Cited by 136Myeloproliferative Neoplasms: Diagnosis and TreatmentPlatelet Disorders and TreatmentsHematological disorders and diagnostics

Inflammation and myeloproliferative neoplasms

Lussana, Federico, Rambaldi, Alessandro·2017·Journal of Autoimmunity

Abstract

Myeloproliferative neoplasms (MPN) include three main entities: Polycythemia Vera (PV), Essential Thrombocythemia (ET), and Myelofibrosis (MF). MPN represent a unique model of the relationship between the clonal development of a hematologic malignancy and chronic inflammation. The neoplastic clone is the main driver of this inflammatory reaction as demonstrated by the curative effect of allogeneic stem cell transplantation which leads not only to a complete restore of the hematopoiesis, but also to regression of bone marrow fibrosis. The neoplastic clone and its differentiated progeny are also the main source of an indirect paracrine secretion of inflammatory cytokines released by different normal cells present within the tumor microenvironment. In the end, the cytokine storm within the bone marrow niche leads to fibrosis. In addition, chronic inflammation is responsible of the constitutional symptoms which negatively affect the quality of life of MPN patients and represents a major driver for the development of premature atherosclerosis and disease progression. Here we describe the available data about the link between MPN and chronic inflammation in animal models as well as in clinical studies. We also review the practical value of including acute phase inflammatory proteins such as high sensitivity C-reactive protein (hs-CRP) and pentraxin 3 (PTX-3) in prognostic stratification of MPN patients. Interestingly, the plasma levels of these proteins is often increase in MPN pat…

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Crossref · 10.1016/j.jaut.2017.06.010

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Authors
Lussana, F., & Rambaldi, A.
Affiliations
  • Ospedale Papa Giovanni XXIII (IT)
  • University of Milan (IT)
Corresponding author
Rambaldi, Alessandro
Venue
Journal of Autoimmunity
Publisher
Elsevier BV
Published
2017-12
Volume
85
Pages
58-63
ISSN
0896-8411 (ISSN-L)
OpenAlex
W2732945346
Cited by
136
References
3
Citations by year
2026: 5 · 2025: 13 · 2024: 16 · 2023: 13 · 2022: 18 · 2021: 29 · 2020: 25 · 2019: 8 · 2018: 8 · 2017: 1
FWCI
1.62
Type
journal article
Language
en
Open access
closed
Locations
  • Journal of Autoimmunity (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Genetics › Myeloproliferative Neoplasms: Diagnosis and Treatment
  • Health Sciences › Medicine › Hematology › Platelet Disorders and Treatments
  • Health Sciences › Medicine › Emergency Medicine › Hematological disorders and diagnostics
Keywords
myeloproliferative neoplasms, chronic inflammation, polycythemia vera, essential thrombocythemia, myelofibrosis, bone marrow fibrosis, tumor microenvironment, acute phase proteins, C-reactive protein, pentraxin 3, thrombosis risk, atherosclerosis, ruxolitinib, allogeneic stem cell transplantation, prognostic stratification, constitutional symptoms
MeSH terms
Animals, Humans, Inflammation, Myeloproliferative Disorders, Neoplasms, Polycythemia Vera, Prognosis, Thrombocythemia, Essential, Primary Myelofibrosis

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