Revisiting Circulating Extracellular Matrix Fragments as Disease Markers in Myelofibrosis and Related Neoplasms
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 mar...
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-08-01
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| Series: | Cancers |
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| Online Access: | https://www.mdpi.com/2072-6694/15/17/4323 |
| _version_ | 1797582749600579584 |
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| author | Hans Carl Hasselbalch Peter Junker Vibe Skov Lasse Kjær Trine A. Knudsen Morten Kranker Larsen Morten Orebo Holmström Mads Hald Andersen Christina Jensen Morten A. Karsdal Nicholas Willumsen |
| author_facet | Hans Carl Hasselbalch Peter Junker Vibe Skov Lasse Kjær Trine A. Knudsen Morten Kranker Larsen Morten Orebo Holmström Mads Hald Andersen Christina Jensen Morten A. Karsdal Nicholas Willumsen |
| author_sort | Hans Carl Hasselbalch |
| collection | DOAJ |
| description | 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 <i>JAK2V617F</i> 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 dysfunction and ultimately, organ failure, due to excessive accumulation of extracellular matrix (ECM). Considering that MPNs are acquired clonal stem cell diseases developing in an inflammatory microenvironment in which the hematopoietic cell populations are progressively replaced by stromal proliferation—“a wound that never heals”—we herein aim to provide a comprehensive review of previous promising research in the field of circulating ECM fragments in the diagnosis, treatment and monitoring of MPNs. We address the rationales and highlight new perspectives for the use of circulating ECM protein fragments as biologically plausible, noninvasive disease markers in the management of MPNs. |
| first_indexed | 2024-03-10T23:26:56Z |
| format | Article |
| id | doaj.art-a4f1f67afe154f11831e6acc1a11d00e |
| institution | Directory Open Access Journal |
| issn | 2072-6694 |
| language | English |
| last_indexed | 2024-03-10T23:26:56Z |
| publishDate | 2023-08-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cancers |
| spelling | doaj.art-a4f1f67afe154f11831e6acc1a11d00e2023-11-19T07:56:12ZengMDPI AGCancers2072-66942023-08-011517432310.3390/cancers15174323Revisiting Circulating Extracellular Matrix Fragments as Disease Markers in Myelofibrosis and Related NeoplasmsHans Carl Hasselbalch0Peter Junker1Vibe Skov2Lasse Kjær3Trine A. Knudsen4Morten Kranker Larsen5Morten Orebo Holmström6Mads Hald Andersen7Christina Jensen8Morten A. Karsdal9Nicholas Willumsen10Department of Hematology, Zealand University Hospital, 4000 Roskilde, DenmarkDepartment of Rheumatology, Odense University Hospital, 5000 Odense, DenmarkDepartment of Hematology, Zealand University Hospital, 4000 Roskilde, DenmarkDepartment of Hematology, Zealand University Hospital, 4000 Roskilde, DenmarkDepartment of Hematology, Zealand University Hospital, 4000 Roskilde, DenmarkDepartment of Hematology, Zealand University Hospital, 4000 Roskilde, DenmarkNational Center for Cancer Immune Therapy, Herlev Hospital, 2730 Herlev, DenmarkNational Center for Cancer Immune Therapy, Herlev Hospital, 2730 Herlev, DenmarkNordic Bioscience A/S, 2730 Herlev, DenmarkNordic Bioscience A/S, 2730 Herlev, DenmarkNordic Bioscience A/S, 2730 Herlev, DenmarkPhiladelphia 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 <i>JAK2V617F</i> 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 dysfunction and ultimately, organ failure, due to excessive accumulation of extracellular matrix (ECM). Considering that MPNs are acquired clonal stem cell diseases developing in an inflammatory microenvironment in which the hematopoietic cell populations are progressively replaced by stromal proliferation—“a wound that never heals”—we herein aim to provide a comprehensive review of previous promising research in the field of circulating ECM fragments in the diagnosis, treatment and monitoring of MPNs. We address the rationales and highlight new perspectives for the use of circulating ECM protein fragments as biologically plausible, noninvasive disease markers in the management of MPNs.https://www.mdpi.com/2072-6694/15/17/4323myeloproliferative neoplasmsMPNsMPNcirculating extracellular matrix (ECM) proteinsserum procollagen III N-terminal propeptide (PIIINP)procollagen type I C-terminal propeptide (PICP) |
| spellingShingle | Hans Carl Hasselbalch Peter Junker Vibe Skov Lasse Kjær Trine A. Knudsen Morten Kranker Larsen Morten Orebo Holmström Mads Hald Andersen Christina Jensen Morten A. Karsdal Nicholas Willumsen Revisiting Circulating Extracellular Matrix Fragments as Disease Markers in Myelofibrosis and Related Neoplasms Cancers myeloproliferative neoplasms MPNs MPN circulating extracellular matrix (ECM) proteins serum procollagen III N-terminal propeptide (PIIINP) procollagen type I C-terminal propeptide (PICP) |
| title | Revisiting Circulating Extracellular Matrix Fragments as Disease Markers in Myelofibrosis and Related Neoplasms |
| title_full | Revisiting Circulating Extracellular Matrix Fragments as Disease Markers in Myelofibrosis and Related Neoplasms |
| title_fullStr | Revisiting Circulating Extracellular Matrix Fragments as Disease Markers in Myelofibrosis and Related Neoplasms |
| title_full_unstemmed | Revisiting Circulating Extracellular Matrix Fragments as Disease Markers in Myelofibrosis and Related Neoplasms |
| title_short | Revisiting Circulating Extracellular Matrix Fragments as Disease Markers in Myelofibrosis and Related Neoplasms |
| title_sort | revisiting circulating extracellular matrix fragments as disease markers in myelofibrosis and related neoplasms |
| topic | myeloproliferative neoplasms MPNs MPN circulating extracellular matrix (ECM) proteins serum procollagen III N-terminal propeptide (PIIINP) procollagen type I C-terminal propeptide (PICP) |
| url | https://www.mdpi.com/2072-6694/15/17/4323 |
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