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Thrombocythemia: Current Status, Challenges and Future Directions
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Thrombocythemia: Current Status, Challenges and Future Directions

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Hematology".

Deadline for manuscript submissions: closed (15 December 2022) | Viewed by 3014

Special Issue Editor

Dr. Emma C. Cacciola

E-Mail Website
Guest Editor
Hemostasis Unit, Department of Medical, Surgical Sciences and Advanced Technologies “G.F. Ingrassia”, University of Catania, Catania, Italy
Interests: myeloproliferative neoplasms; haemostasis; diagnosis; therapy
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Essential Thrombocythemia (ET) is characterized by abnormal megakaryocytes and thrombocytosis. The ET is  generally associated with mutations such as JAK2 exon 14, CALR exon 9, or MPL exon 10 (W515R/G/S). However, about 10% of patients with ET are triple negative (TN). TN patients can have “noncanonical”  MPL mutations (T119I, S204F/P, E230G, Y252H  and Y591D/N, R537W (exon 11), P453R and S505N (exon 9)). In addition, ET patient genes of platelet proliferation (ITGA2B and ITGB3) and DNA methylation profiles can characterize the ET and represent potential novel mechanisms of disease initiation. In parallel with these genes of upregulated megakaryopoiesis, the signaling pathways involved in hematopoiesis, inflammation, and immune regulation (NFkB, MAPK, TNF) have been identified. Thrombosis and myelofibrosis progression are a leading cause of morbidity and mortality in ET. Patients with the JAK2V617F mutation have a higher risk of thrombosis and may progress to PV or myelofibrosis, whereas those with CALR mutation have a low risk of thrombosis and higher risk of progression to myelofibrosis. ET is managed with aspirin and selected use of hydroxycarbamide (HC), interferon α (IFN α), or anagrelide. However, therapy remains suboptimal with the ongoing risk for thrombosis and risk for transformation. An emerging therapy is represented by the JAK inhibitor (ruxolitinib).

Prof. Dr. Emma C. Cacciola
Guest Editor

Manuscript Submission Information

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Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • thrombocythemia
  • genes
  • thrombosis
  • myelofibrosis

Published Papers (2 papers)

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Research

15 pages, 8035 KiB  
Article
Proteomic-Based Platelet Activation-Associated Protein SELP May Be a Novel Biomarker for Coagulation and Prognostic in Essential Thrombocythemia
by Dehao Wang, Pei Zhao, Yan Lv, Jing Ming, Ziqing Wang, Erpeng Yang, Yumeng Li, Mingjing Wang, Jicong Niu, Yanyu Zhang, Yan Sun, Yi Chen, Ke Chen, Zhuo Chen, Weiyi Liu and Xiaomei Hu
J. Clin. Med. 2023, 12(3), 1078; https://doi.org/10.3390/jcm12031078 - 30 Jan 2023
Cited by 2 | Viewed by 1261
Abstract
Abnormal platelet activation can lead to thrombosis in essential thrombocythemia (ET) and thus impact patient prognosis. Platelet activation-associated proteins are key molecules for platelet activation. However, it is unclear which proteins are most closely associated with the disease’s prognosis. To determine which platelet [...] Read more.
Abnormal platelet activation can lead to thrombosis in essential thrombocythemia (ET) and thus impact patient prognosis. Platelet activation-associated proteins are key molecules for platelet activation. However, it is unclear which proteins are most closely associated with the disease’s prognosis. To determine which platelet activation-related proteins can be employed as ET patient prognosis predictors, we used label-free quantification (LFQ) and parallel reaction monitoring (PRM) technology and first determined the serum proteomic expression levels and the differential proteins of ET patients. Then, based on the IPSET (International Prognostic Score for ET), the differential protein associated with the prognostic score was found. To investigate potential processes affecting prognosis, the connection of this protein with prognostic markers, such as thrombotic history, age, white blood cell count, coagulation factors, and inflammatory factors, were further examined. The levels of platelet activation-related proteins GPIbα, SELP, PF4, MMP1, and FLNA were significantly higher in ET patients, according to LFQ and PRM analyses (p < 0.01). Based on regression analysis of the IPSET prognostic score, it is suggested that the SELP level was positively correlated with the prognostic score and prognostic risk factor analysis (p < 0.05). Further regression analysis of SELP with coagulation factors showed that antithrombin (AT-III) was negatively correlated with SELP levels (p < 0.05). Further regression analysis of the inflammatory factors with AT-III and SELP revealed that IL-10, IL-12P70, and IL-31 were negatively correlated with AT-III and SELP (p < 0.01). Platelet activation pathway-related proteins are expressed more frequently in ET patients, and serum SELP may be a prognostic marker for these individuals by encouraging leukocyte increase and inflammatory factor expression and causing aberrant coagulation. Full article
(This article belongs to the Special Issue Thrombocythemia: Current Status, Challenges and Future Directions)
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13 pages, 1661 KiB  
Article
TET2 Mutation May Be More Valuable in Predicting Thrombosis in ET Patients Compared to PV Patients: A Preliminary Report
by Ziqing Wang, Weiyi Liu, Dehao Wang, Erpeng Yang, Yujin Li, Yumeng Li, Yan Sun, Mingjing Wang, Yan Lv and Xiaomei Hu
J. Clin. Med. 2022, 11(22), 6615; https://doi.org/10.3390/jcm11226615 - 08 Nov 2022
Cited by 6 | Viewed by 1258
Abstract
Thrombosis is a common complication of myeloproliferative neoplasm (MPN), and it is a major cause of disability and death. With the development of next-generation gene-sequencing technology, the relationship between non-driver mutations and thrombotic risk factors has also attracted considerable attention. To analyze the [...] Read more.
Thrombosis is a common complication of myeloproliferative neoplasm (MPN), and it is a major cause of disability and death. With the development of next-generation gene-sequencing technology, the relationship between non-driver mutations and thrombotic risk factors has also attracted considerable attention. To analyze the risk factors of thrombosis in patients with essential thrombocythemia (ET) and polycythemia vera (PV), we retrospectively analyzed the clinical data of 125 MPN patients (75 ET and 50 PV) and performed a multivariate analysis of the risk factors of thrombosis using a Cox proportional risk model. Among the 125 patients, 35 (28.0%) had thrombotic events, and the incidence of thrombotic events was 21.3% and 38.0% in ET and PV patients, respectively. In ET patients, the multivariate analysis showed that a TET2 mutation and history of remote thrombosis were independent risk factors for thrombosis in ET patients, with an HR of 4.1 (95% CI: 1.40–12.01; p = 0.01) for TET2 mutation and 6.89 (95% CI: 1.45–32.68; p = 0.015) for a history of remote thrombosis. In PV patients, the multivariate analysis presented the neutrophil-to-lymphocyte ratio (NLR) (HR: 4.77, 95% CI: 1.33–17.16; p = 0.017) and a history of remote thrombosis (HR: 1.67, 95% CI: 1.03–1.32; p = 0.014) as independent risk factors for thrombosis, with no significant change in the risk of thrombosis in patients with TET2 mutations. A further analysis of the clinical characteristics and coagulation occurring in ET patients with a TET2 mutation revealed that the values of age and D-dimer were significantly higher and antithrombin III was significantly lower in TET2-mutated ET patients compared to TET2-unmutated patients. In summary, TET2 mutation may be more valuable in predicting thrombosis in ET patients than in PV patients. ET patients with a TET2 mutation are older and present differences in coagulation compared to TET2-unmutated patients. Full article
(This article belongs to the Special Issue Thrombocythemia: Current Status, Challenges and Future Directions)
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