47,388 results match your criteria Myeloproliferative Disease


Myeloid malignancies with isolated 7q deletion can be further characterized by their accompanying molecular mutations.

Genes Chromosomes Cancer 2019 Apr 17. Epub 2019 Apr 17.

MLL Munich Leukemia Laboratory, Max-Lebsche-Platz 31, 81377 Munich, Germany.

Deletions in the long arm of chromosome 7 (del(7q)) are recurrent cytogenetic aberrations in myeloid neoplasms. They occur either isolated or as part of a complex karyotype and are associated with unfavorable prognosis in certain disease entities. We performed detailed cytogenetic analysis, molecular analysis and array comparative genomic hybridization (aCGH) in a cohort of 81 patients with a variety of myeloid malignancies and del(7q) as sole chromosomal alteration. Read More

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http://dx.doi.org/10.1002/gcc.22761DOI Listing

SOHO State of the Art Updates and Next Questions: Myelofibrosis.

Clin Lymphoma Myeloma Leuk 2019 Mar 22. Epub 2019 Mar 22.

University of Michigan Rogel Cancer Center, Ann Arbor, MI.

The discovery of a mutation in the Janus Kinase 2 gene in 2005 spurred significant progress in the field of myeloproliferative neoplasms. A comprehensive description of genomic factors at play in the malignant clone in myeloproliferative neoplasms, particularly myelofibrosis (MF), have recently led to more precise, personalized prognostic tools. Despite this, understanding of the disease pathogenesis remains relatively limited. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S21522650193017
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http://dx.doi.org/10.1016/j.clml.2019.03.011DOI Listing
March 2019
1 Read

Relevant updates in systemic mastocytosis.

Leuk Res 2019 Apr 4;81:10-18. Epub 2019 Apr 4.

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

Systemic Mastocytosis (SM) is a rare myeloproliferative neoplasm (MPN) that is characterized by a clonal proliferation of mast cells (MCs). The symptoms and clinical presentation of SM are the result of both MC proliferation as well as activation and degranulation, causing hyperactive and over-exaggerated hypersensitivity responses, as well as organ infiltration by pathogenic MCs. The clinical presentation and course of SM is varied and organ involvement can lead to significant morbidity and mortality in some cases. Read More

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http://dx.doi.org/10.1016/j.leukres.2019.04.001DOI 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

Addiction to DUSP1 protects JAK2V617F-driven polycythemia vera progenitors against inflammatory stress and DNA damage, allowing chronic proliferation.

Oncogene 2019 Apr 9. Epub 2019 Apr 9.

Department of Biology, Faculty of Medicine and Dentistry, Palacky University, Olomouc, Czech Republic.

Inflammatory and oncogenic signaling converge in disease evolution of BCR-ABL-negative myeloproliferative neoplasms, clonal hematopoietic stem cell disorders characterized by gain-of-function mutation in JAK2 kinase (JAK2V617F), with highest prevalence in patients with polycythemia vera (PV). Despite the high risk, DNA-damaging inflammatory microenvironment, PV progenitors tend to preserve their genomic stability over decades until their progression to post-PV myelofibrosis/acute myeloid leukemia. Using induced pluripotent stem cells-derived CD34 progenitor-enriched cultures from JAK2V617F PV patient and from JAK2 wild-type healthy control, CRISPR-modified HEL cells and patients' bone marrow sections from different disease stages, we demonstrate that JAK2V617F induces an intrinsic IFNγ- and NF-κB-associated inflammatory program, while suppressing inflammation-evoked DNA damage both in vitro and in vivo. Read More

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http://www.nature.com/articles/s41388-019-0813-7
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http://dx.doi.org/10.1038/s41388-019-0813-7DOI Listing
April 2019
3 Reads

Colony-Forming Cell Assay Detecting the Co-Expression of JAK2V617F and BCR-ABL1 in the Same Clone: A Case Report.

Acta Haematol 2019 Apr 9;141(4):261-267. Epub 2019 Apr 9.

Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy.

