6,917 results match your criteria Agnogenic Myeloid Metaplasia With Myelofibrosis


Chromosome 12 Rearrangement in an Adolescent with Primary Myelofibrosis.

Indian J Hematol Blood Transfus 2019 Apr 6;35(2):387-389. Epub 2018 Dec 6.

1Molecular Pathology and Cytogenetics Section, Pathology Department, Children'S Medical Center, Tehran University of Medical Sciences, Tehran, Iran.

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http://dx.doi.org/10.1007/s12288-018-1055-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439120PMC

Impact of 2016-WHO diagnosis of early and overt primary myelofibrosis on presentation and outcome of 232 patients treated with ruxolitinib.

Hematol Oncol 2019 Apr 15. Epub 2019 Apr 15.

Division of Cellular Biotechnologies and Hematology, University Sapienza, Roma.

2016-WHO criteria identified early-Primary Myelofibrosis as an individual entity with milder clinical features and better outcome compared to overt-PMF. Here, we compared early and overt-PMF patients treated with ruxolitinib in terms of baseline clinical/laboratory characteristics, response and toxicity to treatment. We observed that early-PMF patients achieve better and more stable spleen and symptoms responses, with significantly lower rates of haematological toxicities. Read More

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http://dx.doi.org/10.1002/hon.2619DOI Listing
April 2019
1 Read

Splenectomy following JAK1/JAK2 inhibitor therapy in patients with myelofibrosis undergoing allogeneic stem cell transplantation.

Hematol Oncol Stem Cell Ther 2019 Apr 6. Epub 2019 Apr 6.

Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation, Department of Hematology, Transfusiology, Transplantation, Faculty of Postgraduate Education, Pavlov First Saint Petersburg State Medical University, Saint-Petersburg, Russia.

Background: Allogeneic hematopoietic stem cell transplantation (alloHSCT) is the only treatment option with curative potential in patients with myelofibrosis (MF). The aim of our study was to evaluate the safety of splenectomy before alloHSCT in MF patients who failed to achieve significant spleen response after ruxolitinib therapy.

Methods: Splenectomy was performed in 12 patients for alloHSCT with myelofibrosis-primary (6 patients), post-polycythemia vera (3 patients). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S16583876193002
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http://dx.doi.org/10.1016/j.hemonc.2019.03.001DOI Listing
April 2019
4 Reads

Clinical and molecular features of patients with prefibrotic primary myelofibrosis previously diagnosed as having essential thrombocythemia in Japan.

Eur J Haematol 2019 Apr 12. Epub 2019 Apr 12.

Department of Hematology and Stem Cell Regulation, Juntendo University Graduate School of Medicine, Tokyo.

Objective: Prefibrotic/early primary myelofibrosis (pre-PMF) and essential thrombocythemia (ET) exhibited different features of bone marrow, however, this is not always easy to judge objectively, making pathologists' distinction often suboptimal. In the WHO 2008 criteria, pre-PMF was not defined as a subgroup of PMF; therefore, affected patients were at a higher risk of misdiagnosis with ET. In this study, we examined the prevalence of pre-PMF patients among those previously diagnosed with ET in Japan. Read More

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http://dx.doi.org/10.1111/ejh.13236DOI Listing
April 2019
1 Read

Development of a symptom assessment in patients with myelofibrosis: qualitative study findings.

Health Qual Life Outcomes 2019 Apr 11;17(1):61. Epub 2019 Apr 11.

Guy's and St. Thomas' NHS Foundation Trust, St. Thomas Hospital, Westminster Bridge Rd. Lambeth, London, SE1 7EH, UK.

Background: The goal of the research reported here was to understand the patient experience of living with myelofibrosis (MF) and establish content validity of the Modified Myeloproliferative Neoplasm Symptom Assessment Diary (MPN-SD).

Methods: Qualitative interviews were performed in patients with MF, including both concept elicitation and cognitive debriefing. Patients with MF were asked to spontaneously report on their signs, symptoms, and impacts of MF, as well as their understanding of the MPN-SD content, and use of the tool on an electronic platform. Read More

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http://dx.doi.org/10.1186/s12955-019-1121-1DOI Listing
April 2019
1 Read

Osteolytic Lesions in Primary Myelofibrosis and Effect of Ruxolitinib Therapy: Report of a Case and Literature Review.

