49,243 results match your criteria Myeloproliferative Disease


Real-World Outcomes of Ruxolitinib Treatment for Polycythemia Vera.

Clin Lymphoma Myeloma Leuk 2020 May 29. Epub 2020 May 29.

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

Introduction: Ruxolitinib is approved for the treatment of polycythemia vera (PV) with hydroxyurea resistance or intolerance. Approval was based on the phase III RESPONSE trial, which demonstrated efficacy in a highly selected patient population.

Materials And Methods: To characterize the tolerability and outcomes of ruxolitinib outside of a clinical trial, we performed a multi-center retrospective analysis of patients with PV treated with ruxolitinib at 11 participating sites across the United States. Read More

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http://dx.doi.org/10.1016/j.clml.2020.05.019DOI Listing

Blood pressure profile, sympathetic nervous system activity and subclinical target organ damage in patients with polycythemia vera.

Pol Arch Intern Med 2020 Jul 4. Epub 2020 Jul 4.

Introduction: Polycythemia vera (PV) is a rare myeloproliferative disease associated with an increased prevalence of hypertension and increased risk of cardiovascular complications. The precise mechanisms leading to elevation of blood pressure (BP) and secondary target organ damage remains, however, poorly understood.

Objectives: The aims were to: evaluate BP profile, assess sympathetic nervous system and renin-angiotensin system activities and provide a comprehensive assessment of subclinical target organ damage in PV patients. Read More

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http://dx.doi.org/10.20452/pamw.15473DOI Listing

Extra-medullary recurrence of myeloid leukemia as myeloid sarcoma after allogeneic stem cell transplantation: impact of conditioning intensity.

Bone Marrow Transplant 2020 Jun 30. Epub 2020 Jun 30.

Klinik für Innere Medizin II, Hämatologie und Internistische Onkologie, Universitätsklinikum Jena, Jena, Germany.

Myeloid sarcoma (MS) as a solid extra-medullary (EM) manifestation of acute myeloid leukemia (AML), myeloproliferative or myelodysplastic syndromes is a rare presentation of relapse after allogeneic hematopoietic stem cell transplantation (HSCT). The databases of the Departments of Hematology and Oncology of the University Hospitals of Jena and Rostock were screened for patients aged 18 years or older for onset of MS after HSCT for myeloid malignancies between 2002 and 2019. Nineteen patients with MS were identified, the majority of whom had received reduced-intensity conditioning (RIC). Read More

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http://dx.doi.org/10.1038/s41409-020-0984-4DOI Listing

Immunotherapy in Myeloproliferative Diseases.

Cells 2020 Jun 26;9(6). Epub 2020 Jun 26.

Department of Medicine I, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79106 Freiburg, Germany.

Myeloproliferative diseases, including myeloproliferative neoplasms (MPN) and myelodysplastic syndromes (MDS), are driven by genetic abnormalities and increased inflammatory signaling and are at high risk to transform into acute myeloid leukemia (AML). Myeloid-derived suppressor cells were reported to enhance leukemia immune escape by suppressing an effective anti-tumor immune response. MPNs are a potentially immunogenic disease as shown by their response to interferon-α treatment and allogeneic hematopoietic stem-cell transplantation (allo-HSCT). Read More

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http://dx.doi.org/10.3390/cells9061559DOI Listing

[Clinical features and diagnosis of Ph - negative myeloproliferative neoplasms occurring in conjunction with the antiphospholipid syndrome].

Ter Arkh 2019 Jul 15;91(7):93-99. Epub 2019 Jul 15.

National Research Center for Hematology.

Thrombosis is a serious and extremely dangerous disease that has a negative impact on the quality and longevity. Antiphospholipid syndrome (APS) is a pathology characterized by recurring venous, arterial, microvasculature thrombosis, pregnancy pathology with loss of the fetus and the synthesis of antiphospholipid antibodies. A high risk of thrombotic complications is also observed in patients with myeloproliferative neoplasms (MPN). Read More

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http://dx.doi.org/10.26442/00403660.2019.07.000324DOI Listing

[Brain MRI-findings in Ph - negative myeloproliferative disorders].

Ter Arkh 2019 Jul 15;91(7):29-34. Epub 2019 Jul 15.

Research center of Neurology.

Myeloproliferative disorders (MPD) are accompanied by a high proportion of thrombotic complications, which may lead to cerebrovascular disease (CVD).

Aim: To describe MRI-findings in patients with Ph - negative MPD and evaluate any cerebrovascular disease.

Materials And Methods: We included 104 patients with Ph - negative MPD (age varied between 20 and 58) with clinical correlates of cerebrovascular pathology. Read More

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http://dx.doi.org/10.26442/00403660.2019.07.000329DOI Listing

[Somatic mutation of the V617F JAK2 gene in patients of the cardiovascular diseases].

Ter Arkh 2019 Jul 15;91(7):25-28. Epub 2019 Jul 15.