BCR-ABL1-negative myeloproliferative disorders and chronic myeloid leukaemia are haematologic malignancies characterised by single and mutually exclusive genetic alterations. Nevertheless, several patients co-expressing the JAK2V617F mutation and the BCR-ABL1 transcript have been described in the literature. We report the case of a 61-year-old male who presented with an essential thrombocythaemia phenotype and had a subsequent diagnosis of chronic phase chronic myeloid leukaemia. Read More

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http://dx.doi.org/10.1159/000496821DOI Listing
April 2019
1 Read

Suboptimal Response Rates to Hypomethylating Agent Therapy in Chronic Myelomonocytic Leukemia; a Single Institutional Study of 121 Patients.

Am J Hematol 2019 Apr 9. Epub 2019 Apr 9.

Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN.

Hypomethylating agents (HMA) are currently the only FDA approved therapy for patients with chronic myelomonocytic leukemia (CMML). In the current retrospective study, we assessed response rates as adjudicated by the IWG (International Working Group) MDS (myelodysplastic syndrome) and MDS/myeloproliferative neoplasm (MPN) overlap syndrome response criteria, in 121 CMML patients treated with Azacitidine (AZA, n=56) and Decitabine (DAC, n=65). The overall response rates were 41% by the IWG MDS (AZA- 45%, DAC-39%) and 56% by the IWG MDS/MPN (AZA-56%, DAC-58%) response criteria, with CR (complete remission) rates of <20% for both agents, by both criteria; without significant differences in response rates between proliferative and dysplastic CMML. Read More

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http://dx.doi.org/10.1002/ajh.25488DOI Listing
April 2019
1 Read

Rigosertib ameliorates the effects of oncogenic KRAS signaling in a murine model of myeloproliferative neoplasia.

Oncotarget 2019 Mar 8;10(20):1932-1942. Epub 2019 Mar 8.

Department of Oncological Sciences, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA.

Aberrant signaling triggered by oncogenic or hyperactive RAS proteins contributes to the malignant phenotypes in a significant percentage of myeloid malignancies. Of these, juvenile myelomonocytic leukemia (JMML), an aggressive childhood cancer, is largely driven by mutations in genes and those that encode regulators of these proteins. The mouse model mirrors several key features of this disease and has been used extensively to determine the utility and mechanism of small molecule therapeutics in the context of RAS-driven myeloproliferative disorders. Read More

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http://dx.doi.org/10.18632/oncotarget.26735DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6443005PMC
March 2019
1 Read

Asrij/OCIAD1 suppresses CSN5-mediated p53 degradation and maintains mouse hematopoietic stem cell quiescence.

Blood 2019 Apr 5. Epub 2019 Apr 5.

Molecular Biology and Genetics Unit, Jawaharlal Nehru Centre for Advanced Scientific Research, India

Inactivation of the tumor suppressor p53 is essential for unrestrained growth of cancers. However, only 11% of hematological malignancies have mutant p53. Mechanisms that cause wild type p53 dysfunction and promote leukemia are inadequately deciphered. Read More

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http://dx.doi.org/10.1182/blood.2019000530DOI Listing
April 2019
5 Reads

Oncogenic D816V-KIT signaling in mast cells causes persistent IL-6 production.

Haematologica 2019 Apr 4. Epub 2019 Apr 4.

Mast Cell Biology Section, Laboratory of Allergic Diseases, NIAID, NIH;

Persistent dysregulation of IL-6 production and signaling have been implicated in the pathology of various cancers. In systemic mastocytosis, increased serum levels of IL-6 associate with disease severity and progression, although the mechanisms involved are not well understood. Since systemic mastocytosis often associates with the presence in hematopoietic cells of a somatic gain-of-function variant in KIT, D816V-KIT, we examined its potential role in IL-6 upregulation. 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.2018.212126DOI Listing
April 2019
5 Reads

Distinguishing essential thrombocythemia JAK2V617F from polycythemia vera: limitations of erythrocyte values.

Haematologica 2019 Apr 4. Epub 2019 Apr 4.

Weill Cornell Medicine.

Distinguishing essential thrombocythemia JAK2V617F from polycythemia vera is difficult because of shared mutation and phenotypic characteristics. The World Health Organization suggested hemoglobin and hematocrit values to diagnose polycythemia vera, but their sensitivity and specificity were not tested. Moreover, red cell values do not accurately predict red cell mass, which we use to discriminate essential thrombocythemia JAK2V617F from polycythemia vera. Read More

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http://dx.doi.org/10.3324/haematol.2018.213108DOI Listing
April 2019
1 Read

Cytokine regulated phosphorylation and activation of TET2 by JAK2 in hematopoiesis.

