6,872 results match your criteria Agnogenic Myeloid Metaplasia With Myelofibrosis


Feasibility of Qualitative Testing of BCR-ABL and JAK2 V617F in Suspected Myeloproliperative Neoplasm (MPN) Using RT-PCR Reversed Dot Blot Hybridization (RT-PCR RDB).

Clin Lymphoma Myeloma Leuk 2019 Jan 19. Epub 2019 Jan 19.

Stem Cell and Cancer Institute, Jakarta, Indonesia; Kalbe Genomics Laboratory, Jakarta, Indonesia.

Background: Defining the presence of BCR-ABL transcript in suspected myeloproliferative neoplasm is essential in establishing chronic myeloid leukemia. In the absence of BCR-ABL, the conventional diagnostic algorithm recommends JAK2 V617F mutation testing to support diagnosis of other MPN diseases such as polycythemia vera, essential thrombocythemia, and primary myelofibrosis. In certain cases of thrombocythemia, simultaneous upfront testing of both BCR-ABL and JAK2 may be desirable. Read More

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http://dx.doi.org/10.1016/j.clml.2019.01.005DOI Listing
January 2019
1 Read

F-FLT PET/MRI for bone marrow failure syndrome-initial experience.

EJNMMI Res 2019 Feb 15;9(1):16. Epub 2019 Feb 15.

Biomedical Imaging Research Center, University of Fukui, 23-3 Matsuoka-Shimoaizuki, Eiheiji-cho, Fukui, 910-1193, Japan.

Background: Bone marrow failure syndrome (BMFS) is a heterogeneous group of disorders associated with single- or multiple-lineage cytopenia and failure of normal hematopoiesis. We assessed the feasibility of integrated PET/MRI with 3'-deoxy-3'-F-fluorothymidine (F-FLT) to assess the pathophysiology of whole-body bone marrow for the diagnosis and monitoring of BMFS. Twenty-five consecutive patients with BMFS underwent a pre-treatment F-FLT PET/MRI scan. Read More

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http://dx.doi.org/10.1186/s13550-019-0490-0DOI Listing
February 2019
1 Read

The marrow stem cell niche in normal and malignant hematopoiesis.

Ann N Y Acad Sci 2019 Feb 14. Epub 2019 Feb 14.

Stony Brook University School of Medicine, Stony Brook, New York.

The hematopoietic niche is composed of endothelial cells, mesenchymal stromal cells of several types, and megakaryocytes, and functions to support the survival, proliferation, and differentiation of normal hematopoietic stem cells (HSCs). An abundance of evidence from a range of hematological malignancies supports the concept that the niche also participates in the pathogenesis of malignant hematopoiesis, differentially supporting malignant stem or progenitor cells over that of normal blood cell development. In 2005, patients with myeloproliferative neoplasms were reported to harbor an acquired, activating, missense V617F mutation of the cytokine-signaling Janus kinase (JAK)-2, JAK2 , present in virtually all patients with polycythemia vera and half of patients with essential thrombocythemia and primary myelofibrosis. Read More

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http://dx.doi.org/10.1111/nyas.14028DOI Listing
February 2019
1 Read

A 54-year-old Woman with Myelofibrosis and Massive Hemothorax Due to Primary Extramedullary Hematopoiesis of the Pleura.

Cureus 2018 Dec 3;10(12):e3675. Epub 2018 Dec 3.

Internal Medicine, Westchester Medical Center, Valhalla, USA.

Extramedullary hematopoiesis, which represents ectopic blood cell production, is usually an incidental finding accompanying hematologic pathology. The liver and spleen are the most common sites of extramedullary hematopoiesis, but thoracic involvement is likewise observed. Pleural effusions in the setting of intrathoracic extramedullary hematopoiesis have been attributed to mechanical interactions between the pleural surface and neighboring paravertebral masses consisting of hematopoietic tissue. Read More

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http://dx.doi.org/10.7759/cureus.3675DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367118PMC
December 2018
1 Read

Comprehensive clinical-molecular transplant scoring system for myelofibrosis undergoing stem cell transplantation.

Blood 2019 Feb 13. Epub 2019 Feb 13.

Department of Stem Cell Transplantation, University Medical Center Hamburg-Eppendorf, Hamburg, Germany;

Allogeneic hematopoietic stem cell transplantation is curative in myelofibrosis and current prognostic scoring systems aim to select patients for transplantation. Here, we aimed to develop a prognostic score to determine prognosis after transplantation itself using clinical, molecular and transplant-specific information of a total of 361 myelofibrosis patients. Of these, 205 patients were used as a training cohort to create a clinical-molecular myelofibrosis transplant scoring system (MTSS), which was then externally validated in a cohort of 156 patients. Read More

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http://www.bloodjournal.org/lookup/doi/10.1182/blood-2018-12
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http://dx.doi.org/10.1182/blood-2018-12-890889DOI Listing
February 2019
1 Read

[Clinical Analysis of 208 Patiets with BCR/ABL Negative Myeloproliferative Neoplasms].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2019 Feb;27(1):159-164

Department of Hematology, the Second Affiliated Hospital of Hebei Medical University, Shijiazhuang 050000, Hebei Province, China.E-mail:

Objective: To analyze the incidence, hemogram, genetics, clinical manifestations, therapeutic efficacy and outcome of patients with myeloproliferative neoplasms(MPN) so as to provide much more therapeutic basis for clinically studying the pathogenesis, diagnosis, and treatment as well as evaluating the prognosis of MPN patients.

