{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T22:52:21Z","timestamp":1792450341236,"version":"4.5.0"},"reference-count":65,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2023,12,6]],"date-time":"2023-12-06T00:00:00Z","timestamp":1701820800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2024,2]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Mastocytosis constitutes the neoplastic proliferation of mast cells and is broadly classified into systemic mastocytosis (SM), cutaneous mastocytosis and mast cell sarcoma. SM is further partitioned into advanced (AdvSM) and non\u2010advanced (SM\u2010non\u2010Adv) subcategories. AdvSM includes aggressive SM (ASM), SM with an associated haematological neoplasm (SM\u2010AHN) and mast cell leukaemia (MCL). In 2022, two separate expert committees representing the 5th edition of the World Health Organization (WHO5) and the International Consensus (ICC) classification systems submitted revised classification criteria for SM, highlighted by the ICC\u2010proposed incorporation of mast cell cytomorphology in the diagnostic criteria for MCL and myeloid\u2010lineage restriction for the AHN component in SM\u2010AHN. Recent developments in SM also include the introduction of KIT\u2010targeting tyrosine kinase inhibitors (KITi), including midostaurin and avapritinib, both drugs have shown potent activity in reducing mast cell and mutant <jats:italic>KIT<\/jats:italic> burden and alleviating mast cell\u2010associated organopathy and mediator symptoms; however, their overall impact on survival or superiority over pre\u2010KITi era treatment options (e.g. cladribine) has not been studied in a controlled setting. In the current review, we provide a summary of recent changes in disease classification and an analysis of recent clinical trials and their impact on our current treatment approach in AdvSM.<\/jats:p>","DOI":"10.1111\/bjh.19245","type":"journal-article","created":{"date-parts":[[2023,12,6]],"date-time":"2023-12-06T10:36:59Z","timestamp":1701859019000},"page":"402-414","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Advanced systemic mastocytosis\u2014Revised classification, new drugs and how we treat"],"prefix":"10.1111","volume":"204","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9084-4148","authenticated-orcid":false,"given":"Animesh","family":"Pardanani","sequence":"first","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaaren","family":"Reichard","sequence":"additional","affiliation":[{"name":"Division of Hematopathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4605-3821","authenticated-orcid":false,"given":"Ayalew","family":"Tefferi","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,12,6]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-022-01613-1"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2022015850"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-02-205237"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/HS9.0000000000000646"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.26966"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.27113"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2018026245"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.27105"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.27089"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0145-2126(03)00259-5"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.patbio.2004.04.012"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.20211"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199202273260907"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.3109\/10428199609054789"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.1998.02131.x"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.21561"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0145-2126(02)00168-6"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00663015"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/35.9.898"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200101253440415"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.17932"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-12-614743"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2003-05-1699"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-023-05180-y"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.21.01849"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2016.04.018"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2014.55.2018"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1038\/bmt.2012.198"},{"key":"e_1_2_10_30_1","first-page":"14","article-title":"Successful outcome of allogeneic hematopoietic stem cell transplantation for systemic mastocytosis complicated with acute myeloid leukemia","volume":"63","author":"Maegaki M","year":"2022","journal-title":"Rinsho Ketsueki"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.13528"},{"key":"e_1_2_10_32_1","doi-asserted-by":"publisher","DOI":"10.3343\/alm.2013.33.2.125"},{"key":"e_1_2_10_33_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1705245"},{"key":"e_1_2_10_34_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.26857"},{"key":"e_1_2_10_35_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-05-714030"},{"key":"e_1_2_10_36_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41591-021-01538-9"},{"key":"e_1_2_10_37_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41591-021-01539-8"},{"key":"e_1_2_10_38_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-09-458521"},{"key":"e_1_2_10_39_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.HS9.0000970992.31848.76"},{"key":"e_1_2_10_40_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms22062983"},{"key":"e_1_2_10_41_1","first-page":"62","volume-title":"WHO classification of tumors of hematopoietic and lymphoid tissues","author":"Horny HP","year":"2017"},{"key":"e_1_2_10_42_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.19.00640"},{"key":"e_1_2_10_43_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2021-150872"},{"key":"e_1_2_10_44_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-10-4149"},{"key":"e_1_2_10_45_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1513098"},{"key":"e_1_2_10_46_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2017.234"},{"key":"e_1_2_10_47_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2017-01-764423"},{"key":"e_1_2_10_48_1","doi-asserted-by":"publisher","DOI":"10.1097\/HS9.0000000000000478"},{"key":"e_1_2_10_49_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.retram.2023.103398"},{"key":"e_1_2_10_50_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.15980"},{"key":"e_1_2_10_51_1","doi-asserted-by":"publisher","DOI":"10.1002\/jha2.22"},{"key":"e_1_2_10_52_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.2677"},{"key":"e_1_2_10_53_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-04-015545"},{"key":"e_1_2_10_54_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.24426"},{"key":"e_1_2_10_55_1","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.13894"},{"key":"e_1_2_10_56_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1614359"},{"key":"e_1_2_10_57_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2011.37.1286"},{"key":"e_1_2_10_58_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.21.02823"},{"key":"e_1_2_10_59_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1408617"},{"key":"e_1_2_10_60_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1409405"},{"key":"e_1_2_10_61_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2022008292"},{"key":"e_1_2_10_62_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2017.163964"},{"key":"e_1_2_10_63_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-04-1568"},{"key":"e_1_2_10_64_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.26962"},{"key":"e_1_2_10_65_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2022-156311"},{"key":"e_1_2_10_66_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2022-167410"}],"container-title":["British Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bjh.19245","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,2,7]],"date-time":"2024-02-07T06:02:48Z","timestamp":1707285768000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/bjh.19245"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,12,6]]},"references-count":65,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2024,2]]}},"alternative-id":["10.1111\/bjh.19245"],"URL":"https:\/\/doi.org\/10.1111\/bjh.19245","archive":["Portico"],"relation":{},"ISSN":["0007-1048","1365-2141"],"issn-type":[{"value":"0007-1048","type":"print"},{"value":"1365-2141","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,12,6]]},"assertion":[{"value":"2023-10-31","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-11-24","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-12-06","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}