bjh.17932","volume":"196","author":"A Tefferi","year":"2021","unstructured":"Tefferi A, Kittur J, Farrukh F et al (2021) Cladribine therapy for advanced and indolent systemic mastocytosis: Mayo Clinic experience in 42 consecutive cases. Br J Haematol 196(4):975\u2013983","journal-title":"Br J Haematol"},{"issue":"8","key":"5180_CR19","doi-asserted-by":"publisher","first-page":"1009","DOI":"10.1182\/blood-2014-12-614743","volume":"126","author":"S Barete","year":"2015","unstructured":"Barete S, Lortholary O, Damaj G et al (2015) Long-term efficacy and safety of cladribine (2-CdA) in adult patients with mastocytosis. Blood 126(8):1009\u20131016","journal-title":"Blood"},{"issue":"13","key":"5180_CR20","doi-asserted-by":"publisher","first-page":"4270","DOI":"10.1182\/blood-2003-05-1699","volume":"102","author":"HC Kluin-Nelemans","year":"2003","unstructured":"Kluin-Nelemans HC, Oldhoff JM, Van Doormaal JJ et al (2003) Cladribine therapy for systemic mastocytosis. Blood 102(13):4270\u20134276","journal-title":"Blood"},{"issue":"12","key":"5180_CR21","doi-asserted-by":"publisher","first-page":"790","DOI":"10.1002\/ajh.21561","volume":"84","author":"KH Lim","year":"2009","unstructured":"Lim KH, Pardanani A, Butterfield JH, Li CY, Tefferi A (2009) Cytoreductive therapy in 108 adults with systemic mastocytosis: Outcome analysis and response prediction during treatment with interferon-alpha, hydroxyurea, imatinib mesylate or 2-chlorodeoxyadenosine. Am J Hematol 84(12):790\u2013794","journal-title":"Am J Hematol"},{"issue":"6","key":"5180_CR22","doi-asserted-by":"publisher","first-page":"1035","DOI":"10.3324\/haematol.2017.163964","volume":"102","author":"M Jawhar","year":"2017","unstructured":"Jawhar M, Schwaab J, Meggendorfer M et al (2017) The clinical and molecular diversity of mast cell leukemia with or without associated hematologic neoplasm. Haematologica 102(6):1035\u20131043","journal-title":"Haematologica"},{"issue":"16","key":"5180_CR23","doi-asserted-by":"publisher","first-page":"1783","DOI":"10.1200\/JCO.21.01849","volume":"40","author":"J L\u00fcbke","year":"2022","unstructured":"L\u00fcbke J, Schwaab J, Naumann N et al (2022) Superior efficacy of midostaurin over cladribine in advanced systemic mastocytosis: a registry-based analysis. J Clin Oncol 40(16):1783\u20131794","journal-title":"J Clin Oncol"},{"issue":"13","key":"5180_CR24","doi-asserted-by":"publisher","first-page":"1583","DOI":"10.2217\/fon-2021-1509","volume":"18","author":"H Pilkington","year":"2022","unstructured":"Pilkington H, Smith S, Roskell N, Iannazzo S (2022) Indirect treatment comparisons of avapritinib versus midostaurin for patients with advanced systemic mastocytosis. Future Oncol 18(13):1583\u20131594","journal-title":"Future Oncol"},{"issue":"8","key":"5180_CR25","doi-asserted-by":"publisher","first-page":"2108","DOI":"10.1038\/s41375-022-01615-z","volume":"36","author":"A Reiter","year":"2022","unstructured":"Reiter A, Gotlib J, \u00c1lvarez-Twose I et al (2022) Efficacy of avapritinib versus best available therapy in the treatment of advanced systemic mastocytosis. Leukemia 36(8):2108\u20132120","journal-title":"Leukemia"},{"issue":"21","key":"5180_CR26","doi-asserted-by":"publisher","first-page":"5750","DOI":"10.1182\/bloodadvances.2022007539","volume":"6","author":"A Reiter","year":"2022","unstructured":"Reiter A, Schwaab J, DeAngelo DJ et al (2022) Efficacy and safety of avapritinib in previously treated patients with advanced systemic mastocytosis. Blood Adv 6(21):5750\u20135762","journal-title":"Blood