The Federal Center of Cardiovascular Surgery of Krasnoyarsk.

The JAK2 V617F somatic mutation is one of the most frequent markers of CHIP (clonal hematopoiesis of indeterminate potential). CHIP is characterized by the presence of a myeloid cells clone in peripheral blood in the absence of the sufficient reasons to diagnose the hematologic disease. The CHIP is proposed as a potential independent risk factor for vascular pathology. Read More

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http://dx.doi.org/10.26442/00403660.2019.07.000245DOI Listing

Clinical Features and Outcomes of Patients With Chronic Myeloid Leukemia Presenting With Isolated Thrombocytosis: A Systematic Review and a Case From Our Institution.

Cureus 2020 Jun 23;12(6):e8788. Epub 2020 Jun 23.

Hematology and Oncology, Creighton University Arizona Health Education Alliance/Valleywise Health Medical Center, Phoenix, USA.

Chronic myeloid leukemia (CML) represents a common condition in the spectrum of myeloproliferative disorders (MPD). It classically exhibits leukocytosis, but rarely presents with isolated thrombocytosis. This paper is designed to review the clinicopathologic features, treatment, and outcomes of patients with CML who present with isolated thrombocytosis. Read More

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http://dx.doi.org/10.7759/cureus.8788DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7314366PMC

Extreme Levels of Platelet Count in Essential Thrombocythemia: Management and Outcome, Report of Two Cases.

Case Rep Oncol 2020 May-Aug;13(2):606-610. Epub 2020 Jun 4.

National Center for Cancer Care and Research, Department of Oncology, Hematology and BMT Section, Hamad Medical Corporation, Doha, Qatar.

Myeloproliferative neoplasms including essential thrombocythemia (ET) is usually caused by somatic mutations in multiple genes, including the JAK2 (most frequently), CALR gene, and MPL. In rare cases, the disease is caused by other mutations such as THPO or TET2 gene; however, around 10-15% with ET might have triple-negative mutations. Here we present 2 cases of ET who were asymptomatic on diagnoses, but found to have extremely high platelet counts as never reported earlier. Read More

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http://dx.doi.org/10.1159/000507363DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7315211PMC

Management of challenging myelofibrosis after JAK inhibitor failure and/or progression.

Blood Rev 2020 May 30:100716. Epub 2020 May 30.

UT Health San Antonio MD Anderson Cancer Center, University of Texas Health Science Center San Antonio, San Antonio, TX 78249, USA. Electronic address:

The myeloproliferative neoplasms (MPNs) encompass a heterogenous set of diseases that have variable survival, but in the setting of treatment refractory and progressive disease, prognosis has been characteristically poor. JAK inhibition with ruxolitinib or fedratinib therapy has become the first line treatment for symptomatic or intermediate to high risk myelofibrosis. However, after three years of ruxolitinib therapy, approximately half of all patients with myelofibrosis will likely have stopped treatment. Read More

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http://dx.doi.org/10.1016/j.blre.2020.100716DOI Listing

Myeloid diseases in the lung and pleura.

Semin Diagn Pathol 2020 Jun 12. Epub 2020 Jun 12.

Department of Pathology, UT Southwestern Medical Center, BioCenter EB3.234, 2330 Inwood Road, EB3.234, Dallas, TX 75390-9317, USA. Electronic address:

Myeloid diseases detected as primary or secondary lesions in the lung and pleura are rare. Clinical presentations and radiographic results may vary significantly depending on the nature of the diseases. The most common diseases associated with lung and pleura involvement are myeloid sarcoma/acute myeloid leukemia (AML) and extramedullary hematopoiesis (EMH). Read More

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http://dx.doi.org/10.1053/j.semdp.2020.06.002DOI Listing

Top-Down Proteomics of Human Saliva Discloses Significant Variations of the Protein Profile in Patients with Mastocytosis.

J Proteome Res 2020 Jul 6. Epub 2020 Jul 6.

Dipartimento di Scienze della Vita e dell'Ambiente, Università di Cagliari, 09124 Cagliari, Italy.

Mastocytosis is a myeloproliferative neoplasm causing abnormal clonal mast cell accumulation in different tissues, such as skin and bone marrow. A cutaneous subtype (CM) is distinguished from a systemic one (SM); SM patients can be grouped into SM with (SM+C) or without (SM-C) additional cutaneous lesions, and their classification is often challenging. This study was purposed to highlight variations in the salivary proteome of patients with different mastocytosis subtypes and compared to healthy controls. Read More

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http://dx.doi.org/10.1021/acs.jproteome.0c00207DOI Listing

Large Copy-Number Variants in UK Biobank Caused by Clonal Hematopoiesis May Confound Penetrance Estimates.

Am J Hum Genet 2020 Jun 17. Epub 2020 Jun 17.