Chemotherapy 2019 Apr 9;63(6):340-344. Epub 2019 Apr 9.

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

Here, we report the case of a young female affected by primary myelofibrosis (PMF) who developed an osteolytic lesion of the humerus during the follow-up, and the possible efficacy of ruxolitinib in controlling this rare event. After 26 years of follow-up, the patient reported onset of acute pain at the proximal region of the left upper limb. An X-ray revealed an osteolytic bone lesion in the proximal third of the humeral shaft, which was then confirmed by magnetic resonance imaging. Read More

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http://dx.doi.org/10.1159/000497246DOI Listing
April 2019
3 Reads

Pre-hematopoietic cell transplant Ruxolitinib in patients with primary and secondary myelofibrosis.

Bone Marrow Transplant 2019 Apr 8. Epub 2019 Apr 8.

Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA, USA.

Ruxolitinib (Rux), a Jak1/2 inhibitor, results in reduced spleen size and improvement in constitutional symptoms in the majority of patients with myelofibrosis (MF). Therefore Rux, when given prior to hematopoietic cell transplantation (HCT) in patients with MF was hypothesized to improve engraftment, decrease incidence and severity of graft-versus-host disease, and lower non-relapse mortality (NRM). We conducted a phase II prospective trial to assess the effects of pre-HCT Rux on post-HCT outcomes in patients with MF. Read More

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http://dx.doi.org/10.1038/s41409-019-0523-3DOI Listing
April 2019
1 Read

Characteristics and Outcomes of Young Adults with Philadelphia negative Myeloproliferative Neoplasms.

Eur J Haematol 2019 Apr 2. Epub 2019 Apr 2.

Rappaport faculty of medicine, Technion, Haifa, Israel.

Objective: Approximately 10% of Philadelphia (Ph) negative myeloproliferative neoplasms (NPM) are diagnosed at young adulthood. We aim to define the features of this group.

Methods: A multicenter retrospective study, including patients 18-45 years of age, diagnosed with Ph negative MPN between 1985-2017. Read More

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http://dx.doi.org/10.1111/ejh.13232DOI Listing
April 2019
5 Reads

Increased monocyte count as a cellular biomarker for poor outcomes in fibrotic diseases: a retrospective, multicentre cohort study.

Lancet Respir Med 2019 Mar 29. Epub 2019 Mar 29.

Institute for Immunity, Transplantation, and Infection, Stanford University School of Medicine, Stanford, CA, USA; Division for Biomedical Informatics Research, Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA. Electronic address:

Background: There is an urgent need for biomarkers to better stratify patients with idiopathic pulmonary fibrosis by risk for lung transplantation allocation who have the same clinical presentation. We aimed to investigate whether a specific immune cell type from patients with idiopathic pulmonary fibrosis could identify those at higher risk of poor outcomes. We then sought to validate our findings using cytometry and electronic health records. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22132600183050
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http://dx.doi.org/10.1016/S2213-2600(18)30508-3DOI Listing
March 2019
6 Reads

Copy number alterations and copy-neutral loss of heterozygosity in Ukrainian patients with primary myelofibrosis.

Exp Oncol 2019 Mar;41(1):53-56

Cancer Research Institute, Beth Israel Deaconess Medical Center, Boston 02215, MA, USA.

Aim: To examine frequencies and spectrum of genomic alterations in Ukrainian patients diagnosed with primary myelofibrosis (PMF).

Materials And Methods: We enrolled 30 Ukrainian patients diagnosed with PMF who were previously tested for usual mutations in mye-loproliferative neoplasms driver genes (JAK2, MPL and CALR). Genomic DNA samples were obtained from peripheral blood leukocytes of these patients. Read More

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March 2019
1 Read

[How I treat primary myelofibrosis].

Authors:
Z J Xiao

Zhonghua Xue Ye Xue Za Zhi 2019 Mar;40(3):179-181

Blood Diseases Hospital and Institute of Hematology, CAMS & PUMC, Tianjin 300020, China.