Adv"},{"issue":"2","key":"5180_CR27","doi-asserted-by":"publisher","first-page":"137","DOI":"10.1182\/blood-2017-01-764423","volume":"130","author":"M Jawhar","year":"2017","unstructured":"Jawhar M, Schwaab J, Naumann N et al (2017) Response and progression on midostaurin in advanced systemic mastocytosis: KIT D816V and other molecular markers. Blood 130(2):137\u2013145","journal-title":"Blood"},{"issue":"5","key":"5180_CR28","doi-asserted-by":"publisher","first-page":"1195","DOI":"10.1038\/s41375-019-0450-8","volume":"33","author":"J L\u00fcbke","year":"2019","unstructured":"L\u00fcbke J, Naumann N, Kluger S et al (2019) Inhibitory effects of midostaurin and avapritinib on myeloid progenitors derived from patients with KIT D816V positive advanced systemic mastocytosis. Leukemia 33(5):1195\u20131205","journal-title":"Leukemia"},{"issue":"7","key":"5180_CR29","doi-asserted-by":"publisher","first-page":"603","DOI":"10.1016\/S0145-2126(01)00038-8","volume":"25","author":"P Valent","year":"2001","unstructured":"Valent P, Horny HP, Escribano L et al (2001) Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leuk Res 25(7):603\u2013625","journal-title":"Leuk Res"},{"issue":"16","key":"5180_CR30","doi-asserted-by":"publisher","first-page":"1365","DOI":"10.1182\/blood.2019000932","volume":"135","author":"A Reiter","year":"2020","unstructured":"Reiter A, George TI, Gotlib J (2020) New developments in diagnosis, prognostication, and treatment of advanced systemic mastocytosis. Blood 135(16):1365\u20131376","journal-title":"Blood"},{"issue":"7","key":"5180_CR31","doi-asserted-by":"publisher","first-page":"1703","DOI":"10.1038\/s41375-022-01613-1","volume":"36","author":"JD Khoury","year":"2022","unstructured":"Khoury JD, Solary E, Abla O et al (2022) he 5th edition of the World Health Organization classification of haematolymphoid tumours: myeloid and histiocytic\/dendritic neoplasms. Leukemia 36(7):1703\u20131719","journal-title":"Leukemia"},{"issue":"5","key":"5180_CR32","doi-asserted-by":"publisher","first-page":"392","DOI":"10.1111\/eci.12607","volume":"46","author":"M Jawhar","year":"2016","unstructured":"Jawhar M, Schwaab J, Horny HP et al (2016) Impact of centralized evaluation of bone marrow histology in systemic mastocytosis. Eur J Clin Invest 46(5):392\u2013397","journal-title":"Eur J Clin Invest"},{"issue":"1","key":"5180_CR33","doi-asserted-by":"publisher","first-page":"81","DOI":"10.1007\/s00277-013-1964-1","volume":"93","author":"P Erben","year":"2014","unstructured":"Erben P, Schwaab J, Metzgeroth G et al (2014) The KIT D816V expressed allele burden for diagnosis and disease monitoring of systemic mastocytosis. Ann Hematol 93(1):81\u201388","journal-title":"Ann Hematol"},{"issue":"12","key":"5180_CR34","doi-asserted-by":"publisher","first-page":"e638","DOI":"10.1016\/S2352-3026(19)30166-8","volume":"6","author":"WR Sperr","year":"2019","unstructured":"Sperr WR, Kundi M, Alvarez-Twose I et al (2019) International prognostic scoring system for mastocytosis (IPSM): a retrospective cohort study. Lancet Haematol 6(12):e638\u2013e649","journal-title":"Lancet Haematol"},{"issue":"5","key":"5180_CR35","doi-asserted-by":"publisher","first-page":"2562","DOI":"10.3390\/ijms22052562","volume":"22","author":"N Naumann","year":"2021","unstructured":"Naumann N, L\u00fcbke J, Baumann S et al (2021) Adverse prognostic impact of the KIT D816V transcriptional activity in advanced systemic mastocytosis. Int J Mol Sci 22(5):2562","journal-title":"Int J Mol Sci"},{"issue":"7","key":"5180_CR36","doi-asserted-by":"publisher","first-page":"635","DOI":"10.1016\/S0145-2126(02)00168-6","volume":"27","author":"P