Institute of Biomedical and Clinical Science, University of Exeter Medical School, RILD Building, Royal Devon & Exeter Hospital, Barrack Road, Exeter EX2 5DW, UK. Electronic address:

Large copy-number variants (CNVs) are strongly associated with both developmental delay and cancer, but the type of disease depends strongly on when and where the mutation occurred, i.e., germline versus somatic. Read More

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http://dx.doi.org/10.1016/j.ajhg.2020.06.001DOI Listing

Genomic heterogeneity in myeloproliferative neoplasms and applications to clinical practice.

Blood Rev 2020 May 19:100708. Epub 2020 May 19.

Wellcome Sanger Institute, Hinxton, Cambridgeshire, UK; Cambridge Stem Cell Institute, Jeffrey Cheah Biomedical Centre, Cambridge Biomedical Campus, Puddicombe Way, Cambridge, UK; Department of Haematology, University of Cambridge, Cambridge, UK; Haematopathology and Oncology Diagnostics Service/ Department of Haematology, Cambridge University Hospitals NHS Foundation Trust, Hills Rd, Cambridge CB2 0QQ, UK. Electronic address:

The myeloproliferative neoplasms (MPN) polycythaemia vera, essential thrombocythaemia and primary myelofibrosis are chronic myeloid disorders associated most often with mutations in JAK2, MPL and CALR, and in some patients with additional acquired genomic lesions. Whilst the molecular mechanisms downstream of these mutations are now clearer, it is apparent that clinical phenotype in MPN is a product of complex interactions, acting between individual mutations, between disease subclones, and between the tumour and background host factors. In this review we first discuss MPN phenotypic driver mutations and the factors that interact with them to influence phenotype. Read More

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http://dx.doi.org/10.1016/j.blre.2020.100708DOI Listing

[Coexistence of mycosis fungoides and essential thrombocythemia with JAK2V617F].

Rev Med Inst Mex Seguro Soc 2019 Sep 2;57(5):329-333. Epub 2019 Sep 2.

Instituto Mexicano del Seguro Social, Hospital General Regional No. 251, Departamento de Hematología. Metepec, Estado de México, México.

Background: The coexistence of myeloproliferative neoplasms (MPNs), specifically essential thrombocythemia and lymphoproliferative neoplasms, are a very rare finding with a frequency < 1%.

Case Report: We present the case of a woman with diagnosis of mycosis fungoides early stage IB, of 5 months of evolution, she received systemic treatment based on methotrexate orally for 4 months; after this, she started with important thrombocythemia reaching up to 1 200 000/mm3 platelets and leukocytosis ranging from 10 000 - 13000/mL. A study protocol for chronic myeloproliferative disease was performed, reporting 90% cellular bone biopsy, erythroid myeloid ratio 5:1, 25 megakaryocytes per mm3, some with hyperlobed nuclei, and giant nuclei. Read More

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September 2019

Outcomes and Clinical Characteristics of Intracranial Hemorrhage in Patients with Hematological Malignancies: A Systematic Literature Review.

World Neurosurg 2020 Jun 18. Epub 2020 Jun 18.

Department of Neurological Surgery, University Hospitals Cleveland Medical Center, Cleveland Ohio.

Background: Many clinical and demographic factors can influence survival of patients with hematological malignancies who have intracranial hemorrhages. Understanding the influence of these factors on patient survival can guide treatment decisions and may inform prognostic discussions. We conducted a systematic literature review to determine survival of patients with intracranial hemorrhages and concomitant hematologic malignancy. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.06.091DOI Listing

Sequential allogeneic hematopoietic stem cell transplantation for active refractory/relapsed myeloid malignancies: results of a reduced-intensity conditioning preceded by clofarabine and cytosine arabinoside, a retrospective study on behalf of the SFGM-TC.

Ann Hematol 2020 Aug 20;99(8):1855-1862. Epub 2020 Jun 20.

Department of Hematology, CHU Hôtel Dieu, Place A. Ricordeau, 44093, Nantes Cedex, France.

Allogeneic stem cell transplantation (allo-SCT) represents the most beneficial treatment for patients with active relapsed/refractory (R/R) hematologic malignancies. Recently, sequential regimens combining debulking chemotherapy followed by reduced-intensity conditioning (RIC) have shown encouraging results for these patients. In this retrospective study, we report the extended results of a sequential regimen of clofarabine, cytosine arabinoside, and RIC in 131 adults with active R/R myeloid disease at transplant. Read More

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http://dx.doi.org/10.1007/s00277-020-04074-7DOI Listing

Lenograstim-Induced Nodal Extramedullary Hematopoiesis: A Challenging Diagnosis in Lymphoma Evaluation With 18F-FDG PET/CT.

Clin Nucl Med 2020 Aug;45(8):e373-e375

From the Department of Nuclear Medicine and Molecular Imaging, ICANS; Department of Pathology, Strasbourg University Hospital, Strasbourg; and Department of Medical Oncology, Clinique St Luc, Schirmeck, France.

