Real-world risk assessment and treatment initiation among patients with myelofibrosis at community oncology practices in the United States
This study was conducted to evaluate how physicians risk-stratify patients at the time of MF diagnosis, the accuracy of the risk stratification, and its effect on treatment selection. Medical charts were reviewed at US community hematology/oncology practices in the Cardinal Health Oncology Provider Extended Network; patient clinical characteristics, risk stratification, and treatment data were collected. Physician-assigned risk categorizations were compared with data-derived risk categorizations based on the International Prognostic Scoring System, the system recommended at diagnosis. A total of 491 patients diagnosed with...
Source: Annals of Hematology - May 6, 2020 Category: Hematology Source Type: research

Platelet Toll-Like Receptors Mediate Thromboinflammatory Responses in Patients With Essential Thrombocythemia
In conclusion, exacerbated response to TLR stimulation may promote platelet activation in ET, boosting platelet/leukocyte/endothelial interactions and secretion of inflammatory mediators, overall reinforcing the thromboinflammatory state. These findings highlight the role of platelets as inflammatory sentinels in MPN prothrombotic scenario and provide additional evidence for the close intertwining between thrombosis and inflammation in this setting. (Source: Frontiers in Immunology)
Source: Frontiers in Immunology - April 29, 2020 Category: Allergy & Immunology Source Type: research

Cancers, Vol. 12, Pages 903: Role of DNA Damage Response in Suppressing Malignant Progression of Chronic Myeloid Leukemia and Polycythemia Vera: Impact of Different Oncogenes
ladimir Divoky Inflammatory and oncogenic signaling, both known to challenge genome stability, are key drivers of BCR-ABL-positive chronic myeloid leukemia (CML) and JAK2 V617F-positive chronic myeloproliferative neoplasms (MPNs). Despite similarities in chronic inflammation and oncogene signaling, major differences in disease course exist. Although BCR-ABL has robust transformation potential, JAK2 V617F-positive polycythemia vera (PV) is characterized by a long and stable latent phase. These differences reflect increased genomic instability of BCR-ABL-positive CML, compared to genome-stable PV with rare cytogenetic ab...
Source: Cancers - April 6, 2020 Category: Cancer & Oncology Authors: Jan Stetka Jan Gursky Julie Li ñan Velasquez Renata Mojzikova Pavla Vyhlidalova Lucia Vrablova Jiri Bartek Vladimir Divoky Tags: Review Source Type: research

Use of rotational thromboelastometry for a global screening of coagulation profile in patients of myeloproliferative neoplasms.
Authors: Şahin DG, Akay OM, Uskudar Teke H, Andıc N, Gunduz E Abstract Chronic myeloproliferative neoplasms (MPN) are clonal disorders of hematopoietic stem/progenitor cell characterized by thrombohemorrhagic complications and a tendency to transform into acute leukemia. The pathogenesis of thrombosis in MPN is complex and results from a multifaceted interplay of clinical and disease-related factors. Rotational thromboelastometry (ROTEM) provides the complete and rapid information about all stages of the coagulation process. Here, we assess ROTEM parameters as a screening of coagulation profile in patien...
Source: Platelets - April 6, 2020 Category: Hematology Tags: Platelets Source Type: research

Association of mast cells and bone marrow reticulin fibrosis in patients with bcr-abl negative chronic myeloproliferative neoplasms
Publication date: Available online 5 March 2020Source: Blood Cells, Molecules, and DiseasesAuthor(s): Hakan Keski (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - March 6, 2020 Category: Hematology Source Type: research

Association of mast cells and bone marrow reticulin fibrosis in patients with bcr-abl negative chronic myeloproliferative neoplasms.
CONCLUSION: MCN and reticulin fibrosis were significantly increased in MPN patients. JAK2 positivity had significantly increased MCN compared to patients without JAK2. JAK2 was associated with increased reticulin fibrosis. PMID: 32179412 [PubMed - as supplied by publisher] (Source: Blood Cells, Molecules and Diseases)
Source: Blood Cells, Molecules and Diseases - March 4, 2020 Category: Hematology Authors: Keski H Tags: Blood Cells Mol Dis Source Type: research

Retrospective analysis of the clinical features of 172 patients with BCR-ABL1-negative chronic myeloproliferative neoplasms
To explore the clinical features of the patients with BCR-ABL1-negative chronic myeloproliferative neoplasms (MPNs) in our hospital and to reveal the unique features of BCR-ABL1-negative MPNs patients in our cent... (Source: Molecular Cytogenetics)
Source: Molecular Cytogenetics - February 17, 2020 Category: Molecular Biology Authors: Xiaolan Lin, Huifang Huang and Ping Chen Tags: Research Source Type: research