Valent","year":"2003","unstructured":"Valent P, Akin C, Sperr WR et al (2003) Aggressive systemic mastocytosis and related mast cell disorders: current treatment options and proposed response criteria. Leuk Res 27(7):635\u2013641","journal-title":"Leuk Res"},{"issue":"4","key":"5180_CR37","doi-asserted-by":"publisher","first-page":"1090","DOI":"10.1038\/s41375-019-0632-4","volume":"34","author":"HC Kluin-Nelemans","year":"2019","unstructured":"Kluin-Nelemans HC, Reiter A, Illerhaus A et al (2019) Prognostic impact of eosinophils in mastocytosis: analysis of 2350 patients collected in the ECNM Registry. Leukemia 34(4):1090\u20131101","journal-title":"Leukemia"},{"issue":"5","key":"5180_CR38","doi-asserted-by":"publisher","first-page":"1124","DOI":"10.1038\/s41375-018-0346-z","volume":"33","author":"M Jawhar","year":"2019","unstructured":"Jawhar M, Dohner K, Kreil S et al (2019) KIT D816 mutated\/CBF-negative acute myeloid leukemia: a poor-risk subtype associated with systemic mastocytosis. Leukemia 33(5):1124\u20131134","journal-title":"Leukemia"}],"container-title":["Annals of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00277-023-05180-y.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00277-023-05180-y\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00277-023-05180-y.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,7,13]],"date-time":"2023-07-13T07:05:09Z","timestamp":1689231909000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00277-023-05180-y"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,4,4]]},"references-count":38,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2023,8]]}},"alternative-id":["5180"],"URL":"https:\/\/doi.org\/10.1007\/s00277-023-05180-y","relation":{},"ISSN":["0939-5555","1432-0584"],"issn-type":[{"value":"0939-5555","type":"print"},{"value":"1432-0584","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,4,4]]},"assertion":[{"value":"7 November 2022","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"13 March 2023","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 April 2023","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The study design adhered to the tenets of the Declaration of Helsinki and was approved by the institutional review board of the Medical Faculty of Mannheim, Heidelberg University, Germany.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Research involving human participants"}},{"value":"Written informed consent was provided by all patients.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}},{"value":"Disclosures of conflict of interest: H.-P.H. served as a consultant for Novartis and Blueprint. P.V. received a research grant from Blueprint and Celgene, served as a consultant in a midostaurin trial with Novartis, and received consultancy honoraria from Blueprint, Deciphera, Novartis, Celgene and Pfizer. A.R. was a member of the Study Steering Committee (SSC) for the global trial of midostaurin in advanced systemic mastocytosis (AdvSM) (Novartis), the Response Adjudication Committee (RAC) for studies of avapritinib in AdvSM (Blueprint Medicines), and the SSC for the phase II trial of ripretinib in AdvSM (Deciphera Pharmaceuticals); has received funding for the conduct of these trials; and has received honoraria and reimbursement of travel expenses from Novartis, Blueprint Medicines and Deciphera Pharmaceuticals. J.S. has served as a member in the advisory board of Blueprint for studies of avapritinib in indolent SM and received honoraria from Novartis.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Disclosure of potential conflicts of interest"}}]}}