Methods: The clinical data and related laboratory test results in 208 cases of BCR/ABL fusion gene regative MPN were collected and analyzed retrospectively.

Results: The MPN could occur at any age, but the highest incidence was observed in patients aged 40-79. Read More

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http://dx.doi.org/10.7534/j.issn.1009-2137.2019.01.025DOI Listing
February 2019
2 Reads

EDA fibronectin-TLR4 axis sustains megakaryocyte expansion and inflammation in bone marrow fibrosis.

J Exp Med 2019 Feb 7. Epub 2019 Feb 7.

Department of Molecular Medicine, University of Pavia, Pavia, Italy

The fibronectin EDA isoform (EDA FN) is instrumental in fibrogenesis but, to date, its expression and function in bone marrow (BM) fibrosis have not been explored. We found that mice constitutively expressing the EDA domain (EIIIA), but not EDA knockout mice, are more prone to develop BM fibrosis upon treatment with the thrombopoietin (TPO) mimetic romiplostim (TPO). Mechanistically, EDA FN binds to TLR4 and sustains progenitor cell proliferation and megakaryopoiesis in a TPO-independent fashion, inducing LPS-like responses, such as NF-κB activation and release of profibrotic IL-6. Read More

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http://dx.doi.org/10.1084/jem.20181074DOI Listing
February 2019
3 Reads

Long-term outcome after allogeneic hematopoietic cell transplantation for myelofibrosis.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

University Hospital Eppendorf, Hamburg, Germany.

Allogeneic hematopoietic stem cell transplant remains the only curative treatment for myelofibrosis. Most post-transplantation events occur during the first 2 years and hence we aimed to analyze the outcome of 2-year disease-free survivors. 1055 patients with myelofibrosis transplanted between 1995 and 2014 and registered in the registry of the European Society for Blood and Marrow Transplantation were included. Read More

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http://www.haematologica.org/lookup/doi/10.3324/haematol.201
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http://dx.doi.org/10.3324/haematol.2018.205211DOI Listing
February 2019
3 Reads

Discovery and evaluation of ZT55, a novel highly-selective tyrosine kinase inhibitor of JAK2 against myeloproliferative neoplasms.

J Exp Clin Cancer Res 2019 Feb 4;38(1):49. Epub 2019 Feb 4.

State Key Laboratory of Bioactive Substance and Function of Natural Medicines, Institute of Materia Medica, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100050, China.

Background: The JAK2-STAT signaling pathway plays a critical role in myeloproliferative neoplasms (MPN). An activating mutation in JAK2 (V617F) is present in ~ 95% of polycythemia vera, essential thrombocythemia, and primary myelofibrosis cases. This study aims to explore the selective JAK2 inhibitor, evaluate the efficacy and possible mechanism of ZT55 on MPN. Read More

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http://dx.doi.org/10.1186/s13046-019-1062-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360668PMC
February 2019
2 Reads

A Rare Case of Spontaneous Tumor Lysis Syndrome in Idiopathic Primary Myelofibrosis.

Am J Case Rep 2019 Feb 3;20:146-150. Epub 2019 Feb 3.

Department of Hemato-Oncology, Chosun University Hospital, Gwangju, South Korea.

BACKGROUND Tumor lysis syndrome (TLS) is an oncologic emergency resulting from the massive destruction of tumor cells after cytotoxic chemotherapy for chemosensitive malignancies with a high tumor burden. Its clinical manifestations include severe electrolyte disturbances, metabolic acidosis, acute renal failure secondary to urate deposition in the kidney, heart, and skeletal muscle, and nervous system dysfunction. We report an extremely rare case of spontaneous TLS (STLS) in idiopathic primary myelofibrosis (PMF). Read More

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http://dx.doi.org/10.12659/AJCR.912682DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6369657PMC
February 2019
2 Reads

[Ruxolitinib combined with prednisone, thalidomide and danazol for treatment of myelofibrosis: a pilot study].

Zhonghua Xue Ye Xue Za Zhi 2019 Jan;40(1):24-28

Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, The State Key Laboratory of Experimental Hematology, Tianjin 300020, China.

To evaluate the efficacy and tolerability of ruxolitinib combined with prednisone, thalidomide and danazol for treatment of in myelofibrosis (MF). Patients of MF according to the WHO 2016 criteria, received ruxolitinib (RUX) combined with prednisone, thalidomide and danazol (PTD). The response, changes of blood counts and adverse events were evaluated. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2019.01.005DOI Listing
January 2019
2 Reads

[A study of clinical characteristics and prognosis of primary myelofibrosis patients with thrombocytopenia in varied degrees].

Zhonghua Xue Ye Xue Za Zhi 2019 Jan;40(1):12-16

Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, The State Key Laboratory of Experimental Hematology, Tianjin 300020, China.

To evaluate clinical characteristics and prognosis of primary myelofibrosis (PMF) patients with thrombocytopenia in varied degrees. Clinical features and survival data of 1 305 Chinese patients with PMF were retrospectively analyzed. The prognostic value of thrombocytopenia in patients with PMF was evaluated. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2019.01.003DOI Listing
January 2019
1 Read

[Chinese guideline on the diagnosis and treatment of primary myelofibrosis (2019)].

Authors:

Zhonghua Xue Ye Xue Za Zhi 2019 Jan;40(1):1-7

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2019.01.001DOI Listing
January 2019
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Leukemic transformation among 1306 patients with primary myelofibrosis: risk factors and development of a predictive model.

