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Review
. 2020 Jul 22;9(8):2334.
doi: 10.3390/jcm9082334.

The Role of Inflammation and Inflammasome in Myeloproliferative Disease

Lucia Longhitano  1 , Giovanni Li Volti  1 , Cesarina Giallongo  2 , Mariarita Spampinato  1 , Ignazio Barbagallo  3 , Michelino Di Rosa  4 , Alessandra Romano  5 , Roberto Avola  1 , Daniele Tibullo  1 , Giuseppe Alberto Palumbo  2
Affiliations
Review

The Role of Inflammation and Inflammasome in Myeloproliferative Disease

Lucia Longhitano et al. J Clin Med. .

Abstract

Polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) are rare hematological conditions known as myeloproliferative neoplasms (MPNs). They are characterized for being BCR-ABL negative malignancies and affected patients often present with symptoms which can significantly impact their quality of life. MPNs are characterized by a clonal proliferation of an abnormal hematopoietic stem/progenitor cell. In MPNs; cells of all myeloid lineages; including those involved in the immune and inflammatory response; may belong to the malignant clone thus leading to an altered immune response and an overexpression of cytokines and inflammatory receptors; further worsening chronic inflammation. Many of these cytokines; in particular, IL-1β and IL-18; are released in active form by activating the inflammasome complexes which in turn mediate the inflammatory process. Despite this; little is known about the functional effects of stem cell-driven inflammasome signaling in MPN pathogenesis. In this review we focused on the role of inflammatory pathway and inflammasome in MPN diseases. A better understanding of the inflammatory-state-driving MPNs and of the role of the inflammasome may provide new insights on possible therapeutic strategies.

Keywords: essential thrombocythemia (ET); inflammasome; inflammation; myelofibrosis (MF); myeloproliferative neoplasms (MPNs); polycythemia vera (PV).

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
(A) Schematic representation of Pattern Recognition receptors (PRR) and nucleotide-binding oligomerization domain (NOD)-like receptors (NLRs); (B) schematic representation of inflammasome complex.
Figure 2
Figure 2
Graphic representation of the dual role of the inflammasome complex in cancer.
Figure 3
Figure 3
Cycles represent cytokines and chemokines associated with myeloproliferative neoplasms (MPNs) main entities.
See this image and copyright information in PMC

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