7,537 results match your criteria Agnogenic Myeloid Metaplasia With Myelofibrosis


Bone Marrow Soluble Mediator Signatures of Patients With Philadelphia Chromosome-Negative Myeloproliferative Neoplasms.

Front Oncol 2021 18;11:665037. Epub 2021 May 18.

Department of Clinical Analyses, Toxicology and Food Science, School of Pharmaceutical Sciences of Ribeirão Preto, University of São Paulo, Ribeirão Preto, Brazil.

Background: Essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis (PMF) are clonal hematological diseases classified as Philadelphia chromosome-negative myeloproliferative neoplasms (MPN). MPN pathogenesis is associated with the presence of somatic driver mutations, bone marrow (BM) niche alterations, and tumor inflammatory status. The relevance of soluble mediators in the pathogenesis of MPN led us to analyze the levels of cytokines, chemokines, and growth factors related to inflammation, angiogenesis and hematopoiesis regulation in the BM niche of MPN patients. Read More

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Ruxolitinib discontinuation in polycythemia vera: Patient characteristics, outcomes, and salvage strategies from a large multi-institutional database.

Leuk Res 2021 May 27;109:106629. Epub 2021 May 27.

Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, United States. Electronic address:

Ruxolitinib is approved for the treatment of patients with polycythemia vera (PV) who are intolerant or resistant to hydroxyurea. While ruxolitinib discontinuation in myelofibrosis is associated with dismal outcomes, the analogous experience in PV has not been reported. Using a large, multi-institutional database of PV patients, we identified 93 patients with PV who were treated with ruxolitinib, of whom 22 discontinued therapy. Read More

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Impact of the rs1024611 Polymorphism of on the Pathophysiology and Outcome of Primary Myelofibrosis.

Cancers (Basel) 2021 May 22;13(11). Epub 2021 May 22.

Department of Medicine and Surgery, Anatomy Unit, University of Parma, 43126 Parma, Italy.

Single nucleotide polymorphisms (SNPs) can modify the individual pro-inflammatory background and may therefore have relevant implications in the MPN setting, typified by aberrant cytokine production. In a cohort of 773 primary myelofibrosis (PMF), we determined the contribution of the rs1024611 SNP of CCL2-one of the most potent immunomodulatory chemokines-to the clinical and biological characteristics of the disease, demonstrating that male subjects carrying the homozygous genotype G/G had an increased risk of PMF and that, among PMF patients, the G/G genotype is an independent prognostic factor for reduced overall survival. Functional characterization of the SNP and the CCL2-CCR2 axis in PMF showed that i) homozygous PMF cells are the highest chemokine producers as compared to the other genotypes; ii) PMF CD34+ cells are a selective target of CCL2, since they uniquely express CCR2 (CCL2 receptor); iii) activation of the CCL2-CCR2 axis boosts pro-survival signals induced by driver mutations via Akt phosphorylation; iv) ruxolitinib effectively counteracts CCL2 production and down-regulates CCR2 expression in PMF cells. Read More

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MAPK14 over-expression is a transcriptomic feature of polycythemia vera and correlates with adverse clinical outcomes.

J Transl Med 2021 May 31;19(1):233. Epub 2021 May 31.

Department of Hematology, China-Japan Friendship Hospital, Yinghua East Street, Beijing, China.

Background: The transcriptomic signature has not been fully elucidated in PV, as well as mRNA markers for clinical variables (thrombosis, leukemic transformation, survival, etc.). We attempted to reveal and validate crucial co-expression modules and marker mRNAs correlating with polycythemia vera (PV) by weighted gene co-expression network analysis (WGCNA). Read More

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Outcomes of allogeneic hematopoietic cell transplantation in patients with myelofibrosis - a systematic review and meta-analysis: Allo-HCT myelofibrosis.

Transplant Cell Ther 2021 May 27. Epub 2021 May 27.

Leukemia Service, Memorial Sloan Kettering Cancer Center, New York, NY. Electronic address:

Background: Allogeneic hematopoietic cell transplant (allo-HCT) remains the only potentially curative therapeutic modality for patients with primary or secondary myelofibrosis (MF). However, many patients are considered ineligible for allo-HCT and transplant-related mortality can be substantial. Data on the efficacy and safety of allo-HCT are mixed and largely derived from retrospective studies. Read More

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Ga-Pentixafor-PET/CT imaging represents a novel approach to detect chemokine receptor CXCR4 expression in myeloproliferative neoplasms.

J Nucl Med 2021 May 28. Epub 2021 May 28.

Nuclear Medicine, Medical Faculty, University of Augsburg, Germany.