We report the case of a 23-year-old man with nodal EMH (extramedullary hematopoiesis) occurring during treatment for a stage IIA "gray-zone" lymphoma. Although it is often related to myeloproliferative bone marrow disease, benign etiologies such as lenograstim treatment after chemotherapy can also induce EMH and be responsible for false-positive F-FDG PET/CT examinations. In this respect, GLUT overexpression in hematopoietic lineages and macrophages of the inflammatory environment are responsible for increased F-FDG uptake. Read More

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http://dx.doi.org/10.1097/RLU.0000000000003111DOI Listing

A novel translocation t(10;17)(p13;q11.2) harboring two cryptic deletions identified by array-CGH and characterized by SUZ12 overexpression in a patient with chronic thrombocytosis.

Genes Chromosomes Cancer 2020 Jun 19. Epub 2020 Jun 19.

School of Biomedical Sciences, The University of Western Australia, Crawley, WA, Australia.

No specific translocation is associated with myeloproliferative neoplasms (MPNs). However, an interstitial deletion involving subband 17q11.2 which includes the NF1 gene, although rare, is a recurrent aberration in several myeloid disorders including MPNs. Read More

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http://dx.doi.org/10.1002/gcc.22881DOI Listing

Anagrelide influences thrombotic risk, and prolongs progression-free and overall survival in essential thrombocythaemia vs hydroxyurea plus aspirin.

Eur J Haematol 2020 Jun 4. Epub 2020 Jun 4.

Department of Haematology, Somogy County Kaposi Mor Teaching Hospital, Kaposvár, Hungary.

Objective: We report an extension study of patients with essential thrombocythaemia (ET) in the Hungarian Myeloproliferative Neoplasm (HUMYPRON) Registry, which demonstrated that over 6 years anagrelide significantly decreased the number of patients experiencing minor arterial and minor venous thrombotic events (TEs) vs hydroxyurea+aspirin.

Methods: Data on patients with ET were collected through completion of a questionnaire developed according to 2008 WHO diagnostic criteria and with regard to Landolfi, Tefferi and IPSET criteria for thrombotic risk. Data were entered into the registry from 14 haematological centres. Read More

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http://dx.doi.org/10.1111/ejh.13459DOI Listing

Idiopathic hypereosinophilic syndrome associated with rapid progression of cardiac, pulmonary and skin infiltration.

Cardiovasc J Afr 2020 Jun 12;31:1-7. Epub 2020 Jun 12.

Department of Cardiology, China-Japan Union Hospital of Jilin University, Cardiovascular Institute of Jilin Province, Changchun 130033, Jilin Province, China.

Idiopathic hypereosinophilic syndrome (IHES) is a rare myeloproliferative disease characterised by multisystem dysfunction and persistent, extreme eosinophilia of unknown cause. Here we present a 42-year-old patient complaining of moderate to severe chest pain and shortness of breath, and typical ischaemic electrocardiography changes were recorded. He was initially suspected of having acute coronary syndrome, however the coronary angiogram excluded coronary abnormalities. Read More

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http://dx.doi.org/10.5830/CVJA-2020-009DOI Listing

The acquired von Willebrand syndrome focused for hematologists.

Haematologica 2020 Jun 18. Epub 2020 Jun 18.

Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy

The acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder with laboratory findings similar to those of inherited von Willebrand disease. However, unlike the inherited disease, the AVWS occurs in persons with no personal or family history of bleeding and is often associated with a variety of underlying diseases, most frequently lymphoproliferative, myeloproliferative and cardiovascular disorders. After the presentation of a typical case, in this narrative review we discuss the more recent data on the pathophysiology, clinical, laboratory and therapeutic aspects of this acquired bleeding syndrome. Read More

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http://dx.doi.org/10.3324/haematol.2020.255117DOI Listing

[Research Advance on Reversing Multidrug Resistance of Chronic Myeloid Leukemia by Chinese Herbal Monomer--Review].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2020 Jun;28(3):1064-1068

Department of Pediatrics, Children's Blood and Tumor Research Laboratory, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province,

Abstract  Chronic myeloid leukemia (CML) is a malignant myeloproliferative tumor which is originating from hematopoietic stem cells. Chemotherapy is the preferred made of treatment for the disease. However, in recent years, more and more patients have multidrug resistance (MDR) during treatment, which is the main cause of failure treatment, therefore, the search for effective reversal agents has important clinical significance. Read More

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http://dx.doi.org/10.19746/j.cnki.issn.1009-2137.2020.03.058DOI Listing

[Application of Single Cell Sequencing in Diagnosis and Treatment of Malignant Hematological Diseases--Review].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2020 Jun;28(3):1059-1063

Department of Hematology, Xiyuan Hospital, China Academy of Traditional Chinese Medical Sciences, Beijing 100091, China,E-mail:

Abstract  Single cell sequencing technology is different from traditional sequencing method, which is based on population cell average level. It has been widely used in many fields and made great progress in the application of malignant hematological diseases. In this review, the principle, methodology and application of single cell sequencing technology in malignant hematological diseases are summarized briefly, including the study of the pathogenesis in myelodysplastic syndrome, the mechanism of transformation into leukemia, accurate diagnosis and classification, differential diagnosis, evaluation of targeted drug therapeutic efficacy and exploration of biomarkers; specific diagnostic indicators for myeloproliferative diseases, progression of disease monitoring and epidemiological studies; moreover, the pathogenesis and drug resistance of acute myeloid leukemia (AML), which can provide reference for the diagnosis and research of malignant hematological diseases. Read More

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http://dx.doi.org/10.19746/j.cnki.issn.1009-2137.2020.03.057DOI Listing

Current management strategies for polycythemia vera and essential thrombocythemia.