Cancer Discov 2019 Apr 3. Epub 2019 Apr 3.

Medicine, MC2115, University of Chicago

Even though the TET enzymes catalyze the generation of 5-hydroxymethyl-cytosines required for lineage commitment and subsequent differentiation of stem cells into erythroid cells, the mechanisms that link extra-cellular signals to TET activation and DNA hydroxymethylation are unknown. We demonstrate that hematopoietic cytokines phosphorylate TET2 leading to its activation in erythroid progenitors. Specifically, cytokine receptor associated, JAK2, phosphorylates TET2 at tyrosines 1939 and 1964. Read More

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http://cancerdiscovery.aacrjournals.org/lookup/doi/10.1158/2
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http://dx.doi.org/10.1158/2159-8290.CD-18-1138DOI Listing
April 2019
10 Reads

[Prognosis of clonal chromosomal abnormalities in Philadelphia negative metaphases cells in chronic myeloid leukemia with tyrosine kinase inhibitor therapy].

Zhonghua Xue Ye Xue Za Zhi 2019 Mar;40(3):209-214

Department of Hematology, Henan Cancer Hospital, the Affiliated Cancer Hospital of Zhengzhou University, Zhengzhou 450008, China.

To investigate the characteristics and prognosis of clonal chromosomal abnormalities appearing in Philadelphia negative metaphases (CCA/Ph(-)) cells in chronic myeloid leukemia (CML) with tyrosine kinase inhibitor (TKI) therapy. The clinical data of 30 cases with CCA/Ph(-) during TKI treatment in Henan Cancer Hospital from August 2007 to July 2017 were retrospectively analyzed. The univariate factor was analyzed by Kaplan-Meier method. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2019.03.009DOI Listing
March 2019
1 Read

[Clinical analysis of myeloid neoplasms with t (3;21) (q26;q22)].

Zhonghua Xue Ye Xue Za Zhi 2019 Mar;40(3):195-199

Peking University People's Hospital, Peking University Institute of Hematology, Beijing Key Laboratory of Hematopoietic Stem Cell Transplantation, Beijing 100044, China.

To analyze the characteristics of myeloid neoplasms with t (3;21) (q26;q22) . Clinical data of patients with t (3; 21) (q26; q22) , diagnosed as hematologic malignancies in Peking University people's hospital from January 2011 to March 2018, were collected retrospectively. 19 patients in our hospital and forty-eight patients bearing t (3;21) (q26;q22) with detailed survival data reported in literature were summarized. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2019.03.006DOI Listing
March 2019
2 Reads

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

Contemporary management of essential thrombocythemia in children.

Expert Rev Hematol 2019 Mar 29. Epub 2019 Mar 29.

b Department of Women's and Children's Health, Pediatric Hemato-Oncology , University of Padova , Padova , Italy.

Introduction: Essential thrombocythemia (ET) is a disease which is extremely rare in children. Only recently, data on pediatric ET have become available Areas covered: In children with sustained platelet count over 450 x 10/L, secondary thrombocytosis must be ruled out. ET workup comprehends research of JAK2V617F, CALR and MPL mutations and bone marrow biopsy (BM). Read More

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https://www.tandfonline.com/doi/full/10.1080/17474086.2019.1
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http://dx.doi.org/10.1080/17474086.2019.1602034DOI Listing
March 2019
17 Reads

Serum α-l-fucosidase activities are significantly increased in patients with preeclampsia.

Prog Mol Biol Transl Sci 2019 8;162:349-362. Epub 2019 Mar 8.

Systems Biology and Medicine Center for Complex Diseases, Affiliated Hospital of Qingdao University, Qingdao, China. Electronic address:

Fucosylated glycans are essential molecules that facilitate signal transductions in several signal pathways and play important roles in development, inflammation, infection, and tumor metastasis. The fucosylated glycans are difficult to be developed into clinical biomarkers due to their complicated structures, but the decreased or increased activities of serum α-l-fucosidase, the lysosomal enzyme required for fucosylated glycan degradation, are diagnostic biomarker for patients with fucosidosis and hepatocellular carcinoma, respectively. However, the relationship between serum α-l-fucosidase activities and other human diseases is largely unknown. Read More

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http://dx.doi.org/10.1016/bs.pmbts.2018.12.008DOI Listing
March 2019
2 Reads

Calreticulin mutants as oncogenic rogue chaperones for TpoR and traffic-defective pathogenic TpoR mutants.