Blood Cancer J 2019 Jan 25;9(2):12. Epub 2019 Jan 25.

Departments of Divisions of Hematology, Mayo Clinic, Rochester, MN, USA.

Among 1306 patients with primary myelofibrosis (PMF), we sought to identify risk factors that predicted leukemic transformation (LT) in the first 5 years of disease and also over the course of the disease. 149 (11%) LT were documented; patients who subsequently developed LT (n = 149), compared to those who remained in chronic phase disease (n = 1,157), were more likely to be males (p = 0.02) and display higher circulating blasts (p = 0. Read More

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http://www.nature.com/articles/s41408-019-0175-y
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http://dx.doi.org/10.1038/s41408-019-0175-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6347609PMC
January 2019
10 Reads

[Differential Diagnosis of Erythrocytosis - Background and Clinical Relevance].

Dtsch Med Wochenschr 2019 Jan 23;144(2):128-135. Epub 2019 Jan 23.

Due to its rare incidence, erythrocytosis frequently represents a challenge for the treating doctors. The erythropoiesis (= production of erythrocytes) is located in the bone marrow, and the hormone erythropoietin (EPO) takes control in its regulation. Therefore, measurement of EPO in serum is one of the main diagnostic steps. Read More

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http://dx.doi.org/10.1055/a-0739-8340DOI Listing
January 2019
5 Reads

Hypersegmentation of granulocytes and monocytes in patient with primary myelofibrosis treated with hydroxycarbamide

Turk J Haematol 2019 Jan 23. Epub 2019 Jan 23.

Central Laboratory at Public Central Teaching Hospital in Warsaw, Poland

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http://dx.doi.org/10.4274/tjh.2018.0395DOI Listing
January 2019

Detection of Mutations Using High Resolution Melting Curve Analysis (HRM-A); Application on a Large Cohort of Greek ET and MF Patients.

Mediterr J Hematol Infect Dis 2019 1;11(1):e2019009. Epub 2019 Jan 1.

Haematology Research Laboratory, Biomedical Research Foundation, Academy of Athens, Athens, Greece.

Background And Objectives: Somatic mutations in the calreticulin gene () are detected in approximately 70% of patients with essential thrombocythemia (ET) and primary or secondary myelofibrosis (MF), lacking the and mutations. To determine the prevalence of frameshift mutations in a population of MPN patients of Greek origin, we developed a rapid low-budget PCR-based assay and screened samples from 5 tertiary Haematology units. This is a first of its kind report of the Greek patient population that also disclosed novel mutants. Read More

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http://dx.doi.org/10.4084/MJHID.2019.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6328041PMC
January 2019
1 Read

Epigenomic profiling of myelofibrosis reveals widespread DNA methylation changes in enhancer elements and ZFP36L1 as a potential tumor suppressor gene epigenetically regulated.

Haematologica 2019 Jan 17. Epub 2019 Jan 17.

Centro de Investigacion Medica Aplicada, Universidad de Navarra, Pamplona, Spain;

In this study we have interrogated the DNA methylome of myelofibrosis patients using high-density DNA methylation arrays. We detected 35,215 differentially methylated CpGs corresponding to 10,253 genes between myelofibrosis patients and healthy controls. These changes were present both in primary and secondary myelofibrosis, which showed no differences between them. Read More

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http://www.haematologica.org/lookup/doi/10.3324/haematol.201
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http://dx.doi.org/10.3324/haematol.2018.204917DOI Listing
January 2019
5 Reads

Tuberculosis and atypical mycobacterial infections in ruxolitinib-treated patients with primary or secondary myelofibrosis or polycythemia vera.

Int J Infect Dis 2019 Jan 11;80:134-136. Epub 2019 Jan 11.

Department of Oncology and Haematology, Hôpitaux Universitaires de Strasbourg, Strasbourg, France; Université de Strasbourg, Inserm UMR-S1113/IRFAC, Strasbourg, France.

Ruxolitinib is a JAK-1/JAK-2 inhibitor indicated for the treatment of polycythemia vera and primary or secondary myelofibrosis. Only one patient (0.2%) was diagnosed with tuberculosis among the 485 patients receiving ruxolitinib in the four pivotal trials. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S12019712193001
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http://dx.doi.org/10.1016/j.ijid.2019.01.002DOI Listing
January 2019
8 Reads

Recurrent ischaemic cerebrovascular events as presenting manifestations of myeloproliferative neoplasms.

Eur J Neurol 2019 Jan 10. Epub 2019 Jan 10.

Department of Neurology and Stroke, Hertie Institute for Clinical Brain Research, Eberhard-Karls University of Tübingen, Tübingen, Germany.

Background And Purpose: Myeloproliferative neoplasms (MPNs) - polycythemia vera, essential thrombocythemia and primary myelofibrosis - are associated with increased risk for ischaemic cerebrovascular events (ICVEs). Due to their low prevalence, MPNs often remain undiagnosed as the cause of ICVEs.

Methods: Case records at the University of Tübingen between 2014 and 2017 were screened to identify patients with MPN-related ICVEs. Read More

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http://doi.wiley.com/10.1111/ene.13907
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http://dx.doi.org/10.1111/ene.13907DOI Listing
January 2019
9 Reads

Plerixafor for the Treatment of WHIM Syndrome.