C-X-C motif chemokine receptor 4 (CXCR4) is an attractive target for cancer diagnosis and treatment, as it is overexpressed in many solid and hematological malignancies. This study investigated the feasibility of CXCR4-directed imaging with positron emission tomography/computed tomography (PET/CT) using Ga-Pentixafor to visualize and quantify disease involvement in myeloproliferative neoplasms (MPNs). 12 patients with MPNs ( = 4 primary myelofibrosis, = 6 essential thrombocythemia, = 2 polycythemia vera) and 5 controls underwent Ga-Pentixafor-PET/CT. Read More

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Effects of strong and moderate CYP3A4 inducers on the pharmacokinetics of fedratinib in healthy adult participants.

Cancer Chemother Pharmacol 2021 May 21. Epub 2021 May 21.

Bristol Myers Squibb, Princeton, NJ, USA.

Purpose: Fedratinib is an oral and selective Janus kinase 2 inhibitor that is indicated for treatment of adults with intermediate-2 or high-risk primary or secondary myelofibrosis. Fedratinib is metabolized by cytochrome P450s (CYPs), primarily CYP3A4. The objective of this study was to determine the effects of the strong CYP3A4 inducer rifampin and moderate CYP3A4 inducer efavirenz on the pharmacokinetics of single doses of fedratinib. Read More

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Immunoglobulin G4-Related Disease Accompanied by Primary Myelofibrosis: Case Report.

Front Med (Lausanne) 2021 4;8:638794. Epub 2021 May 4.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Science, Beijing, China.

Immunoglobulin G4-related disease (IgG4-RD) is a heterogeneous autoimmune fibrosing disorder that presents common pathologic features but with unclear etiology. We report a rare case of IgG4-RD accompanied by primary myelofibrosis that eventually transformed into acute myeloid leukemia. A 50-year-old woman suffered from progressive lacrimal and parotid gland enlargement, diaphoresis, and rapid weight loss. Read More

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Efficacy and safety of a novel dosing strategy for ruxolitinib in the treatment of patients with myelofibrosis and anemia: the REALISE phase 2 study.

Leukemia 2021 May 20. Epub 2021 May 20.

University Hospital Halle, Halle (Saale), Germany.

Anemia is a frequent manifestation of myelofibrosis (MF) and there is an unmet need for effective treatments in anemic MF patients. The REALISE phase 2 study (NCT02966353) evaluated the efficacy and safety of a novel ruxolitinib dosing strategy with a reduced starting dose with delayed up-titration in anemic MF patients. Fifty-one patients with primary MF (66. Read More

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Immune Complex Associated Glomerulonephritis in a Patient with Prefibrotic Primary Myelofibrosis: A Case Report.

Indian J Nephrol 2021 Jan-Feb;31(1):50-53. Epub 2021 Jan 27.

Department of Radiodiagnosis, All India Institute of Medical Sciences Bhopal, Madhya Pradesh, India.

A case of prefibrotic myelofibrosis with immune complex-mediated glomerulonephritis is presented. A 45-year-old female, with history of right subclavian and axillary vein thrombosis, presented with abdominal distension, facial puffiness, and pedal edema. Evaluation revealed deranged renal functions with nephrotic range proteinuria and acute kidney injury. Read More

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January 2021

Quantification of JAK2V617F mutation load by droplet digital PCR can aid in diagnosis of myeloproliferative neoplasms.

Int J Lab Hematol 2021 May 11. Epub 2021 May 11.

Institute of Hematology, The Second Hospital of Shanxi Medical University, Taiyuan, China.

Introduction: This study developed a method for quantifying the JAK2V617F mutation load in patients with myeloproliferative neoplasm (MPN) using droplet digital PCR (ddPCR), which provides a new laboratory method for diagnosing polycythemia vera (PV), essential thrombocythemia (ET), and pre-primary myelofibrosis (pre-PMF).

Methods: Patients with MPN who had JAK2V617F mutations from March 2013 to August 2019 were enrolled in this study. JAK2V617F mutation loads were quantified using ddPCR technology. Read More

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Coexisting -Mutated Langerhans Cell Histiocytosis and Primary Myelofibrosis with Shared Mutation.

Case Rep Hematol 2021 16;2021:6623706. Epub 2021 Apr 16.

Department of Hematology, Aarhus University Hospital, Palle Juul-Jensens Boulevard 35, Aarhus N 8200, Denmark.