Blood 2019 Mar 22. Epub 2019 Mar 22.

Ludwig Institute for Cancer Research Brussels, Brussels, Belgium;

+1 frameshift mutations in exon 9 are prevalent in myeloproliferative neoplasms. Mutant CALRs possess a new C-terminal sequence rich in positively charged amino acids, leading to activation of the thrombopoietin receptor (TpoR/MPL). We show that the new sequence endows the mutant CALR with rogue chaperone activity, stabilizing a dimeric state and transporting TpoR and mutants thereof to the cell-surface in states that would not pass quality control, and this function is absolutely required for oncogenic transformation. Read More

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http://dx.doi.org/10.1182/blood-2018-09-874578DOI Listing
March 2019
1 Read

mutations define a specific subgroup of MDS and MDS/MPN patients with favorable outcomes with intensive chemotherapy.

Blood Adv 2019 Mar;3(6):922-933

Department of Leukemia and.

Nucleophosmin () mutations are common in acute myeloid leukemia and are associated with high remission rates and prolonged survival with intensive chemotherapy. mutations are rare in myelodysplastic syndromes (MDS) or myelodysplastic/myeloproliferative neoplasm (MDS/MPN), and the clinical outcomes of these patients, when treated with intensive chemotherapy, are unknown. We retrospectively evaluated the clinicopathologic characteristics and the impact of therapy in 31 patients with MDS or MDS/MPN and mutations. Read More

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http://dx.doi.org/10.1182/bloodadvances.2018026989DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6436014PMC
March 2019
1 Read

Granulocytic sarcoma: An uncommon cause of systemic inflammatory response syndrome.

Clin Case Rep 2019 Mar 31;7(3):469-473. Epub 2019 Jan 31.

Derriford Hospital Plymouth UK.

Granulocytic sarcoma rarely arises from adrenal glands. Its necrosis can lead to systemic inflammatory response syndrome (SIRS), causing clinical difficulty in diagnosis without imaging and both biochemical and histological analysis. Compressive effects of the tumor may mask its source, and therefore, prompt resuscitation, symptom control, and investigation are vital in preventing clinical deterioration. Read More

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http://doi.wiley.com/10.1002/ccr3.1779
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http://dx.doi.org/10.1002/ccr3.1779DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6406164PMC
March 2019
5 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

Genetic landscape of RASopathies in Chinese: Three decades' experience in Hong Kong.

Am J Med Genet C Semin Med Genet 2019 Mar 21. Epub 2019 Mar 21.

Department of Health, Clinical Genetic Service, HKSAR, Hong Kong.

RASopathies are a group of genetic disorders due to dysregulation of the RAS-MAPK signaling pathway, which is important in regulating cell growth, proliferation, and differentiation. These include Noonan syndrome (NS), Noonan syndrome with multiple lentigines (NSML), cardiofaciocutaneous (CFC) syndrome, and Costello syndrome (CS), clinical manifestations include growth retardation, developmental delay, cardiac defects, and specific dysmorphic features. There were abundant publications describing the genotype and phenotype from the Western populations. Read More

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http://dx.doi.org/10.1002/ajmg.c.31692DOI Listing
March 2019
1 Read

Evaluation of platelet function in essential thrombocythemia under different analytical conditions.

Platelets 2019 Mar 20:1-8. Epub 2019 Mar 20.

a Medicina II, ASST Santi Paolo e Carlo - Dipartimento di Scienze della Salute , Università degli Studi di Milano , Milano Italy.

Background: Studies of platelet aggregation (PA) in essential thrombocythemia (ET) reported contrasting results, likely due to differences in analytical conditions.

Objective: We investigated platelet aggregation using different techniques and analytical conditions.

Patients And Methods: PA was studied by light-transmission aggregometry (LTA) in platelet-rich plasma (PRP) and impedance aggregometry in PRP and whole blood (WB). Read More

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http://dx.doi.org/10.1080/09537104.2019.1584668DOI Listing
March 2019
1 Read

GATA-1 a potential novel biomarker for the differentiation of essential thrombocythaemia and myelofibrosis.

