[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"doc-seo-379210-105":3,"detail-sidebar-cat-0-en-105":81,"doc-detail-379210-en":131},{"code":4,"msg":5,"data":6},0,"ok",{"site_id":7,"language":8,"slug":9,"title":10,"keywords":11,"description":12,"schema_data":13,"social_meta":74,"head_meta":76,"extra_data":78,"updated_unix":80},105,"en","revisiting-circulating-extracellular-matrix-fragments-as-disease-markers-in-myelofibrosis-and-related-neoplasms-review","Revisiting Circulating Extracellular Matrix Fragments as Disease Markers in Myelofibrosis and Related Neoplasms - Review","","Philadelphia chromosome-negative chronic myeloproliferative neoplasms develop over 10–30 years from early disease stages toward myelofibrosis and bone marrow failure. JAK2V617F is the most prevalent driver mutation, and chronic inflammation fuels both initiation and progression through persistent connective tissue remodeling. The resulting extracellular matrix accumulation contributes to organ dysfunction. This review synthesizes prior evidence on circulating ECM fragments, framing them as biologically plausible, noninvasive biomarkers for diagnosis, treatment monitoring, and future research directions in MPN management.",{"@graph":14,"@context":73},[15,34,56],{"@type":16,"itemListElement":17},"BreadcrumbList",[18,23,27,31],{"item":19,"name":20,"@type":21,"position":22},"https://docshare.wps.com","Home","ListItem",1,{"item":24,"name":25,"@type":21,"position":26},"https://docshare.wps.com/document/","Document",2,{"item":28,"name":29,"@type":21,"position":30},"https://docshare.wps.com/document/healthcare/","Healthcare",3,{"item":32,"name":10,"@type":21,"position":33},"https://docshare.wps.com/document/revisiting-circulating-extracellular-matrix-fragments-as-disease-markers-in-myelofibrosis-and-related-neoplasms-review/379210/",4,{"url":32,"name":10,"@type":35,"image":36,"author":41,"headline":10,"publisher":44,"fileFormat":47,"inLanguage":8,"description":12,"dateModified":48,"datePublished":49,"encodingFormat":47,"isAccessibleForFree":50,"interactionStatistic":51},"DigitalDocument",{"url":37,"@type":38,"width":39,"height":40},"https://docshare.wps.com/thumbnails/revisiting-circulating-extracellular-matrix-fragments-as-disease-markers-in-myelofibrosis-and-related-neoplasms-review/379210.png","ImageObject",300,407,{"name":42,"@type":43},"Rizky","Person",{"url":19,"name":45,"@type":46},"DocShare","Organization","application/pdf","2026-10-27","2026-09-24",true,{"@type":52,"interactionType":53,"userInteractionCount":55},"InteractionCounter",{"@type":54},"ViewAction",14,{"@type":57,"mainEntity":58},"FAQPage",[59,65,69],{"name":60,"@type":61,"acceptedAnswer":62},"What drives the development and progression of Philadelphia chromosome-negative MPNs?","Question",{"text":63,"@type":64},"These cancers arise from acquired somatic driver mutations and evolve over decades. Chronic inflammation is a key pathogenetic factor that contributes to disease progression via persistent connective tissue remodeling.","Answer",{"name":66,"@type":61,"acceptedAnswer":67},"What is the role of circulating extracellular matrix (ECM) fragments in MPNs?",{"text":68,"@type":64},"The review focuses on circulating ECM fragments as biologically plausible, noninvasive disease markers. They are considered for diagnosis, treatment and monitoring to reflect fibrotic progression and disease dynamics.",{"name":70,"@type":61,"acceptedAnswer":71},"Which driver mutation is most prevalent in MPNs?",{"text":72,"@type":64},"JAK2V617F is the most prevalent driver mutation. It occurs in virtually all patients with polycythemia vera and in about half of patients with essential thrombocythemia or primary