Langerhans cell histiocytosis (LCH) is an infrequent disease, characterized by oligoclonal proliferation of immature myeloid-derived cells. However, the exact pathogenesis remains unknown. In rare cases, LCH is present in patients with concomitant myeloid proliferative neoplasms. Read More

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Response to pegylated interferon in a COVID-19-positive elderly woman with primary myelofibrosis treated with ruxolitinib.

Clin Case Rep 2021 Apr 7;9(4):2228-2235. Epub 2021 Mar 7.

Department of Hematology Zealand University Hospital Roskilde Denmark.

An 83-year-old female had asymptomatic SARS-CoV-2 infection while taking ruxolitinib. She remained RT-PCR positive for viral RNA for >120 days, and Pegylated interferon for 4 weeks led to viral RNA clearance. The observations support combination therapy of ruxolitinib + interferon for COVID-19. Read More

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JAK2, CALR, and MPL Mutation Profiles in BCR-ABL Negative Myeloproliferative Neoplasms, a Referral Center Experience in the Middle East.

Iran J Pathol 2021 24;16(2):190-194. Epub 2021 Jan 24.

Molecular Pathology and Cytogenetic Section, Department of Pathology, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.

Background & Objective: JAK2, CALR, and MPL genes play pivotal roles in the pathogenesis of BCR-ABL negative myeloproliferative neoplasms. This study was conducted to evaluate the frequency of JAK2, CALR, and MPL mutations in BCR-ABL negative myeloproliferative neoplasms and their association with demographic data and hematologic parameters in a referral center, in the Middle East.

Methods: Seventy-one patients with BCR-ABL negative myeloproliferative neoplasms were evaluated for JAK2 V617F, CALR type 1, CALR type 2, and MPL by allele-specific PCR and conventional PCR from 2018 to 2019. Read More

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January 2021

Novel findings of splenic extramedullary hematopoiesis during primary myelofibrosis, post-essential thrombocythemia, and post-polycythemia vera myelofibrosis.

Virchows Arch 2021 May 1. Epub 2021 May 1.

Pathology Department, University Hospital Center of Bordeaux, Haut-Lévêque Hospital, 33600, Pessac, France.

BCR-ABL-fusion-negative myeloproliferative neoplasms (MPNs) with myelofibrosis (MF) include primary MF, post-polycythemia vera MF and post-essential thrombocythemia MF. Clonal extramedullary hematopoiesis (EMH) can occur during MPN pathogenesis. Although histopathological bone-marrow (BM) features during clonal EMH have been investigated, those of the spleen have been poorly described. Read More

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Clinical and Pathologic Spectrum of DDX41-Mutated Hematolymphoid Neoplasms.

Am J Clin Pathol 2021 Apr 30. Epub 2021 Apr 30.

Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA.

Objectives: This study seeks to further characterize the clinicopathologic spectrum of DDX41-mutated hematolymphoid malignancies.

Methods: We identified DDX41 mutations from a cohort of known or suspected hematologic disorders and reviewed the corresponding clinical, genetic, phenotypic, and morphologic findings.

Results: DDX41 mutations were identified in 20 (1. Read More

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Low V617F Allele Burden in Ph-Negative Chronic Myeloproliferative Neoplasms Is Associated with Additional or Gene Mutations.

Genes (Basel) 2021 Apr 12;12(4). Epub 2021 Apr 12.

National Research Center for Hematology, Novy Zykovski lane 4a, 125167 Moscow, Russia.

(Janus kinase 2) V617F, (Calreticulin) exon 9, and (receptor for thrombopoietin) exon 10 mutations are associated with the vast majority of Ph-negative chronic myeloproliferative neoplasms (MPNs). These mutations affect sequential stages of proliferative signal transduction and therefore, after the emergence of one type of mutation, other types should not have any selective advantages for clonal expansion. However, simultaneous findings of these mutations have been reported by different investigators in up to 10% of MPN cases. Read More

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The Epidemiology of Myeloproliferative Neoplasms in New Zealand between 2010 and 2017: Insights from the New Zealand Cancer Registry.

Curr Oncol 2021 Apr 18;28(2):1544-1557. Epub 2021 Apr 18.

Blood and Cancer Biology Laboratory, Department of Molecular Medicine & Pathology, School of Medical Sciences, The University of Auckland, Auckland 1023, New Zealand.

Background: There is a paucity of data on ethnic disparities in patients with the classical Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs): polycythaemia vera (PV), essential thrombocythaemia (ET) and primary myelofibrosis (PMF).

Methods: This study analysed the demographic data for PV, ET and PMF collected by the New Zealand Cancer Registry (NZCR) between 2010 and 2017.