Blood Rev 2020 Jun 3:100714. Epub 2020 Jun 3.

CRIMM, Centro di Ricerca e Innovazione per le Malattie Mieloproliferative, Azienda Ospedaliera Universitaria Careggi, Dipartimento di Medicina Sperimentale e Clinica, Università degli Studi, Firenze, DENOTHE Excellence Center, Italy. Electronic address:

Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms characterized by increased rate of cardiovascular events, a varying burden of symptoms, and an intrinsic risk of evolution to secondary forms of myelofibrosis and acute leukemia; however, survival is only modestly reduced in most instances. In the last few years, following the description of driver mutations in JAK2, MPL and CALR, the diagnostic criteria for PV and ET were revised, making the identification of very early stages feasible. Scores for identifying patients at different risk of thrombosis were refined, and they largely guide therapeutic decisions. Read More

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http://dx.doi.org/10.1016/j.blre.2020.100714DOI Listing

Sex determines the presentation and outcomes in MPN and is related to sex-specific differences in the mutational burden.

Blood Adv 2020 Jun;4(12):2567-2576

Division of Adult Hematology, Department of Medicine.

The factors underlying the variable presentation and clinical course of myeloproliferative neoplasms (MPNs) remain unclear. The aim of this study was to evaluate the independent effect of sex on MPN presentation and outcomes. A total of 815 patients with essential thrombocytosis, polycythemia vera, or primary myelofibrosis were evaluated between 2005 and 2019, and the association of sex with presenting phenotype, JAK2 V617F burden, progression, and survival was examined. Read More

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http://dx.doi.org/10.1182/bloodadvances.2019001407DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7322953PMC

Reduced CXCR4-expression on CD34-positive blood cells predicts outcomes of persons with primary myelofibrosis.

Leukemia 2020 Jun 14. Epub 2020 Jun 14.

Center for the Study of Myelofibrosis, Laboratory of Biochemistry, Biotechnology and Advanced Diagnostics, Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S. Matteo Foundation, Pavia, Italy.

The expression of the CXCR4 chemokine receptor on CD34-positive blood cells is reduced in persons with primary myelofibrosis (PMF). We analyzed the relevance of cytofluorimetric assessment of the percentage of CD34-positive blood cells that had a positive CXCR4 surface expression (CD34/CXCR4-se) in a large cohort of subjects with myeloproliferative neoplasms. Mean CD34/CXCR4-se was lower in subjects with PMF compared with those with essential thrombocythemia (ET) or polycythemia vera (PV). Read More

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http://dx.doi.org/10.1038/s41375-020-0926-6DOI Listing

Current and future therapies for myelofibrosis.

Blood Rev 2020 May 30:100715. Epub 2020 May 30.

Department of Haematology, Guy's and St Thomas' NHS Foundation Trust, London SE1 9RT, UK. Electronic address:

Myelofibrosis is classified as a 'Philadelphia-chromosome negative' clonal myeloproliferative disorder. The heterogeneity of this condition and patient population and array of often challenging clinical manifestations can frequently make therapeutic decisions challenging. Despite many advances in therapy with targeted and combination approaches, following an enhanced understanding of underlying disease pathogenesis, cure only remains achievable with allogeneic stem cell transplant. Read More

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http://dx.doi.org/10.1016/j.blre.2020.100715DOI Listing

MECOM rearrangement involving the MYC locus: Two additional patients with the rare translocation, t(3;8)(q26.2;q24), and molecular review.

Leuk Res 2020 May 24;95:106387. Epub 2020 May 24.

University of Nebraska Medical Center, United States; Human Genetics Laboratory, Munroe-Meyer Institute for Genetics and Rehabilitation, Omaha, NE, United States. Electronic address:

A relatively small subset of myeloid neoplasms involve rearrangements of cytoband 3q26.2. Such rearrangements are often in response to therapy and carry a poor prognosis. Read More

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http://dx.doi.org/10.1016/j.leukres.2020.106387DOI Listing

Prevalence and causes of elevated bone mass.

Bone 2020 Jun 11;138:115476. Epub 2020 Jun 11.