N Engl J Med 2019 01;380(2):163-170

From the Laboratory of Molecular Immunology, National Institute of Allergy and Infectious Diseases (D.H.M., D.V., E.C., Q.L., P.M.M.), the Laboratories of Cellular Oncology (D.V.P., C.B.B.) and Pathology (S.P.), National Cancer Institute, the Department of Laboratory Medicine, Clinical Center (K.R.C.), the National Institute of Dental and Craniofacial Research (P.J.G.), and the National Institute on Deafness and Other Communication Disorders (D.A.B.), National Institutes of Health, and Kozloff and Trout MDs (H.H.T.), Bethesda, MD; the Infectious Diseases Unit and Primary Immunodeficiencies Unit, Hospital Dona Estefânia, Pediatric University Hospital (J.F.N.), and Centro de Imunodeficiências Primárias, Academic Medical Center of Lisbon (S.L.S.), Lisbon, Portugal; and the University of Chicago Medical Center, Chicago (E.A.B., E.M.L.).

WHIM syndrome (warts, hypogammaglobulinemia, infections, and myelokathexis), a primary immunodeficiency disorder involving panleukopenia, is caused by autosomal dominant gain-of-function mutations in CXC chemokine receptor 4 (CXCR4). Myelokathexis is neutropenia caused by neutrophil retention in bone marrow. Patients with WHIM syndrome are often treated with granulocyte colony-stimulating factor (G-CSF), which can increase neutrophil counts but does not affect cytopenias other than neutropenia. Read More

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http://dx.doi.org/10.1056/NEJMoa1808575DOI Listing
January 2019
1 Read

Splenic irradiation before allogeneic stem cell transplantation for myelofibrosis.

Med Oncol 2019 Jan 8;36(2):16. Epub 2019 Jan 8.

Department of Hematology and Bone Marrow Transplantation, School of Medicine in Katowice, Medical University of Silesia, Dąbrowski Street 25, 40-032, Katowice, Poland.

Splenectomy before allogeneic stem cell transplantation (ASCT) for patients with myelofibrosis (MF) remains a matter of debate, and conflicting results have been reported to date. The procedure seems to fasten post-transplant hematological recovery, but it does not have an impact on survival. The role of pre-transplant splenic irradiation (SI) is much more difficult to evaluate. Read More

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http://dx.doi.org/10.1007/s12032-019-1245-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6326000PMC
January 2019
1 Read

Decitabine combined with all-trans retinoic acid as treatment in a case of primary myelofibrosis transforming into acute myeloid leukaemia.

J Int Med Res 2019 Feb 7;47(2):1064-1071. Epub 2019 Jan 7.

Department of Haematology, The First People's Hospital of Changzhou, Third Affiliated Hospital of Soochow University, Changzhou, China.

Primary myelofibrosis (PMF) is a type of cloned myeloproliferative neoplasm stemming from haematopoietic stem cells, and tends to transform to acute myeloid leukaemia (AML) in approximately 10-20% of cases over a 10-year period. The transformation into AML has a poor prognosis, with a median overall survival of only 2.6 months in patients receiving supportive treatment. Read More

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http://dx.doi.org/10.1177/0300060518820147DOI Listing
February 2019
4 Reads

WT1 gene is overexpressed in myeloproliferative neoplasms, especially in myelofibrosis.

Blood Cells Mol Dis 2019 Mar 20;75:35-40. Epub 2018 Dec 20.

Université Angers, UFR Santé, Angers, France; CHU Angers, Laboratoire d'Hématologie, Angers, France; INSERM, CRCINA, Université de Nantes, Université d'Angers, France; Fédération Hospitalo-Universitaire 'Grand Ouest Against Leukemia' (FHU GOAL), France. Electronic address:

Classical Philadelphia-negative myeloproliferative neoplasms include Polycythemia Vera (PV), Essential Thrombocythemia (ET) and Primary Myelofibrosis (PMF). They are characterized by the presence of driver mutations of JAK2, CALR or MPL genes. Overexpression of WT1 is used as a marker of minimal residual disease in acute myeloid leukemia, especially after allogeneic stem cell transplantation (SCT). Read More

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http://dx.doi.org/10.1016/j.bcmd.2018.12.004DOI Listing
March 2019
3 Reads

Calreticulin mutation specific CAL2 immunohistochemistry accurately identifies rare calreticulin mutations in myeloproliferative neoplasms.

Pathology 2018 Dec 31. Epub 2018 Dec 31.

MTA-SE Momentum Molecular Oncohematology Research Group, Semmelweis University, 1st Department of Pathology and Experimental Cancer Research, Budapest, Hungary. Electronic address:

Mutations of the multifunctional protein calreticulin (CALR) are recognised as one of the main driver alterations involved in the pathogenesis of Philadelphia negative myeloproliferative neoplasms (Ph MPN) and also represent a major diagnostic criterion in the most recent World Health Organization classification of myeloid neoplasms. Nowadays, quantitative assessment of the driver mutations is gaining importance, as recent studies demonstrated the clinical relevance of the mutation load reflecting the size of the mutant clone. Here, we performed for the first time a manual and automated quantitative assessment of the CALR mutation load at protein level using CAL2, a recently developed CALR mutation specific monoclonal antibody, on a cohort of 117 patients with essential thrombocythemia (ET) or primary myelofibrosis (PMF) and compared the CALR protein mutation loads with the CALR mutation load values established by a molecular assay. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00313025183038
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http://dx.doi.org/10.1016/j.pathol.2018.11.007DOI Listing
December 2018
3 Reads

Luspatercept for the treatment of anemia in myelodysplastic syndromes and primary myelofibrosis.

Blood 2019 Feb 2;133(8):790-794. Epub 2019 Jan 2.