J Thromb Haemost 2019 Mar 19. Epub 2019 Mar 19.

School of Life Sciences, University of Lincoln.

Background: The BCR-ABL negative myeloproliferative neoplasms, polycythaemia vera, essential thrombocythaemia (ET) and myelofibrosis (MF) are characterised by mutations in JAK2, CALR or MPL. However, a yet unknown factor drives the precise disease phenotype. The haematopoietic transcription factor GATA-1 and its downstream targets NFE2 and FLI1 are responsible for determining erythroid and megakaryocyte lineages during haematopoietic stem cell differentiation. Read More

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http://dx.doi.org/10.1111/jth.14433DOI Listing
March 2019
6 Reads

Chronic myelogenous leukemia, a still unsolved problem: pitfalls and new therapeutic possibilities.

Drug Des Devel Ther 2019 8;13:825-843. Epub 2019 Mar 8.

Department of Hematology, Military Institute of Medicine, 04-141 Warsaw, Poland,

Chronic myeloid leukemia (CML) is a clonal myeloproliferative disorder of hematopoietic stem cells. At the molecular level, the disorder results from t(9;22)(q34;q11) reciprocal translocation between chromosomes, which leads to the formation of an oncogenic gene fusion. Instead of progress in the understanding of the molecular etiology of CML and the development of novel therapeutic strategies, clinicians still face many challenges in the effective treatment of patients. Read More

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http://dx.doi.org/10.2147/DDDT.S191303DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6415732PMC
March 2019
1 Read

Outcomes of unplanned tyrosine kinase inhibitor discontinuation in patients with chronic myeloid leukemia: retrospective analysis of real-world experience in a single institution.

Hematology 2019 Dec;24(1):355-361

a Department of Hematology and Hematopoietic Stem Cell Transplantation , Yamanashi Prefectural Central Hospital , Kofu , Japan.

Objectives: To explore real-world prognoses for tyrosine kinase inhibitor (TKI) discontinuation in chronic myeloid leukemia (CML) patients and the associated reasons for TKI discontinuation.

Methods: We investigated, using the medical records of 85 consecutive CML patients who received TKIs between December 2001 and August 2016 at our hospital, reasons for discontinuation, duration of TKI treatment before discontinuation, molecular response (MR) status at TKI discontinuation, treatment-free remission (TFR) duration, and overall survival after TKI discontinuation.

Results: TKI therapy was discontinued in 21 patients. Read More

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http://dx.doi.org/10.1080/16078454.2019.1590964DOI Listing
December 2019
1 Read

Thiotepa, Busulfan, and Fludarabine Conditioning Regimen in T Cell-Replete HLA-Haploidentical Hematopoietic Stem Cell Transplantation.

Biol Blood Marrow Transplant 2019 Mar 11. Epub 2019 Mar 11.

Department of Hematology and Cellular Therapy, Saint Antoine Hospital, AP-HP, Paris, France; INSERM, UMR 938, Paris, France; Sorbonne Universités, Université Pierre et Marie Curie Paris 6, Paris, France. Electronic address:

We report the outcomes of 51 patients who underwent unmanipulated haploidentical hematopoietic stem cell transplantation (haplo-HSCT) with post-transplantation cyclophosphamide (PT-Cy) and antithymocyte globulin (ATG), from peripheral blood stem cells (PBSCs) or bone marrow, after receipt of a TBF (thiotepa, busulfan, and fludarabine) conditioning regimen. Their median age was 55 years (range, 16 to 72 years). Hematologic diagnoses included acute leukemias (n = 31), lymphoid neoplasm (n = 12), myeloproliferative neoplasm (n = 5), and myelodysplastic syndromes (n = 3). Read More

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http://dx.doi.org/10.1016/j.bbmt.2019.02.025DOI Listing
March 2019
5 Reads

Exome, transcriptome and miRNA analysis don't reveal any molecular markers of TKI efficacy in primary CML patients.

BMC Med Genomics 2019 Mar 13;12(Suppl 2):37. Epub 2019 Mar 13.

Laboratory of Mutagenesis, Federal State Budgetary Institution, Research Centre for Medical Genetics, Moskvorechie str., 1, Moscow, Russian Federation, 115522.