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2019.03.002DOI Listing
March 2019
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Mice with Calr mutations homologous to human CALR mutations only exhibit mild thrombocytosis.

Blood Cancer J 2019 Mar 29;9(4):42. Epub 2019 Mar 29.

Department of Gastroenterology and Hematology, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan.

Calreticulin (CALR) exon 9 frameshift mutations, commonly detected in essential thrombocythemia (ET) and primary myelofibrosis patients, activate signal transducer and activator of transcription (STAT) proteins in the presence of Myeloproliferative Leukemia Virus (MPL) and induce ET in vivo. Loss of the KDEL motif, an endoplasmic reticulum retention signal, and generation of many positively charged amino acids (AAs) in the mutated C-terminus are thought to be important for disease induction. To test this hypothesis, we generated mice harboring a Calr frameshift mutation using the CRISPR/Cas9 system. Read More

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http://dx.doi.org/10.1038/s41408-019-0202-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6440999PMC
March 2019
2 Reads

Pulmonary hypertension with massive megalosplenia: A case report.

Medicine (Baltimore) 2019 Mar;98(12):e14594

Department of Cardiology.

Rationale: Pulmonary hypertension (PH) is a complicated disease which has complex causes and poor outcome. Many factors are involved in the increase of pulmonary artery pressure. It is often difficult to identify the specific cause of a particular patient. Read More

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http://dx.doi.org/10.1097/MD.0000000000014594DOI Listing
March 2019
2 Reads

Phase 1/2 trial of glasdegib in patients with primary or secondary myelofibrosis previously treated with ruxolitinib.

Leuk Res 2019 Apr 28;79:38-44. Epub 2019 Feb 28.

Comprehensive Cancer Center, University of Michigan, 1500 East Medical Center Dr, Ann Arbor, MI 48109, USA. Electronic address:

Glasdegib is a potent and selective oral inhibitor of the Hedgehog pathway. We report data from the single-arm, lead-in cohort of an open-label phase 1b/2 trial of glasdegib in patients with primary/secondary myelofibrosis (MF) previously treated with at least one Janus kinase inhibitor (JAKi). Patients received glasdegib 100 mg orally once daily until there was no further clinical benefit. Read More

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http://dx.doi.org/10.1016/j.leukres.2019.02.012DOI Listing
April 2019
8 Reads

Comparison of Outcomes of Allogeneic Transplantation for Primary Myelofibrosis among Hematopoietic Stem Cell Source Groups.

Biol Blood Marrow Transplant 2019 Mar 1. Epub 2019 Mar 1.

Department of Hematology, Aiiku Hospital, Sapporo, Japan.

The choice of alternative donor is a major issue in allogeneic hematopoietic stem cell transplantation (HSCT) for patients with primary myelofibrosis (PMF) without an HLA-matched related donor. We conducted this retrospective study using the Japanese national registry data for 224 PMF patients to compare the outcomes of first allogeneic HSCT from HLA-matched related donor bone marrow (Rtd-BM), HLA-matched related donor peripheral blood stem cells (Rtd-PB), HLA-matched unrelated donor bone marrow (UR-BM), unrelated umbilical cord blood (UR-UCB), and other hematopoietic stem cell grafts. Nonrelapse mortality (NRM) rates at 1 year after Rtd-BM, Rtd-PB, UR-BM, UR-UCB, and other transplantations were 16%, 36%, 30%, 41%, and 48%, respectively. Read More

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http://dx.doi.org/10.1016/j.bbmt.2019.02.019DOI Listing
March 2019
3 Reads

3023 Mayo Clinic Patients With Myeloproliferative Neoplasms: Risk-Stratified Comparison of Survival and Outcomes Data Among Disease Subgroups.

Mayo Clin Proc 2019 Apr 26;94(4):599-610. Epub 2019 Feb 26.

Division of Hematology, Department of Internal and Laboratory Medicine, Mayo Clinic, Rochester, MN. Electronic address:

Objective: To document the Mayo Clinic decades-long experience with myeloproliferative neoplasms (MPNs) and provide mature risk-stratified survival data and disease complication estimates.