Medical Clinic and.

Anemia of lower-risk myelodysplastic syndromes (MDSs) and primary myelofibrosis (PMF) generally becomes resistant to available treatments, leading to red blood cell (RBC) transfusions, iron overload, shortened survival, and poor quality of life. The transforming growth factor-β superfamily, including activins and growth differentiation factors (GDFs), is aberrantly expressed in lower-risk MDSs and PMF. Luspatercept (and sotatercept), ligand traps that particularly inhibit GDF11, lead to RBC transfusion independence in 10% to 50% of lower-risk MDSs resistant to available treatments, and have started to be used in PMF. Read More

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http://dx.doi.org/10.1182/blood-2018-11-876888DOI Listing
February 2019
3 Reads

Clinicopathological and molecular features of SF3B1-mutated myeloproliferative neoplasms.

Hum Pathol 2018 Dec 27. Epub 2018 Dec 27.

Department of Pathology, Massachusetts General Hospital, 02114, Boston, MA, USA; Harvard Medical School, 02114, Boston, MA, USA. Electronic address:

The introduction of next-generation sequencing has broadened the genetic landscape of myeloproliferative neoplasms (MPNs) beyond JAK2, MPL and CALR. However, the biological role and clinical impact of most other mutations is not well defined. We interrogated 101 genes in 143 BCR-ABL1-negative MPNs in chronic phase from two large institutions. Read More

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http://dx.doi.org/10.1016/j.humpath.2018.11.022DOI Listing
December 2018
1 Read

EVALUATION OF BURDENSOME SYMPTOMS IN PATIENTS WITH RADIATION6ASSOCIATED AND SPONTANEOUS MYELOPROILIFERATIVE NEOPLASMS WITH THE USE OF OPTIMIZED SELF-ASSESSMENT MPN-SAF TSS.

Probl Radiac Med Radiobiol 2018 Dec;23:510-516

State Institution «National Research Center for Radiation Medicine of the National Academy of Medical Sciences of Ukraine», 53 Melnykova str., Kyiv, 04050, UkraineBogomolets National Medical University, 13, Tarasa Shevchenko Blvd, Kyiv, 01601, Ukraine.

Objective: To investigate the intensity of burdensome symptoms using self-assessment MPN-SAF TSS in patientswith radiation-associated and spontaneous myeloproiliferative neoplasms (MPNs).

Materials And Methods: The study included 89 patients with radiation-associated and spontaneous MPNs, the bur-densome symptoms of MPN were determined using MPN-SAF TSS.

Results: The average score for complaints in patients with radiation-associated MPNs was significantly higher thanin patients with spontaneous MPNs - 43. Read More

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http://dx.doi.org/10.33145/2304-8336-2018-23-510-516DOI Listing
December 2018
1 Read

β-catenin and PPAR-γ levels in bone marrow of myeloproliferative neoplasm: an immunohistochemical and ultrastructural study.

Ultrastruct Pathol 2018 Nov-Dec;42(6):498-507. Epub 2018 Dec 24.

a Department of Molecular Oncology , Institute for Medical Research, University of Belgrade , Belgrade , Serbia.

In accordance with increased proliferation in myeloproliferative neoplasm (MPN), the goal is to evaluate the immunoexpression of: β-catenin, PPAR-γ and Ki67 protein, to compare them with bone marrow ultrastructural characteristics in patients with MPN. Immunoexpression and electron microscopy of bone marrow was analyzed in 30 Ph-negative MPN patients, including per 10 patients with polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). The quantity of β-catenin immunoreactive cells was significantly higher in PV then in ET (p < 0. Read More

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http://dx.doi.org/10.1080/01913123.2018.1558323DOI Listing
December 2018
4 Reads
1.133 Impact Factor

Busulfan- or Thiotepa-Based Conditioning in Myelofibrosis: A Phase II Multicenter Randomized Study from the GITMO Group.

Biol Blood Marrow Transplant 2018 Dec 20. Epub 2018 Dec 20.

"Papa Giovanni XXIII" Hospital, Bergamo, Italy; Department of Hematology-Oncology, University of Milano, Milan, Italy.

We report a randomized study comparing fludarabine in combination with busulfan (FB) or thiotepa (FT), as conditioning regimen for hematopoietic stem cell transplantation (HSCT) in patients with myelofibrosis. The primary study endpoint was progression-free survival (PFS). Sixty patients were enrolled with a median age of 56 years and an intermediate-2 or high-risk score in 65%, according to the Dynamic International Prognostic Staging System (DIPSS). Read More

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http://dx.doi.org/10.1016/j.bbmt.2018.12.064DOI Listing
December 2018
8 Reads

Genetic studies in the evaluation of myeloproliferative neoplasms.

Semin Hematol 2019 Jan 28;56(1):7-14. Epub 2018 May 28.

University of Pennsylvania, Department of Pathology and Laboratory Medicine, Division of Hematopathology, Philadelphia, PA.

Myeloproliferative neoplasms that include the specific entities of chronic myeloid leukemia, chronic neutrophilic leukemia, polycythemia vera, essential thrombocythemia, and primary myelofibrosis are characterized by the clonal expansion of hematopoietic precursor cells and consequent neoplastic production of mature cells of myeloid, erythroid, and/or megakaryocytic lineage. Genetic studies, encompassing both cytogenetic and molecular testing, play a central and ever increasing role in the assessment of these neoplasms and are the focus of this review. Read More

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http://dx.doi.org/10.1053/j.seminhematol.2018.05.004DOI Listing
January 2019
1 Read
3.274 Impact Factor

IL-6 stimulation of DNA replication is JAK1/2 mediated in cross-talk with hyperactivated ERK1/2 signaling.