Background: Approximately 5-20% of chronic myeloid leukemia (CML) patients demonstrate primary resistance or intolerance to imatinib. None of the existing predictive scores gives a good prognosis of TKI efficacy. Gene polymorphisms, expression and microRNAs are known to be involved in the pathogenesis of TKI resistance in CML. Read More

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http://dx.doi.org/10.1186/s12920-019-0481-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6416830PMC
March 2019
3 Reads

Treatment of myelodysplastic syndrome in the era of next-generation sequencing.

J Intern Med 2019 Mar 14. Epub 2019 Mar 14.

Department of Clinical Science, University of Bergen, Bergen, Norway.

Next-generation sequencing (NGS) is rapidly changing the clinical care of patients with myelodysplastic syndrome (MDS). NGS can be used for various applications: (i) in the diagnostic process to discriminate between MDS and other diseases such as aplastic anaemia, myeloproliferative disorders and idiopathic cytopenias; (ii) for classification, for example, where the presence of SF3B1 mutation is one criterion for the ring sideroblast anaemia subgroups in the World Health Organization 2016 classification; (iii) for identification of patients suitable for targeted therapy (e.g. Read More

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http://dx.doi.org/10.1111/joim.12893DOI Listing
March 2019
3 Reads

Gender disparity in the survival of patients with primary myelodysplastic syndrome.

J Cancer 2019 30;10(5):1325-1332. Epub 2019 Jan 30.

Clinical Medical College, Yangzhou University, Yangzhou 225001, China.

Several prognostic scoring systems have been developed to assess prognosis in myelodysplastic syndrome (MDS). However, currently there are no systems that list gender as a prognostic factor. We queried a National Cancer Institute database to investigate the prognostic influence of gender on the survival of patients with MDS. Read More

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http://www.jcancer.org/v10p1325.htm
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http://dx.doi.org/10.7150/jca.28220DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6400681PMC
January 2019
7 Reads

Programmed Cell Death Receptor (PD-1) Ligand (PD-L1) expression in Philadelphia chromosome-negative myeloproliferative neoplasms.

Leuk Res 2019 Apr 28;79:52-59. Epub 2019 Feb 28.

Division of Hematology/Oncology, Brookdale University Hospital Medical Center, Brooklyn, NY 11212, USA.

Programmed Cell Death Receptor (PD-1) and its Ligand (PD-L1) pathway inhibitor therapy has been explored in the field of oncology treatment mainly for solid tumors. In hematologic malignancies, there is limited information except for Hodgkin's lymphoma, and there is even less information regarding myeloproliferative neoplasm (MPN). Therefore, we explored this by first measuring PD-1 and PD-L1 levels (percentage of positive cells) in 63 patients with Philadelphia chromosome-negative MPN (Ph(-) MPN), including 16 MF (12 PMF, 2 post-PV-MF, 2 post-ET-MF), 29 ET, and 18 PV. Read More

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http://dx.doi.org/10.1016/j.leukres.2019.02.010DOI Listing
April 2019
1 Read
2.351 Impact Factor

A case report of systemic mastocytosis associated with multiple hematologic non-mast cell lineage diseases.

Hematol Oncol 2019 Mar 8. Epub 2019 Mar 8.

Hematology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Systemic mastocytosis (SM) is a hematological malignancy characterized by extracutaneous infiltration by atypical mast cells. Together with indolent SM, aggressive SM, and mast cell leukemia, the World Health Organization (WHO) recognizes another major disease subgroup: SM with an associated hematological neoplasm, which is characterized by the presence of a concurrent neoplasm, more commonly, a chronic myelomonocytic leukemia. While KIT D816V is commonly regarded as the driver mutation, the clinical presentation of SM is extremely varied. Read More

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http://dx.doi.org/10.1002/hon.2605DOI Listing
March 2019
3 Reads

Thromboembolic events in polycythemia vera.

Ann Hematol 2019 May 8;98(5):1071-1082. Epub 2019 Mar 8.

Hospital del Mar-IMIM, Passeig Marítim 25-29, 08003, Barcelona, Spain.

