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Primary myelofibrosis

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https://www.readbyqxmd.com/read/28934680/a-phase-1-study-of-the-janus-kinase-2-jak2-v617f-inhibitor-gandotinib-ly2784544-in-patients-with-primary-myelofibrosis-polycythemia-vera-and-essential-thrombocythemia
#1
Srdan Verstovsek, Ruben A Mesa, Mohamed E Salama, Li Li, Celine Pitou, Fabio P Nunes, Gregory L Price, Jennifer L Giles, Deborah N D'Souza, Richard A Walgren, Josef T Prchal
Mutations in Janus kinase 2 (JAK2) are implicated in the pathogenesis of Philadelphia-chromosome negative myeloproliferative neoplasms, including primary myelofibrosis, polycythemia vera, and essential thrombocythemia. Gandotinib (LY2784544), a potent inhibitor of JAK2 activity, shows increased potency for the JAK2(V617F) mutation. The study had a standard 3+3 dose-escalation design to define the maximum-tolerated dose. Primary objectives were to determine safety, tolerability, and recommended oral daily dose of gandotinib for patients with JAK2(V617F)-positive myelofibrosis, essential thrombocythemia, or polycythemia vera...
August 31, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28933777/generation-and-use-of-a-humanized-bone-marrow-ossicle-niche-for-hematopoietic-xenotransplantation-into-mice
#2
Andreas Reinisch, David Cruz Hernandez, Katharina Schallmoser, Ravindra Majeti
Xenotransplantation is frequently used to study normal and malignant hematopoiesis of human cells. However, conventional mouse xenotransplantation models lack essential human-specific bone-marrow (BM)-microenvironment-derived survival, proliferation, and self-renewal signals for engraftment of normal and malignant blood cells. As a consequence, many human leukemias and other hematologic disorders do not robustly engraft in these conventional models. Here, we describe a complete workflow for the generation of humanized ossicles with an accessible BM microenvironment that faithfully recapitulates normal BM niche morphology and function...
October 2017: Nature Protocols
https://www.readbyqxmd.com/read/28930494/simplify-1-a-phase-iii-randomized-trial-of-momelotinib-versus-ruxolitinib-in-janus-kinase-inhibitor-na%C3%A3-ve-patients-with-myelofibrosis
#3
Ruben A Mesa, Jean-Jacques Kiladjian, John V Catalano, Timothy Devos, Miklos Egyed, Andrzei Hellmann, Donal McLornan, Kazuya Shimoda, Elliott F Winton, Wei Deng, Ronald L Dubowy, Julia D Maltzman, Francisco Cervantes, Jason Gotlib
Purpose We evaluated the efficacy and safety of momelotinib, a potent and selective Janus kinase 1 and 2 inhibitor (JAKi), compared with ruxolitinib, in JAKi-naïve patients with myelofibrosis. Patients and Methods Patients (N = 432) with high risk or intermediate-2 risk or symptomatic intermediate-1 risk myelofibrosis were randomly assigned to receive 24 weeks of treatment with momelotinib 200 mg once daily or ruxolitinib 20 mg twice a day (or per label), after which all patients could receive open-label momelotinib...
September 20, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28914569/mutations-in-myeloproliferative-neoplasms-their-significance-and-clinical-use
#4
Fiorella Schischlik, Robert Kralovics
Clonal hematologic diseases of the blood such as polycythemia vera, essential thrombocythemia and primary myelofibrosis belong to the BCR-ABL negative Myeloproliferative Neoplasms (MPN). These diseases are characterized by clonal expansion of hematopoietic precursor cells followed by increased production of differentiated cells of the myeloid lineage. Initiation of clonal hematopoiesis, formation of a clinical phenotype as well as disease progression form part of MPN disease evolution. The disease is driven by acquired somatic mutations in critical pathways such as cytokine signaling, epigenetic regulation, RNA splicing, and transcription factor signaling...
September 15, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28906207/high-absolute-basophil-count-is-a-powerful-independent-predictor-of-inferior-overall-survival-in-patients-with-primary-myelofibrosis
#5
Marko Lucijanic, Ana Livun, Tajana Stoos-Veic, Vlatko Pejsa, Ozren Jaksic, David Cicic, Jelena Lucijanic, Zeljko Romic, Biserka Orehovec, Gorana Aralica, Marko Miletic, Rajko Kusec
OBJECTIVES: To investigate the clinical and prognostic significance of absolute basophil count (ABC) in patients with primary myelofibrosis (PMF). METHODS: We retrospectively investigated 58 patients with PMF treated in our institution in the period from 2006 to 2017. ABC was obtained in addition to other hematological and clinical parameters. Patients were separated into high and low ABC groups using the Receiver operating characteristic curve analysis. RESULTS: ABC was higher in PMF patients than in healthy controls (P < 0...