Cell Biol Int 2019 Feb 7;43(2):192-206. Epub 2019 Jan 7.

Department of Molecular Oncology, Institute for Medical Research, University of Belgrade, Belgrade, Serbia.

Myeloproliferative neoplasms (MPNs) are developing resistance to therapy by JAK1/2 inhibitor ruxolitinib. To explore the mechanism of ruxolitinib's limited effect, we examined the JAK1/2 mediated induction of proliferation related ERK1/2 and AKT signaling by proinflammatory interleukin-6 (IL-6) in MPN granulocytes and JAK2V617F mutated human erythroleukemia (HEL) cells. We found that JAK1/2 or JAK2 inhibition prevented the IL-6 activation of STAT3 and AKT pathways in polycythemia vera and HEL cells. Read More

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http://dx.doi.org/10.1002/cbin.11084DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6347554PMC
February 2019
3 Reads
1.933 Impact Factor

Oxidative and nitrosative stress in myeloproliferative neoplasms: the impact on the AKT / mTOR signaling pathway.

J BUON 2018 Sep-Oct;23(5):1481-1491

Deparment of Molecular Biology, Institute for Medical Research, University of Belgrade, Belgrade, Serbia.

Purpose: A common feature of malignancies is increased reactive oxygen species (ROS) and reactive nitrogen species (RNS). We analyzed the influence of oxidative and nitrosative stress on the activation of AKT/mTOR signaling pathway in myeloproliferative neoplasms (MPN).

Methods: Oxidative stress-induced gene expression in circulatory CD34+ cells of MPN patients was studied by microarray analysis. Read More

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December 2018
11 Reads

The epidemiology and clinical characteristics of myeloproliferative neoplasms in Malaysia.

Exp Hematol Oncol 2018 17;7:31. Epub 2018 Dec 17.

Department of Haematology, Sunway Medical Centre, Kuala Lumpur, Malaysia.

Background: The evolution of molecular studies in myeloproliferative neoplasms (MPN) has enlightened us the understanding of this complex disease consisting of polycythaemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). The epidemiology is well described in the western world but not in Asian countries like Malaysia.

Materials And Methods: This retrospective national registry of MPN was conducted from year 2009 to 2015 in Malaysia. Read More

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https://ehoonline.biomedcentral.com/articles/10.1186/s40164-
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http://dx.doi.org/10.1186/s40164-018-0124-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6296047PMC
December 2018
20 Reads

Nuclear-Cytoplasmic Transport Is a Therapeutic Target in Myelofibrosis.

Clin Cancer Res 2018 Dec 18. Epub 2018 Dec 18.

Huntsman Cancer Institute, The University of Utah, Salt Lake City, Utah.

Myelofibrosis is a hematopoietic stem cell neoplasm characterized by bone marrow reticulin fibrosis, extramedullary hematopoiesis, and frequent transformation to acute myeloid leukemia. Constitutive activation of JAK/STAT signaling through mutations in , or is central to myelofibrosis pathogenesis. JAK inhibitors such as ruxolitinib reduce symptoms and improve quality of life, but are not curative and do not prevent leukemic transformation, defining a need to identify better therapeutic targets in myelofibrosis. Read More

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http://dx.doi.org/10.1158/1078-0432.CCR-18-0959DOI Listing
December 2018
11 Reads
8.722 Impact Factor

Advanced forms of MPNs are accompanied by chromosomal abnormalities that lead to dysregulation of TP53.

Blood Adv 2018 Dec;2(24):3581-3589

Tisch Cancer Institute, Division of Hematology/Oncology, and.

The Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocythemia (ET), and the prefibrotic form of primary myelofibrosis (PMF), frequently progress to more overt forms of MF and a type of acute leukemia termed MPN-accelerated phase/blast phase (MPN-AP/BP). Recent evidence indicates that dysregulation of the tumor suppressor tumor protein p53 (TP53) commonly occurs in the MPNs. The proteins MDM2 and MDM4 alter the cellular levels of TP53. Read More

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http://dx.doi.org/10.1182/bloodadvances.2018024018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6306879PMC
December 2018
9 Reads

Safety and efficacy of combined ruxolitinib and decitabine in accelerated and blast-phase myeloproliferative neoplasms.

Blood Adv 2018 Dec;2(24):3572-3580

Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY.

Myeloproliferative neoplasms (MPN), including polycythemia vera, essential thrombocythemia, and primary myelofibrosis, have a propensity to evolve into accelerated and blast-phase disease (MPN-AP/BP), carrying a dismal prognosis. Conventional antileukemia therapy has limited efficacy in this setting. Thus, MPN-AP/BP is an urgent unmet clinical need. Read More

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http://dx.doi.org/10.1182/bloodadvances.2018019661DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6306885PMC
December 2018
2 Reads

[A case of gout secondary to primary myelofibrosis].

Beijing Da Xue Xue Bao Yi Xue Ban 2018 Dec;50(6):1117-1119

Department of Rheumatology and Immunology,Peking University First Hospital, Beijing 100034, China.