Thromboembolic events and cardiovascular disease are the most prevalent complications in patients with polycythemia vera (PV) compared with other myeloproliferative disorders and are the major cause of morbidity and mortality in this population. Moreover, a vascular complication such as arterial or venous thrombosis often leads to the diagnosis of PV. The highest rates of thrombosis typically occur shortly before or at diagnosis and decrease over time, probably due to the effects of treatment. Read More

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http://dx.doi.org/10.1007/s00277-019-03625-xDOI Listing
May 2019
5 Reads

The role of F-FDG PET in the assessment of a benign hematological disorder: polycythemia.

Hell J Nucl Med 2019 Jan-Apr;22(1):4-5. Epub 2019 Mar 7.

Department of Radiology, Perelman School of Medicine, University of Pennsylvania, PA, USA.

Fluorine-18 fluorodeoxyglucose positron emission tomography (F-FDG PET) imaging was conceived in the early 1970 by investigators at the University of Pennsylvania as a research technique to measure brain metabolism and function by employing a non-invasive imaging approach. Soon after the introduction of whole-body PET instruments, F-FDG was utilized in the assessment of a variety of solid tumors and certain hematological malignancies. Yet, the role of F-FDG in assessing benign and uncommon malignant disorders of the bone marrow has not been investigated to a great extent. Read More

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http://dx.doi.org/10.1967/s002449910951DOI Listing
March 2019
3 Reads

Molecular Cytogenetic Characterization of a Case of a Myelodysplastic/Myeloproliferative Neoplasm, Chronic Myelomonocytic Leukemia-1 (CMML-1) with Abnormal Karyotype with an Apparent Monosomy 7 Resulting in Rearrangements Involving Chromosomes 7 and 21.

J Assoc Genet Technol 2019 ;45(1):14-17

The International Circle of Genetic Studies, Chapter Los Angeles, CA, USA.

Objectives: We report the case of a 69-year-old male with peripheral blood findings of persistent anemia, mild absolute monocytosis with mild dysgranulopoiesis, rare circulating blasts, and mild thrombocytopenia. Bone marrow biopsy revealed hypercellular bone marrow (60%) with 3.4% blasts and mild dysgranulopoiesis, morphologically characteristic of myelodysplastic/myeloproliferative neoplasm, chronic myelomonocytic leukemia-1 (CMML-1). Read More

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January 2019
6 Reads

SOX6 blocks the proliferation of BCR-ABL1 and JAK2V617F leukemic cells.

Sci Rep 2019 Mar 4;9(1):3388. Epub 2019 Mar 4.

Dipartimento di Biotecnologie e Bioscienze, Università degli Studi di Milano-Bicocca, Piazza della Scienza 2, 20126, Milano, Milano, Italy.

SOX6 is a HMG-box transcription factor expressed in a wide range of tissues. Recent data show that SOX6 expression is altered in different cancers, in the majority of cases being downregulated. To date, no data are available about SOX6 role in hematological malignancies. Read More

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http://dx.doi.org/10.1038/s41598-019-39926-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6399316PMC
March 2019
3 Reads

[Efficacy and safety of domestic dasatinib as second-line treatment for chronic myeloid leukemia patients in the chronic phase].

Zhonghua Xue Ye Xue Za Zhi 2019 Feb;40(2):98-104

Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China.

To investigate the efficiency and safety of domestic tyrosine kinase inhibitor (TKI) dasatinib (Yinishu) as second-line treatment for patients with chronic myeloid leukemia in chronic phase (CML-CP). A retrospective analysis of clinical data of CML-CP patients who received domestic dasatinib as second-line treatment in the CML collaborative group hospitals of Hubei province from March 2016 to July 2018 was performed. The optimal response rate, the cumulative complete cytogenetic response (CCyR), the cumulative major molecular responses (MMR), progression free survival (PFS), event free survival (EFS) and adverse effects (AEs) of the patients were assessed at 3, 6 and 12 months of treatment. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2019.02.002DOI Listing
February 2019
1 Read

Pairing JAK with MEK for improved therapeutic efficiency in myeloproliferative disorders.

Authors:
David A Williams

J Clin Invest 2019 Mar 4;130:1519-1521. Epub 2019 Mar 4.

The identification of JAK2 mutations as disease-initiating in myeloproliferative neoplasms (MPNs) has led to new and effective therapies for these diseases. In a study published in this issue of the JCI, Stivala et al. explored the key observation that JAK inhibition successfully suppresses MAPK activation in MPN cell lines and primary MPN cells in vitro, and the finding that it failed to completely and effectively suppress MAPK activation in vivo in two mouse models. Read More

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http://dx.doi.org/10.1172/JCI127582DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6436870PMC
March 2019
2 Reads

Review article: a multidisciplinary approach to the diagnosis and management of Budd-Chiari syndrome.