Univ. Lille, CHU Lille, MABLab ULR 4490, Department of Rheumatology, 59000 Lille, France. Electronic address:

Introduction: Reports of elevated bone mass (EBM) on routine DXA scanning are not infrequent. However, epidemiological studies of EBM are few in number and definition thresholds variable. The purpose of this study was to assess the prevalence and causes of EBM in the general population referred to a single university hospital - catering for a population of 4 million inhabitants - for DXA scanning. Read More

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http://dx.doi.org/10.1016/j.bone.2020.115476DOI Listing

Germline genetic factors in the pathogenesis of myeloproliferative neoplasms.

Blood Rev 2020 May 29:100710. Epub 2020 May 29.

INSERM, UMR1287, Laboratory of Excellence GR-Ex, Villejuif, France; Gustave Roussy, Villejuif, France; Université Paris XI, UMR1287, Gustave Roussy, Villejuif, France. Electronic address:

Myeloproliferative neoplasms (MPN) are clonal hematological malignancies that lead to overproduction of mature myeloid cells. They are due to acquired mutations in genes encoding for AK2, MPL and CALR that result in the activation of the cytokine receptor/JAK2 signaling pathway. In addition, it exists germline variants that can favor the initiation of the disease or may affect its phenotype. Read More

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http://dx.doi.org/10.1016/j.blre.2020.100710DOI Listing

Prognostic models in the myeloproliferative neoplasms.

Authors:
Jacob Grinfeld

Blood Rev 2020 May 30:100713. Epub 2020 May 30.

Department of Paediatric Haematology, Leeds General Infirmary, Great George St, Leeds LS1 3EX, UK. Electronic address:

The management of myelofibrosis (MF) is predominantly supportive, with the use of JAK2 inhibitors or allogeneic stem cell transplantation reserved for patients predicted to have poor overall survival. Identification of these patients is aided by a number of prognostic scoring systems, foremost among them the Dynamic International Prognostic Scoring System (DIPSS). Similarly, the use of cytoreductive therapies in essential thrombocytosis (ET) and polycythemia vera (PV) is targeted to patients identified as at highest risk of thrombosis. Read More

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http://dx.doi.org/10.1016/j.blre.2020.100713DOI Listing

Successful Anti-CLL1 CAR T-Cell Therapy in Secondary Acute Myeloid Leukemia.

Front Oncol 2020 27;10:685. Epub 2020 May 27.

Shenzhen Geno-Immune Medical Institute, Shenzhen, China.

Secondary acute myeloid leukemia (sAML) is a high-risk AML evolving from heterogenous prior hematological disorders. Compared to de novo AML, sAML has even worse responses to current therapy and thus is associated with lower remission rates, inferior overall survival (OS) and higher relapse rates. Many efforts have been devoted to improving the overall but with limited success, and novel strategy is thus highly needed. Read More

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http://dx.doi.org/10.3389/fonc.2020.00685DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7266936PMC

Excessive Leukocytosis Leading to a Diagnosis of Aggressive Thyroid Anaplastic Carcinoma: A Case Report and Relevant Review.

Eur Thyroid J 2020 May 9;9(3):162-168. Epub 2020 Apr 9.

First Department of Pathology/University of Athens, Medical School, Athens, Greece.

Introduction: Leukocytosis and particularly neutrophilia are usually caused by acute infection, inflammation, and myeloproliferative neoplasms. However, leukocytosis can also occur in patients with malignancy either due to bone marrow metastases or in the context of a paraneoplastic syndrome.

Case Presentation: An 86-year-old female was admitted to our hospital due to marked leukocytosis (white blood cells [WBC] >40,000/μL), neutrophilia, and monocytosis. Read More

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http://dx.doi.org/10.1159/000506767DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7265710PMC

Epidemiology of the classical myeloproliferative neoplasms: The four corners of an expansive and complex map.

Blood Rev 2020 May 22:100706. Epub 2020 May 22.

Section of Hematology, Department of Internal Medicine, Yale University School of Medicine, New Haven, USA; Cancer Outcomes, Public Policy, and Effectiveness Research (COPPER) Center, Yale University, New Haven, USA. Electronic address:

The classical myeloproliferative neoplasms (MPNs), specifically chronic myeloid leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF), represent clonal myeloid disorders whose pathogenesis is driven by well-defined molecular abnormalities. In this comprehensive review, we summarize the epidemiological literature and present our own analysis of the most recent the Surveillance, Epidemiology, and End Results (SEER) program data through 2016. Older age and male gender are known risk factors for MPNs, but the potential etiological role of other variables is less established. Read More

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http://dx.doi.org/10.1016/j.blre.2020.100706DOI Listing

[Myeloproliferative neoplasms and chronic inflammatory bowel disease].

Ugeskr Laeger 2020 May;182(22)

Studies have suggested a possible association between inflammatory bowel disease (IBD) and the Philadelphia-negative chronic myeloproliferative neoplasms (MPNs). The mechanisms behind this association have not been investigated yet, but in this review, we find it most likely to involve complex interactions between genetic, treatment-related and inflammation- and immune-mediated factors. When patients with IBD present with persistent leukocytosis and/or thrombocytosis, it may reflect concomitant MPN, and early detection and treatment of MPNs may prevent some of the complications related to these diseases. Read More

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[Acquired von Willebrand syndrome: a hemostatic disorder frequently encountered in the fields of cardiology and gastroenterology].