Results: We found that the NZCR capture rates were lower than average international incidence rates for PV and ET, but higher for PMF (0. Read More

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Are polycythemia vera, essential thrombocytosis, and primary myelofibrosis 1, 2, or 3 diseases?

Authors:
Jerry L Spivak

Leukemia 2021 Apr 28. Epub 2021 Apr 28.

Division of Hematology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

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Refractory primary autoimmune myelofibrosis treated with ruxolitinib.

Am J Hematol 2021 Apr 28. Epub 2021 Apr 28.

Department of Hematology and Hematopoietic Cell Transplantation, Gehr Center for Leukemia Research, City of Hope National Medical Center, California, Duarte, USA.

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Allogeneic haematopoietic cell transplantation for myelofibrosis: a real-life perspective.

Br J Haematol 2021 Apr 21. Epub 2021 Apr 21.

Service d'Hématologie Clinique et Thérapie Cellulaire, Hôpital Saint-Antoine, Sorbonne Université, INSERM UMRs 938, Paris, France.

Myelofibrosis (MF) is a clonal stem cell neoplasm with heterogeneous clinical phenotypes and well-established molecular drivers. Allogeneic haematopoietic stem cell transplantation (HSCT) offers an important curative treatment option for primary MF and post-essential thrombocythaemia/polycythaemia vera MF or secondary MF. With a disease course that varies from indolent to highly progressive, we are now able to stratify risk of mortality through various tools including patient-related clinical characteristics as well as molecular genetic profile. Read More

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Analysis of Common Driver Mutations in Philadelphia-Negative Myeloproliferative Neoplasms.

Clin Lymphoma Myeloma Leuk 2021 Mar 20. Epub 2021 Mar 20.

Seventh Year Medical Student, Faculty of Medicine, Kuwait University, Jabriya, Kuwait.

Background: Philadelphia-negative myeloproliferative neoplasms (MPNs) are a group of hematopoietic stem cell disorders that include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). This study examines the driver mutations among patients with MPNs in Kuwait.

Patients And Methods: This study was a retrospective review of 942 MPN cases with a driver mutation from July 2007 to June 2019 to examine their demographic, clinical, and laboratory attributes. Read More

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Myelofibrosis and Pancytopenia Associated With Primary Hyperparathyroidism.

AACE Clin Case Rep 2021 Jan-Feb;7(1):69-71. Epub 2020 Dec 28.

Department of Endocrinology, Diabetes and Metabolism, Christian Medical College and Hospital, Vellore, India.

Objective: Primary hyperparathyroidism (PHPT) has varied clinical presentations. Hematologic abnormalities secondary to PHPT have been described before. However, pancytopenia as the initial presentation has rarely been reported. Read More

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December 2020

Multi-arm Trial of Inflammatory Signal Inhibitors (MATIS) for hospitalised patients with mild or moderate COVID-19 pneumonia: a structured summary of a study protocol for a randomised controlled trial.

Trials 2021 Apr 12;22(1):270. Epub 2021 Apr 12.

Imperial College, London and Imperial NHS Trust, London, UK.

Objectives: The primary objective of MATIS is to determine the efficacy of ruxolitinib (RUX) or fostamatinib (FOS) compared to standard of care (SOC) with respect to reducing the proportion of hospitalised patients progressing from mild or moderate to severe COVID-19 pneumonia. Secondary objectives, at 14 and 28 days, are to: Determine the efficacy of RUX or FOS to reduce mortality Determine the efficacy of RUX or FOS to reduce the need for invasive ventilation or ECMO Determine the efficacy of RUX or FOS to reduce the need for non-invasive ventilation Determine the efficacy of RUX or FOS to reduce the proportion of participants suffering significant oxygen desaturation Determine the efficacy of RUX or FOS to reduce the need for renal replacement therapy Determine the efficacy of RUX and FOS to reduce the incidence of venous thromboembolism Determine the efficacy of RUX and FOS to reduce the severity of COVID-19 pneumonia [graded by a 9-point modified WHO Ordinal Scale* Determine the efficacy of RUX or FOS to reduce systemic inflammation Determine the efficacy of RUX or FOS to the incidence of renal impairment Determine the efficacy of RUX or FOS to reduce duration of hospital stay Evaluate the safety of RUX and FOS for treatment of COVID-19 pneumonia.

Trial Design: A multi-arm, multi-stage (3-arm parallel-group, 2-stage) randomised controlled trial that allocates participants 1:1:1 and tests for superiority in experimental arms versus standard of care. Read More

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Venetoclax with azacitidine or decitabine in blast-phase myeloproliferative neoplasm: A multicenter series of 32 consecutive cases.