Aliment Pharmacol Ther 2019 Apr 3;49(7):840-863. Epub 2019 Mar 3.

Liver Unit, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.

Background: Budd-Chiari syndrome (BCS) is a rare but fatal disease caused by obstruction in the hepatic venous outflow tract.

Aim: To provide an update of the pathophysiology, aetiology, diagnosis, management and follow-up of BCS.

Methods: Analysis of recent literature by using Medline, PubMed and EMBASE databases. Read More

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http://dx.doi.org/10.1111/apt.15149DOI Listing
April 2019
3 Reads

Development of a Potent Protein Degrader against Oncogenic BCR-ABL Protein.

Chem Pharm Bull (Tokyo) 2019 ;67(3):165-172

Divisions of Molecular Target and Gene Therapy Products, National Institute of Health Sciences.

Chromosomal translocation occurs in some cancer cells, resulting in the expression of aberrant oncogenic fusion proteins that include BCR-ABL in chronic myelogenous leukemia (CML). Inhibitors of ABL tyrosine kinase, such as imatinib and dasatinib, exhibit remarkable therapeutic effects, although emergence of drug resistance hampers the therapy during long-term treatment. An alternative approach to treat CML is to downregulate expression of the BCR-ABL protein. Read More

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http://dx.doi.org/10.1248/cpb.c18-00703DOI Listing
April 2019
1 Read

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

Network meta-analysis: a new analysis tool of the experimental evidence.

Minerva Med 2019 Apr;110(2):173-175

Department of Geriatrics, "Casa Sollievo della Sofferenza" Hospital, San Giovanni Rotondo, Foggia, Italy.

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http://dx.doi.org/10.23736/S0026-4806.18.05768-3DOI Listing
April 2019
2 Reads

Impact of primary disease on outcome after allogeneic stem cell transplantation for transformed secondary acute leukaemia.

Br J Haematol 2019 Feb 28. Epub 2019 Feb 28.

Hôpital St. Louis, Paris, France.

Myelodysplastic syndromes (MDS), myeloproliferative neoplasms (MPN) and chronic myelomonocytic leukaemia (CMML) can progress to secondary acute myeloid leukaemia (sAML). We compared the outcome of 4214 sAML patients who received allogeneic haematopoietic stem cell transplantation (allo-HSCT) from an unrelated (62%) or human leucocyte antigen (HLA)-identical sibling donor (38%) according the underlying disease: MDS (n = 3541), CMML (n = 251) or MPN (n = 422). After a median follow up of 46·5 months, the estimated 3-year progression-free (PFS) and overall survival (OS) for the entire group was 36% (34-37%) and 41% (40-43%), respectively. Read More

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http://dx.doi.org/10.1111/bjh.15819DOI Listing
February 2019
2 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

CircRNAs Are Here to Stay: A Perspective on the Recombinome.

Front Genet 2019 13;10:88. Epub 2019 Feb 13.

Department of Molecular Medicine, University of Padua, Padua, Italy.

Chromosomal translocations harbored by cancer genomes are important oncogenic drivers. In rearranged acute leukemia (MLLre) fuses with over 90 partner genes. Mechanistic studies provided clues of MLL fusion protein leukemogenic potential, but models failed to fully recapitulate the disease. Read More

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http://dx.doi.org/10.3389/fgene.2019.00088DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6382020PMC
February 2019
3 Reads

Imatinib-induced irreversible interstitial lung disease: A case report.

Medicine (Baltimore) 2019 Feb;98(8):e14402

Department of Hematology.

Rationale: Imatinib mesylate (imatinib) is a classic tyrosine kinase inhibitor used to treat chronic myeloid leukemia. Although it is well tolerated by most patients and helps in the achievement of complete remission, a few rare imatinib-associated adverse effects such as pulmonary interstitial fibrosis have been reported. Because of its rareity, the clinical features of imatinib-induced interstitial lung disease (ILD) remain unclear. Read More

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http://dx.doi.org/10.1097/MD.0000000000014402DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6407980PMC
February 2019
1 Read