myelofibrosis.","https://schema.org",{"og:url":32,"og:type":75,"og:title":10,"og:site_name":45,"og:description":12},"article",{"robots":77,"canonical":32},"index,follow",{"doc_id":79,"site_id":7},379210,1790318476,{"code":4,"msg":82,"data":83},"success",[84,88,92,96,101,106,110,115,120,123,127],{"id":22,"doc_module":4,"doc_module_name":25,"category_name":85,"show_sort_weight":86,"slug":87},"Story & Novel",90,"story-novel",{"id":26,"doc_module":4,"doc_module_name":25,"category_name":89,"show_sort_weight":90,"slug":91},"Literature",80,"literature",{"id":33,"doc_module":4,"doc_module_name":25,"category_name":93,"show_sort_weight":94,"slug":95},"Exam",70,"exam",{"id":97,"doc_module":4,"doc_module_name":25,"category_name":98,"show_sort_weight":99,"slug":100},5,"Comic",60,"comic",{"id":102,"doc_module":4,"doc_module_name":25,"category_name":103,"show_sort_weight":104,"slug":105},6,"Technology",50,"technology",{"id":107,"doc_module":4,"doc_module_name":25,"category_name":29,"show_sort_weight":108,"slug":109},7,40,"healthcare",{"id":111,"doc_module":4,"doc_module_name":25,"category_name":112,"show_sort_weight":113,"slug":114},8,"Research & Report",30,"research-report",{"id":116,"doc_module":4,"doc_module_name":25,"category_name":117,"show_sort_weight":118,"slug":119},9,"Religion & Spirituality",20,"religion-spirituality",{"id":118,"doc_module":4,"doc_module_name":25,"category_name":121,"show_sort_weight":118,"slug":122},"World Cup","world-cup",{"id":124,"doc_module":4,"doc_module_name":25,"category_name":125,"show_sort_weight":124,"slug":126},10,"Lifestyle","lifestyle",{"id":128,"doc_module":4,"doc_module_name":25,"category_name":129,"show_sort_weight":97,"slug":130},19,"General","general",{"code":4,"msg":82,"data":132},{"doc_id":79,"user_id":133,"nickname":42,"user_avatar":134,"doc_module":4,"category_id":107,"category_name":29,"doc_title":10,"doc_description":12,"doc_content":135,"file_id":136,"file_url":137,"file_type":138,"file_size":139,"view_count":55,"is_deleted":4,"is_public":22,"is_downloadable":22,"audit_status":22,"page_count":140,"language":141,"language_code":8,"site_id":7,"html_lang":8,"table_of_contents":142,"faqs":143,"seo_title":144,"seo_description":12,"update_tm":145,"read_time":146},962085564807,"https://ap-avatar.wpscdn.com/davatar_6f874abed73319feea01a86fa6f0fab8","cancers   \nReview  \nRevisiting Circulating Extracellular Matrix Fragments  \nas Disease Markers in Myelofibrosis and Related Neoplasms  \nHans Carl Hasselbalch 1, *, Peter Junker 2, Vibe Skov 1, Lasse Kjær 1, Trine A. Knudsen 1, Morten Kranker Larsen 1, Morten Orebo Holmström 3, Mads Hald Andersen 3, Christina Jensen 4, Morten A. Karsdal 4 and Nicholas Willumsen 4  \nCitation: Hasselbalch, H.C.; Junker, P.; Skov, V.; Kjær, L.; Knudsen, T.A.; Larsen, M.K.; Holmström, M.O.; Andersen, M.H.; Jensen, C.; Karsdal, M.A.; et al. Revisiting Circulating Extracellular Matrix Fragments as Disease Markers in Myelofibrosis and Related Neoplasms. Cancers 2023, 15, 4323. [https://doi.org/10.3390/](https://doi.org/10.3390/)[ ](https://doi.org/10.3390/)cancers15174323  \nAcademic Editor: Elisabetta Abruzzese  \nReceived: 25 June 2023  \nRevised: 4 August 2023  \nAccepted: 7 August 2023  \nPublished: 29 August 2023  \nCopyright: © 2023 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license ([https://](https://)[ ](https://)[creativecommons.org/licenses/by/](creativecommons.org/licenses/by/)[ ](creativecommons.org/licenses/by/)[4.0/](4.0/)) .  \n1 Department of Hematology, Zealand University Hospital, 4000 Roskilde, Denmark;  \n[vihs@regionsjaelland.dk](vihs@regionsjaelland.dk) (V.S.); [laskj@regionsjaelland.dk](laskj@regionsjaelland.dk) (L.K.); [trak@regionsjaelland.dk](trak@regionsjaelland.dk) (T.A.K.);  \n[mokl@regionsjaelland.dk](mokl@regionsjaelland.dk) (M.K.L.)  \n2 Department of Rheumatology, Odense University Hospital, 5000 Odense, Denmark; [peter.junker@rsyd.dk](peter.junker@rsyd.dk)  \n3 National Center for Cancer Immune Therapy, Herlev Hospital, 2730 Herlev, Denmark; [morten.orebo.holmstroem@regionh.dk](morten.orebo.holmstroem@regionh.dk) (M.O.H.); [mads.hald.andersen@regionh.dk](mads.hald.andersen@regionh.dk) (M.H.A.)  \n4 Nordic Bioscience A/S, 2730 Herlev, Denmark; [chj@nordicbio.com](chj@nordicbio.com) (C.J.); [mk@nordicbio.com](mk@nordicbio.com) (M.A.K.); [nwi@nordicbio.com](nwi@nordicbio.com) (N.W.)  \n* [Correspondence: hans.hasselbalch@gmail.com](Correspondence: hans.hasselbalch@gmail.com); Tel.: +45-26223678  \nSimple Summary: MPN blood cancers are characterized by elevated blood cell counts. Chronic inflammation drives MPNs, which after 10–30 years, may lead to bone marrow failure due to replacement of bone marrow by connective tissue. Chronic inflammation also impacts other organs causing impaired vision, osteoporosis, fractures, heart failure and decreased lung and kidney function. MPNs are also associated with an increased risk of other cancers. Novel, noninvasive technologies are needed to improve the early detection of fibrosis development and MPN progression and to monitor the efficacy of treatment. Herein, we review current knowledge and focus on novel technologies for the measurement of circulating connective tissue biomarkers in MPNs. Future research directions are provided, suggesting studies on single markers or combinations before and during treatment with old and new agents, both targeting the diseased bone marrow cells and the inflammatory processes that drive the progressive fibrosis, which if left untreated, may ultimately lead to bone marrow failure or leukemic transformation.  \nAbstract: Philadelphia chromosome-negative chronic myeloproliferative neoplasms (MPNs) arise due to acquired somatic driver mutations in stem cells and develop over 10–30 years from the earliest cancer stages (essential thrombocythemia, polycythemia vera) towards the advanced myelofibrosis stage with bone marrow failure. The JAK2V617F mutation is the most prevalent driver mutation. Chronic inflammation is considered to be a major pathogenetic player, both as a trigger of MPN development and as a driver of disease progression. Chronic inflammation in MPNs is characterized by persistent connective tissue remodeling, which leads to organ","cbCaieB0J6tLwMYq","https://ap.wps.com/l/cbCaieB0J6tLwMYq","pdf",2288027,47,"English","# Introduction\n## Chronic Philadelphia-negative MPNs and driver mutations\n## Role of chronic inflammation and ECM remodeling\n## Circulating ECM fragments as diagnostic and monitoring biomarkers","[{\"question\":\"What drives the development and progression of Philadelphia chromosome-negative MPNs?\",\"answer\":\"These cancers arise from acquired somatic driver mutations and evolve over decades. Chronic inflammation is a key pathogenetic factor that contributes to disease progression via persistent connective tissue remodeling.\"},{\"question\":\"What is the role of circulating extracellular matrix (ECM) fragments in MPNs?\",\"answer\":\"The review focuses on circulating ECM fragments as biologically plausible, noninvasive disease markers. They are considered for diagnosis, treatment and monitoring to reflect fibrotic progression and disease dynamics.\"},{\"question\":\"Which driver mutation is most prevalent in MPNs?\",\"answer\":\"JAK2V617F is the most prevalent driver mutation. It occurs in virtually all patients with polycythemia vera and in about half of patients with essential thrombocythemia or primary myelofibrosis.\"}]","Revisiting Circulating Extracellular Matrix Fragments as Disease Markers in Myelofibrosis and Related Neoplasms - Review | PDF",1790234621,118]