Rinsho Ketsueki 2020 ;61(5):542-548

Department of Molecular and Cellular Biology, Institute of Development, Aging and Cancer, Tohoku University.

von Willebrand disease is a genetic hemostatic disorder that is caused by the qualitative or quantitative dysfunction of the von Willebrand factor (VWF), which is involved in hemostasis. A similar dysfunction sometimes develops without mutations, known as acquired von Willebrand syndrome (AVWS) , which has been associated with lymphoproliferative diseases, myeloproliferative diseases, malignant tumors, and hypothyroidism. Recently, it was remarkably noted that cardiovascular diseases with high intravascular shear stress could cause AVWS . Read More

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http://dx.doi.org/10.11406/rinketsu.61.542DOI Listing
January 2020

Role of inflammation in the biology of myeloproliferative neoplasms.

Blood Rev 2020 May 30:100711. Epub 2020 May 30.

Department of Hematology, Oncology, Hemostaselogy, and Stem Cell Transplantation, Faculty of Medicine, RWTH Aachen University, Aachen, Germany. Electronic address:

What is the role of inflammation in Myeloproliferative Neoplasms? This is currently a topic of much debate. In this review, we will discuss experimental results and basic concepts of inflammatory processes in the pathogenesis of myeloproliferative neoplasms (MPN). So, which are the players involved? First, these are the clonal hematopoietic stem cells (HSC) and their normal stem cell counterparts in the bone marrow (BM), as well as their more mature progeny in the BM and the peripheral blood (PB), including neutrophils, macrophages, erythrocytes, and platelets, but also other cell lineages. Read More

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http://dx.doi.org/10.1016/j.blre.2020.100711DOI Listing

Driver Mutations in Leukemia Promote Disease Pathogenesis through a Combination of Cell-Autonomous and Niche Modulation.

Stem Cell Reports 2020 May 22. Epub 2020 May 22.

Department of Pediatrics, Herman B. Wells Center for Pediatric Research, Indiana University School of Medicine, R4, 1044 West Walnut Street, Indianapolis, IN 46202, USA; Department of Microbiology and Immunology, Indiana University School of Medicine, Indianapolis, IN 46202, USA; Department of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, IN 46202, USA; Department of Molecular Biology and Biochemistry, Indiana University School of Medicine, Indianapolis, IN 46202, USA. Electronic address:

Studies of patients with acute myeloid leukemia (AML) have led to the identification of mutations that affect different cellular pathways. Some of these have been classified as preleukemic, and a stepwise evolution program whereby cells acquire additional mutations has been proposed in the development of AML. How the timing of acquisition of these mutations and their impact on transformation and the bone marrow (BM) microenvironment occurs has only recently begun to be investigated. Read More

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http://dx.doi.org/10.1016/j.stemcr.2020.05.002DOI Listing

Enhanced engraftment of human myelofibrosis stem and progenitor cells in MISTRG mice.

Blood Adv 2020 Jun;4(11):2477-2488

Department of Medical Oncology and Hematology, University of Zurich and University Hospital Zurich, Zurich, Switzerland.

The engraftment potential of myeloproliferative neoplasms in immunodeficient mice is low. We hypothesized that the physiological expression of human cytokines (macrophage colony-stimulating factor, interleukin-3, granulocyte-macrophage colony-stimulating factor, and thrombopoietin) combined with human signal regulatory protein α expression in Rag2-/-Il2rγ-/- (MISTRG) mice might provide a supportive microenvironment for the development and maintenance of hematopoietic stem and progenitor cells (HSPC) from patients with primary, post-polycythemia or post-essential thrombocythemia myelofibrosis (MF). We show that MISTRG mice, in contrast to standard immunodeficient NOD. Read More

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http://dx.doi.org/10.1182/bloodadvances.2019001364DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7284099PMC

Chronic Myeloid Leukemia Prognosis and Therapy: Criticisms and Perspectives.

J Clin Med 2020 Jun 2;9(6). Epub 2020 Jun 2.

Institute of Hematology "Lorenzo e Ariosto Seràgnoli", University of Bologna, 40138 Bologna, Italy.

Ph+ chronic myeloid leukemia (CML) is a clonal myeloproliferative disease whose clinical course is characterized by progression disease from the early chronic phase (CP) to the fatal blastic phase (BP). This programmed course is closely related to the translocation t(9;22)(q22;q11) and the resulting BCR-ABL1 fusion protein (p210) that drives the leukemic transformation of hematopoietic stem cells. Therefore, the cure of CML can only pass through the abrogation of the Ph+ clone. Read More

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http://dx.doi.org/10.3390/jcm9061709DOI Listing

[Expanding the possibilities of antithrombotic therapy of cerebrovascular pathology].