A 52-year-old man was referred to our department with a 2-year history of polyarthritis. He was diagnosed as gout due to acute arthritis of bilateral feet dorsum 2 years ago,but he didn't receive any standard treatment. 1 year ago,there were more and more joints evolved during the gout attack, and many subcutaneous nodules occurred. Read More

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December 2018
1 Read

The ruxolitinib effect: understanding how molecular pathogenesis and epigenetic dysregulation impact therapeutic efficacy in myeloproliferative neoplasms.

J Transl Med 2018 Dec 17;16(1):360. Epub 2018 Dec 17.

Centre for Medical Education, Queen's University Belfast, Belfast, UK.

The myeloproliferative neoplasms (MPN), polycythaemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) are linked by a propensity to thrombosis formation and a risk of leukaemic transformation. Activation of cytokine independent signalling through the JAK/STAT cascade is a feature of these disorders. A point mutation in exon 14 of the JAK2 gene resulting in the formation of the JAK2 V617F transcript occurs in 95% of PV patients and around 50% of ET and PMF patients driving constitutive activation of the JAK/STAT pathway. Read More

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http://dx.doi.org/10.1186/s12967-018-1729-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6296062PMC
December 2018
1 Read

Do All Patients With Polycythemia Vera or Essential Thrombocythemia Need Cytoreduction?

J Natl Compr Canc Netw 2018 Dec;16(12):1539-1545

Department of Medicine, and Division of Hematology and Oncology, Northwestern University Feinberg School of Medicine; and Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois.

Polycythemia vera (PV) and essential thrombocythemia (ET) are Philadelphia chromosome-negative chronic myeloproliferative neoplasms (MPNs), characterized by expansion of normal blood counts, bleeding, thrombosis, and the potential for transformation to myelofibrosis (MF) or acute myeloid leukemia (AML). The primary goals of treatment for MPNs are to reduce the risk of thrombosis, alleviate systemic symptom burden (eg, fatigue, pruritus, microvascular symptoms, and symptomatic splenomegaly), and to prevent transformation to MF/AML. Preventing transformation is clearly important, but not expected with current therapies. Read More

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http://dx.doi.org/10.6004/jnccn.2018.7073DOI Listing
December 2018

Ruxolitinib in combination with prednisone and nilotinib exhibit synergistic effects in human cells lines and primary cells from myeloproliferative neoplasms.

Haematologica 2018 Dec 13. Epub 2018 Dec 13.

Hematology Service, Hospital Universitario 12 de Octubre, Madrid, Spain.

Ruxolitinib is the frontline non-palliative treatment for myelofibrosis; however, a significant number of patients lose or present suboptimal response, are resistant or have unacceptable toxicity. In an attempt to improve the response and avoid the adverse effects of this drug, we evaluated the combination of 17 drugs with ruxolitinib in ex vivo models of peripheral blood mononuclear cells from patients with myelofibrosis and cell lines. We found that the combination ruxolitinib and nilotinib had a synergistic effect against myelofibrosis cells (ΔEC50 nilotinib = -21. Read More

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http://dx.doi.org/10.3324/haematol.2018.201038DOI Listing
December 2018

Systemic mastocytosis with an associated hematological neoplasm masquerading as overt primary myelofibrosis.

Blood 2018 Dec;132(24):2613

Royal Jubilee Hospital.

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http://dx.doi.org/10.1182/blood-2018-09-875914DOI Listing
December 2018
10.452 Impact Factor

CD47 expression is decreased in hematopoietic progenitor cells in patients with myelofibrosis.

Braz J Med Biol Res 2018 Dec 10;52(1):e7784. Epub 2018 Dec 10.

Sabin Medicina Diagnóstica, Brasília, DF, Brasil.

Myelofibrosis (MF) is characterized by increased circulating hematopoietic progenitor cells (HPCs), abnormal cytokine levels, and the survival advantage of neoplastic progenitors over their normal counterparts, which leads to progressive disappearance of polyclonal hematopoiesis. CD47 is a surface glycoprotein with many functions, such as acting as a phagocytosis inhibitor of the expressing cell, that is increased in normal hematopoietic stem and progenitor cells mobilized into the blood and several human cancer-initiating cells, such as in acute myeloid leukemia. We compared CD47 expression in hematopoietic stem and progenitor cells of patients with MF and controls and found it to be decreased in progenitors of MF. Read More

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http://dx.doi.org/10.1590/1414-431X20187784DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6301265PMC
December 2018
8 Reads

Heterogeneity in myeloproliferative neoplasms: Causes and consequences.

Adv Biol Regul 2019 Jan 22;71:55-68. Epub 2018 Nov 22.

MRC Molecular Haematology Unit, MRC Weatherall Institute of Molecular Medicine, Radcliffe Department of Medicine, University of Oxford, Oxford, OX3 9DS, United Kingdom; NIHR Biomedical Research Centre, Churchill Hospital, Oxford, UK. Electronic address:

Myeloproliferative neoplasms (MPNs) are haematopoietic stem cell-derived clonal disorders characterised by proliferation of some or all myeloid lineages, depending on the subtype. MPNs are classically categorized into three disease subgroups; essential thrombocythaemia (ET), polycythaemia vera (PV) and primary myelofibrosis (PMF). The majority (>85%) of patients carry a disease-initiating or driver mutation, the most prevalent occurring in the janus kinase 2 gene (JAK2 V617F), followed by calreticulin (CALR) and myeloproliferative leukaemia virus (MPL) genes. Read More

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http://dx.doi.org/10.1016/j.jbior.2018.11.007DOI Listing
January 2019
1 Read

Managing myelofibrosis (MF) that "blasts" through: advancements in the treatment of relapsed/refractory and blast-phase MF.