Am J Hematol 2021 07 6;96(7):781-789. Epub 2021 May 6.

Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.

Venetoclax (Ven) combined with a hypomethylating agent (HMA) has now emerged as an effective treatment regimen for acute myeloid leukemia, in both de novo and relapsed/refractory setting. The current multicenter study retrospectively examined Ven + HMA treatment outcome among 32 patients (median age 69 years; 59% males) with blast-phase myeloproliferative neoplasm (MPN-BP). Pre-leukemic phenotype included essential thrombocythemia (ET)/post-ET myelofibrosis (34%), polycythemia vera (PV)/post-PV myelofibrosis (38%) and primary myelofibrosis (28%). Read More

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Association between Novel Marker (Platelet-Lymphocyte Ratio, Neutrophil-Lymphocyte Ratio, and Delta Neutrophil Index) and Outcomes in Sudden Cardiac Arrest Patients.

Emerg Med Int 2021 24;2021:6650958. Epub 2021 Mar 24.

Department of Emergency Medicine, Yonsei University Wonju College of Medicine, Wonju, Republic of Korea.

Purpose: It is important that clinicians accurately predict the outcome of patients with sudden cardiac arrest (SCA). The complete blood count (CBC) is an easy and inexpensive test that provides information on blood content. Platelet-lymphocyte ratio (PLR), neutrophil-lymphocyte ratio (NLR), and delta neutrophil index (DNI) are relatively novel biomarkers that have been used in the prognosis of various diseases. Read More

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Outcomes following second allogeneic haematopoietic cell transplantation in patients with myelofibrosis: a retrospective study of the Chronic Malignancies Working Party of EBMT.

Bone Marrow Transplant 2021 Apr 6. Epub 2021 Apr 6.

CHU de Lille, univ Lille, INSERM U1286, INFINITE, 59000, Lille, France.

Therapeutic management of patients with primary or secondary myelofibrosis (MF) who experience relapse or graft failure following allogeneic haematopoietic cell transplantation (allo-HCT) remains heterogeneous. We retrospectively analyzed 216 patients undergoing a second allo-HCT for either relapse (56%) or graft failure (31%) between 2010 and 2017. Median age was 57. Read More

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The Contemporary Approach to CALR-Positive Myeloproliferative Neoplasms.

Int J Mol Sci 2021 Mar 25;22(7). Epub 2021 Mar 25.

Department of Hematology, University Medical Centre Ljubljana, Zaloška 7, 1000 Ljubljana, Slovenia.

mutations are a revolutionary discovery and represent an important hallmark of myeloproliferative neoplasms (MPN), especially essential thrombocythemia and primary myelofibrosis. To date, several mutations were identified, with only frameshift mutations linked to the diseased phenotype. It is of diagnostic and prognostic importance to properly define the type of mutation and subclassify it according to its structural similarities to the classical mutations, a 52-bp deletion (type 1 mutation) and a 5-bp insertion (type 2 mutation), using a statistical approximation algorithm (AGADIR). Read More

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Impact of Integrated Genetic Information on Diagnosis and Prognostication for Myeloproliferative Neoplasms in the Next-Generation Sequencing Era.

J Clin Med 2021 Mar 3;10(5). Epub 2021 Mar 3.

Department of Laboratory Medicine, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea.

Since next-generation sequencing has been widely used in clinical laboratories, the diagnosis and risk stratification of hematologic malignancies are greatly dependent on genetic aberrations. In this study, we analyzed the genomic landscapes of 200 patients with myeloproliferative neoplasms (MPNs) and evaluated the impact of the genomic landscape on diagnosis and risk stratification. Mutations in , and were detected in 76. Read More

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Triple-Negative Essential Thrombocythemia: Clinical-Pathological and Molecular Features. A Single-Center Cohort Study.

Front Oncol 2021 12;11:637116. Epub 2021 Mar 12.

Hematology Division, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Lack of demonstrable mutations affecting , or driver genes within the spectrum of -negative myeloproliferative neoplasms (MPNs) is currently referred to as a triple-negative genotype, which is found in about 10% of patients with essential thrombocythemia (ET) and 5-10% of those with primary myelofibrosis (PMF). Very few papers are presently available on triple-negative ET, which is basically described as an indolent disease, differently from triple-negative PMF, which is an aggressive myeloid neoplasm, with a significantly higher risk of leukemic evolution. The aim of the present study was to evaluate the bone marrow morphology and the clinical-laboratory parameters of triple-negative ET patients, as well as to determine their molecular profile using next-generation sequencing (NGS) to identify any potential clonal biomarkers. Read More

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