Zh Nevrol Psikhiatr Im S S Korsakova 2020 ;120(4):123-128

Research Center of Neurology, Moscow, Russia.

The paper provides an overview of the current state of the problem of antithrombotic therapy in angioneurology. Given the importance and prevalence of cerebral atherosclerosis as one of the leading etiological factors of acute and chronic cerebrovascular disorders (CCD), the paper focuses mainly on antiplatelet therapy. The most recent recommendations for the use of acetylsalicylic acid and clopidogrel, the main antiplatelet drugs, are highlighted. Read More

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http://dx.doi.org/10.17116/jnevro2020120041123DOI Listing
January 2020

Extracranial carotid artery aneurysm with myeloproliferative neoplastic cell invasion.

J Vasc Surg Cases Innov Tech 2020 Jun 28;6(2):243-246. Epub 2020 May 28.

Department of Neurosurgery, Toranomon Hospital, Tokyo, Japan.

The major causes of rare extracranial carotid artery aneurysms are arteriosclerosis, trauma, and radiation therapy. Here, we describe a patient with an extracranial carotid artery aneurysm caused by a myeloproliferative neoplasm. A 67-year-old woman underwent excision of an irregularly shaped aneurysm in the left common carotid artery and a saphenous vein graft without major complications. Read More

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http://dx.doi.org/10.1016/j.jvscit.2020.03.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7261954PMC

Splenectomy in Myelofibrosis: Indications, Efficacy, and Complications.

Clin Lymphoma Myeloma Leuk 2020 Apr 30. Epub 2020 Apr 30.

Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy.

Splenomegaly, which may range from a few centimeters below the left costal border to massive dimensions, is one of the most characteristic features in patients with advanced myelofibrosis (MF). Splenectomy may offer an effective therapeutic option for treating massive splenomegaly in patients with MF, and especially in cases of disease refractory to conventional drugs, but it is associated with a number of complications as well as substantial morbidity and mortality. Whether splenectomy should be performed before allogeneic hematopoietic stem-cell transplantation is also controversial, and there is a lack of prospective randomized clinical trials that assess the role of splenectomy before hematopoietic stem-cell transplantation in patients with MF. Read More

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http://dx.doi.org/10.1016/j.clml.2020.04.015DOI Listing

Transient Abnormal Myelopoeisis and Mosaic Down Syndrome in a Phenotypically Normal Newborn.

Children (Basel) 2020 May 28;7(6). Epub 2020 May 28.

Department of Hematology-Oncology, Texas Children's Hospital, Houston, TX 77030, USA.

Transient abnormal myelopoiesis (TAM) is a common and potentially fatal neonatal complication of newborn babies with Down syndrome (DS). Children born with mosaic DS are also at risk of developing TAM. However, due to their variable phenotypes, early identification of patients with mosaic DS may be difficult; thus, early diagnosis of TAM is just as challenging. Read More

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http://dx.doi.org/10.3390/children7060052DOI Listing

Thrombopoietin is required for full phenotype expression in a JAK2V617F transgenic mouse model of polycythemia vera.

PLoS One 2020 1;15(6):e0232801. Epub 2020 Jun 1.

Department of Pathology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, United States of America.

The myeloproliferative neoplasms, polycythemia vera, essential thrombocytosis and primary myelofibrosis are hematopoietic stem cell disorders and share driver mutations that either directly activate the thrombopoietin receptor, MPL, or activate it indirectly through gain-of-function mutations in the gene for JAK2, its cognate tyrosine kinase. Paradoxically, MPL surface expression in hematopoietic stem cells is also reduced in the myeloproliferative neoplasms due to abnormal post-translational glycosylation and premature destruction of JAK2, suggesting that the myeloproliferative neoplasms are disorders of MPL processing since MPL is the only hematopoietic growth factor receptor in hematopoietic stem cells. To examine this possibility, we genetically manipulated MPL expression and maturation in a JAK2V617F transgenic mouse model of polycythemia vera. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0232801PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7263591PMC

Inclusion of molecular monitoring (BCR-ABL1) in the treatment of chronic myeloid leukemia in the Brazilian Public Health System (SUS): an urgent need for treatment management.

Hematol Transfus Cell Ther 2020 May 25. Epub 2020 May 25.

Departamento de Hematologia, Hospital das Clínicas, Universidade de São Paulo, São Paulo, SP, Brazil. Electronic address:

Introduction: Chronic Myeloid Leukemia (CML) is a myeloproliferative disease that affects mainly adults between 50 and 55 years. In Brazil, information from the Sistema Único de Saúde (SUS) Outpatient Information System indicates that 12,531 patients had the Autorização de Procedimento Ambulatorial (APAC) approved for the CML treatment in 2017. Disease monitoring through molecular response evaluation is critical to the care of CML patients. Read More

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http://dx.doi.org/10.1016/j.htct.2020.02.002DOI Listing