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):118-126

Mays Cancer Center, UT Health San Antonio MD Anderson Cancer Center, San Antonio, TX.

Myelofibrosis (MF) is the most aggressive form of Philadelphia chromosome-negative myeloproliferative neoplasm, and it is complicated by severe symptom burden, thrombotic events, infections, cytopenias, and transformation to acute myeloid leukemia (AML). Ruxolitinib, the first-line therapy for symptomatic or intermediate- and high-prognostic risk MF, has improved overall survival for this population. However, approximately one-half of MF patients will discontinue ruxolitinib by the first few years of therapy due to a spectrum of resistance, intolerance, relapse, or progression to blast phase disease. Read More

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http://dx.doi.org/10.1182/asheducation-2018.1.118DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245993PMC
November 2018
8 Reads

Differences in presenting features, outcome and prognostic models in patients with primary myelofibrosis and post-polycythemia vera and/or post-essential thrombocythemia myelofibrosis treated with ruxolitinib. New perspective of the MYSEC-PM in a large multicenter study.

Semin Hematol 2018 Oct 5;55(4):248-255. Epub 2018 Jun 5.

Institute of Hematology "L. and A. Seràgnoli", Sant'Orsola-Malpighi University Hospital, Bologna, Italy.

Recently, the myelofibrosis secondary to PV and ET prognostic model (MYSEC-PM) was introduced to assess prognosis in myelofibrosis (MF) secondary to polycythemia vera and essential thrombocythemia (post-PV and post-ET MF), replacing the International Prognostic Scoring System (IPSS) and/or Dynamic IPSS (DIPSS) that was applied for primary MF (PMF). In a cohort of 421 ruxolitinib (RUX)-treated patients (post-PV and post-ET MF: 44.2%), we evaluated the following: (1) disease phenotype, responses, and toxicity to RUX; and (2) performance of the MYSEC-PM in post-PV or post-ET MF. Read More

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http://dx.doi.org/10.1053/j.seminhematol.2018.05.013DOI Listing
October 2018
3 Reads

Somatic Mutations in Philadelphia Chromosome-Negative Myeloproliferative Neoplasms.

Semin Hematol 2018 Oct 17;55(4):215-222. Epub 2018 Apr 17.

Serviço de Hematologia e Transplantação de Medula, Hospital de Santa Maria, Lisboa, Portugal.

Myeloproliferative neoplasms (MPN) include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). MPN are characterized by clonal proliferation of myeloid progenitors leading to erythrocytosis, thrombocytosis, or leukocytosis, and risk of hemorrhagic and thrombotic events, as well as myelofibrosis and blast transformation. The discovery of somatic mutations in MPN, namely JAK2 V617F, JAK2 exon 12, MPL, and CALR mutations, has permitted a more specific approach to diagnosis and treatment. Read More

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http://dx.doi.org/10.1053/j.seminhematol.2018.04.005DOI Listing
October 2018
4 Reads

20+ Years and alive with primary myelofibrosis: Phenotypic signature of very long-lived patients.

Am J Hematol 2019 Mar 5;94(3):286-290. Epub 2018 Dec 5.

Divisions of Hematology, Mayo Clinic, Rochester, Minnesota.

In the last decade, several prognostic models for primary myelofibrosis (PMF) have been introduced and shown to be effective in predicting overall survival. The main objective for this study was to identify clinical and genetic markers of very long (20+ years) survival in PMF. A total of 1282 patients with PMF were considered (median age 65 years, range 19-92; 63% males); 26 (2%) patients (median age 51 years, range 28-71; 38% males) survived their disease for at least 20 years (long-lived patients) and 626 (49%) patients (median age 68 years, range 27-92; 66% males) died within 5 years of their diagnosis (short-lived patients). Read More

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http://doi.wiley.com/10.1002/ajh.25351
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http://dx.doi.org/10.1002/ajh.25351DOI Listing
March 2019
7 Reads

The germline JAK2 GGCC (46/1) haplotype and survival among 414 molecularly-annotated patients with primary myelofibrosis.

Am J Hematol 2019 Mar 5;94(3):299-305. Epub 2018 Dec 5.

Division of Hematology, Mayo Clinic, Rochester, Minnesota.

JAK2 mutations in myeloproliferative neoplasms (MPNs) are associated with the germline GGCC (46/1) haplotype. In 2010, we reported an association between shortened survival in primary myelofibrosis (PMF) and nullizygosity for the JAK2 46/1 haplotype. In the current study, we have increased the number of informative cases from 130 to 414 (median age 63 years; 63% males), in order to revisit with the phenotypic and prognostic relevance of the JAK2 46/1 haplotype in PMF. Read More

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http://dx.doi.org/10.1002/ajh.25349DOI Listing
March 2019
3 Reads

Next-generation sequencing with a 54-gene panel identified unique mutational profile and prognostic markers in Chinese patients with myelofibrosis.

Ann Hematol 2018 Dec 4. Epub 2018 Dec 4.

Department of Medicine, The University of Hong Kong, Hong Kong, China.

Current prognostication in myelofibrosis (MF) is based on clinicopathological features and mutations in a limited number of driver genes. The impact of other genetic mutations remains unclear. We evaluated for mutations in a myeloid panel of 54 genes using next-generation sequencing. Read More

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http://link.springer.com/10.1007/s00277-018-3563-7
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http://dx.doi.org/10.1007/s00277-018-3563-7DOI Listing
December 2018
4 Reads
2.634 Impact Factor