Distinguishing atypical chronic myeloid leukemia from other Philadelphia-negative chronic myeloproliferative neoplasms
Purpose of review Atypical chronic myeloid leukemia (aCML), BCR-ABL1-negative, is a rare myelodysplastic/myeloproliferative neoplasm (MDS/MPN) characterized by leukocytosis, granulocytic dysplasia, and typically poor patient outcomes. Since its first description as a variant CML lacking the Philadelphia chromosome (Ph), the diagnostic criteria for aCML have evolved significantly. Nevertheless, distinguishing it from other Ph-negative myeloid neoplasms can still be very challenging, and given its generally worse prognosis, this is a clinically important distinction. The purpose of this review is to conceptualize our under...
Source: Current Opinion in Hematology - January 30, 2020 Category: Hematology Tags: MYELOID DISEASE: Edited by Martin S. Tallman Source Type: research

Clinico-biological characteristics of patients with myelofibrosis: an analysis of 1,000 cases from the Spanish Registry of Myelofibrosis.
CONCLUSIONS: Myelofibrosis is a disabling condition mainly affecting elderly people. Its treatment is mostly driven by symptom control. Despite its clinical heterogeneity, several prognostic models are useful to select candidates for transplantation. PMID: 31980217 [PubMed - as supplied by publisher] (Source: Medicina Clinica)
Source: Medicina Clinica - January 27, 2020 Category: General Medicine Tags: Med Clin (Barc) Source Type: research

Evidence of immune elimination, immuno-editing and immune escape in patients with hematological cancer
AbstractThere is mounting evidence that the immune system can spontaneously clear malignant lesions before they manifest as overt cancer, albeit this activity has been difficult to demonstrate in humans. The calreticulin (CALR) exon 9 mutations are driver mutations in patients with chronic myeloproliferative neoplasms (MPN), which are chronic blood cancers. TheCALR mutations generate a neo-antigen that is recognized by patient T cells, and T cells isolated from a patient with aCALR-mutation can recognize and kill autologousCALR-mutant cells. Surprisingly, healthy individuals display frequent and strong T cell responses to ...
Source: Cancer Immunology, Immunotherapy - January 7, 2020 Category: Cancer & Oncology Source Type: research

Risk factors and incidence of thrombosis in a Brazilian cohort of patients with Philadelphia-negative myeloproliferative neoplasms
AbstractFew data are available regarding epidemiology and outcomes of Philadelphia-negative chronic myeloproliferative neoplasms (MPN) in Latin America. Therefore, current models for MPN treatment are based in large cohorts of patients from Europe and North America. In this paper, we conducted a retrospective study to evaluate thrombotic and bleeding events in a cohort of patients with MPN from a reference center in Brazil. A total of 334 patients were included, being essential thrombocythemia the most common diagnosis. Here, we found that 41% of the MPN patients had a thrombotic event prior to the diagnosis. Thrombosis wa...
Source: Journal of Thrombosis and Thrombolysis - January 1, 2020 Category: Hematology Source Type: research

Bioinformatics Analysis of Key Genes and Pathways Associated with Thrombosis in Essential Thrombocythemia.
CONCLUSIONS Bioinformatics analysis identified DEGs and hub genes that interacted with CD34+ cells and neutrophils that may predict an increased risk of thrombosis in patients with ET. These preliminary findings should be validated using next-generation sequencing (NGS) and clinical studies. PMID: 31801935 [PubMed - in process] (Source: Medical Science Monitor)
Source: Medical Science Monitor - December 7, 2019 Category: Research Tags: Med Sci Monit Source Type: research

Concurrent chronic myeloid leukemia and CALR-mutated chronic myeloproliferative neoplasm
Publication date: Available online 30 November 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Francesca Guidotti, Angelo Gardellini, Maddalena Feltri, Michelle Zancanella, Vittoria Saccà, Franco Alberio, Luciana Ambrosiani, Mauro Turrini (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - November 30, 2019 Category: Hematology Source Type: research

Concurrent chronic myeloid leukemia and CALR-mutated chronic myeloproliferative neoplasm.
PMID: 31816513 [PubMed - as supplied by publisher] (Source: Blood Cells, Molecules and Diseases)
Source: Blood Cells, Molecules and Diseases - November 29, 2019 Category: Hematology Authors: Guidotti F, Gardellini A, Feltri M, Zancanella M, Saccà V, Alberio F, Ambrosiani L, Turrini M Tags: Blood Cells Mol Dis Source Type: research

Novel lysyl oxidase inhibitors attenuate hallmarks of primary myelofibrosis in mice.
Abstract Primary myelofibrosis (PMF) is a chronic myeloproliferative neoplasm (MPN) that usually portends a poor prognosis with limited therapeutic options available. Currently, only allogeneic stem cell transplantation is curative in those who are candidates, while administration of the JAK1/2 inhibitor ruxolitinib carries a risk of worsening cytopenia. The limited therapeutic options available highlight the need for the development of novel treatments for PMF. Lysyl oxidase (LOX), an enzyme vital for collagen cross-linking and extracellular matrix stiffening, has been found to be upregulated in PMF. Here...
Source: International Journal of Hematology - October 20, 2019 Category: Hematology Authors: Leiva O, Ng SK, Matsuura S, Chitalia V, Lucero H, Findlay A, Turner C, Jarolimek W, Ravid K Tags: Int J Hematol Source Type: research