Patients And Methods: All Mayo Clinic patients with World Health Organization-defined MPNs constituted the core study group and included those with polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF).

Results: A total of 3023 consecutive patients (median age, 62 years; range, 18-96 years) were considered: 665 PV, 1076 ET, and 1282 PMF. Read More

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http://dx.doi.org/10.1016/j.mayocp.2018.08.022DOI Listing
April 2019
5 Reads

A systematic review and meta-analysis of the prevalence of thrombosis and bleeding at diagnosis of Philadelphia-negative myeloproliferative neoplasms.

BMC Cancer 2019 Feb 28;19(1):184. Epub 2019 Feb 28.

Division of Hematology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkok, 10700, Thailand.

Background: Philadelphia (Ph) chromosome-negative myeloproliferative neoplasms (MPNs) are a heterogeneous group of hematopoietic stem cell clonal diseases. Most patients with MPN are asymptomatic at diagnosis although some of them suffer from constitutional symptoms. Thrombosis and bleeding can also be one of the initial manifestations although the reported prevalence varied considerably across the studies. Read More

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http://dx.doi.org/10.1186/s12885-019-5387-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6393965PMC
February 2019
3 Reads
3.362 Impact Factor

Homozygous CALR Mutation in Primary Myelofibrosis and Its Effect on Disease Phenotype: A Case Report and Review of the Literature.

Case Rep Hematol 2019 20;2019:1430170. Epub 2019 Jan 20.

Department of Clinical Haematology, National Institute of Blood Diseases & Bone Marrow Transplantation, P.O. Box-75300, Karachi, Pakistan.

Somatic mutations in gene have been reported in 60%-88% of patients with essential thrombocythemia (ET) and primary myelofibrosis (PMF) who are negative for and mutations. Most of the mutations analyzed to date are heterozygous mutations in exon 9 of the gene. Homozygosity in gene is rarely reported, and its association with clinical behavior of disease and impact on outcome of patients is not studied so far. Read More

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http://dx.doi.org/10.1155/2019/1430170DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360597PMC
January 2019
3 Reads

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
2 Reads

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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6377687PMC
February 2019
3 Reads

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
4 Reads

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
3 Reads

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
12 Reads

[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
3 Reads

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

J Exp Med 2019 Mar 7;216(3):587-604. 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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6400533PMC
March 2019
7 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
9 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
11 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
8 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
4 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
3 Reads

[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
3 Reads

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
19 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
6 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
2 Reads

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
2 Reads

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
8 Reads

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

Int J Infect Dis 2019 Mar 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
March 2019
16 Reads

Prominence of nestin-expressing Schwann cells in bone marrow of patients with myelodysplastic syndromes with severe fibrosis.

Int J Hematol 2019 Mar 10;109(3):309-318. Epub 2019 Jan 10.

Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Ibaraki, Japan.

Nestin-expressing stromal cells (NESCs) and Schwann cells in the bone marrow (BM) play crucial roles as a niche for normal hematopoietic stem cells in mice. It has been reported that both types of cells are decreased in myeloproliferative neoplasms in patients and also in a mouse model, whereas an increase in NESCs was reported in acute myeloid leukemia. It is thus of interest whether and how these BM stromal cells are structured in myelodysplastic syndromes (MDS). Read More

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http://link.springer.com/10.1007/s12185-018-02576-9
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http://dx.doi.org/10.1007/s12185-018-02576-9DOI Listing
March 2019
14 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
15 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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6425947PMC
January 2019
6 Reads

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
4 Reads

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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6381513PMC
February 2019
7 Reads

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

Blood Cells Mol Dis 2019 03 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
6 Reads

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

Pathology 2019 Apr 31;51(3):301-307. 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
April 2019
5 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
5 Reads

Clinicopathological and molecular features of SF3B1-mutated myeloproliferative neoplasms.

Hum Pathol 2019 Apr 27;86:1-11. 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 are not well defined. We interrogated 101 genes in 143 BCR-ABL1-negative MPNs in chronic phase from 2 large institutions. Read More

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http://dx.doi.org/10.1016/j.humpath.2018.11.022DOI Listing
April 2019
5 Reads

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
4 Reads