September 14, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28903325/cxcl12-cxcr4-pathway-is-activated-by-oncogenic-jak2-in-a-pi3k-dependent-manner
#6
Hadjer Abdelouahab, Yanyan Zhang, Monika Wittner, Shinya Oishi, Nobutaka Fujii, Rodolphe Besancenot, Isabelle Plo, Vincent Ribrag, Eric Solary, William Vainchenker, Giovanni Barosi, Fawzia Louache
JAK2 activation is the driver mechanism in BCR-ABL-negative myeloproliferative neoplasms (MPN). These diseases are characterized by an abnormal retention of hematopoietic stem cells within the bone marrow microenvironment and their increased trafficking to extramedullary sites. The CXCL12/CXCR4 axis plays a central role in hematopoietic stem cell/ progenitor trafficking and retention in hematopoietic sites. The present study explores the crosstalk between JAK2 and CXCL12/CXCR4 signaling pathways in MPN. We show that JAK2, activated by either MPL-W515L expression or cytokine stimulation, cooperates with CXCL12/CXCR4 signaling to increase the chemotactic response of human cell lines and primary CD34(+) cells through an increased phosphatidylinositol-3-kinase (PI3K) signaling...
August 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28895103/a-case-of-perirenal-extramedullary-hematopoiesis-in-a-patient-with-primary-myelofibrosis
#7
Kentaro Imai, Tomonori Aoi, Hiroki Kitai, Nobuhide Endo, Masahiko Fujino, Shizunori Ichida
Extramedullary hematopoiesis (EMH) is hematopoiesis in organs outside the bone marrow and most frequently occurs in the liver, spleen, and lymph nodes. We report a case of perirenal EMH revealed by kidney biopsy in a patient with primary myelofibrosis. We observed only bilateral kidney enlargement with plain computed tomography (CT) and ultrasonography before obtaining a renal biopsy. We obtained a percutaneous biopsy from the lower pole of the left kidney using ultrasonographic guidance. Ultrasonography just after the renal biopsy revealed no bleeding around the kidney...
September 11, 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28866671/autoimmune-myelofibrosis-clinical-features-course-and-outcome
#8
Caroline I Piatek, Maria E Vergara-Lluri, Vinod Pullarkat, Imran N Siddiqi, Casey O'Connell, Russell K Brynes, Donald I Feinstein
BACKGROUND: Autoimmune myelofibrosis (AIMF) is an underrecognized cause of nonmalignant bone marrow fibrosis which occurs in the presence or absence of a defined systemic autoimmune disease. Patients with AIMF present with cytopenias and autoantibodies, and have a distinctive nonclonal myelofibrosis on bone marrow examination. AIMF is distinguished from primary myelofibrosis by the absence of splenomegaly, eosinophilia, or basophilia, and the absence of abnormal myeloid, erythroid, or megakaryocytic morphology...
September 2, 2017: Acta Haematologica
https://www.readbyqxmd.com/read/28821362/membranous-nephropathy-and-intrarenal-extramedullary-hematopoiesis-in-a-patient-with-myelofibrosis
#9
Carole Philipponnet, Pierre Ronco, Julien Aniort, Jean-Louis Kemeny, Anne-Elisabeth Heng
Kidney disease in the setting of a hematologic malignancy is common, with the frequency and type of kidney disease varying depending on the specific malignancy. Various glomerular diseases and tumor infiltration of the kidneys have been reported in patients with lymphoproliferative disorders. Descriptions of kidney involvement in myeloproliferative disorders have been much rarer. We report a case of membranous nephropathy accompanied by kidney injury in a patient with primary myelofibrosis with additional features considered related to the patient's myeloproliferative disorder...
August 16, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28819279/application-of-current-prognostic-models-for-primary-myelofibrosis-in-the-setting-of-post-polycythemia-vera-or-post-essential-thrombocythemia-myelofibrosis
#10
A Tefferi, L Saeed, C A Hanson, R P Ketterling, A Pardanani, N Gangat
Leukemia accepted article preview online, 18 August 2017. doi:10.1038/leu.2017.268.
August 18, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28809641/cementless-total-hip-arthroplasty-in-primary-myelofibrosis-a-case-report
#11
S Brodt, E Eigendorff, G Matziolis
There is a great deal of data available, in part contradictory, on the best fixation technique to use for total hip arthroplasty (THA) in hip osteoarthritis. Both the cementless and the cemented versions offer excellent long-term outcomes - if the respective technique is applied correctly. However, as far as we know, no recommendation has been made regarding cemented vs. cementless THA in primary myelofibrosis. The case described here concerns a very active 76-year-old patient with primary myelofibrosis. This is a rare hematological disease...
2017: Acta Chirurgiae Orthopaedicae et Traumatologiae Cechoslovaca
https://www.readbyqxmd.com/read/28808761/the-underappreciated-risk-of-thrombosis-and-bleeding-in-patients-with-myelofibrosis-a-review
#12
REVIEW
Devendra Kc, Lorenzo Falchi, Srdan Verstovsek
Bleeding and thrombosis are long recognized complications of myelofibrosis (MF) and contribute significantly to its morbidity and mortality. However, so far, few studies have evaluated the frequency of these events, their characteristics, and their prognostic impact. Based on these studies, thrombotic events in MF are about as common as in essential thrombocytemia (ET) but less common than in polycythemia vera (PV), while bleeding events are relatively more common in MF than in ET or PV. The emergence of the concept of prefibrotic primary MF (PMF), which is associated with a higher frequency of thrombohemorrhagic complications than ET, and the growing evidence that prefibrotic PMF may also have a different thrombotic and bleeding risk profiles than fibrotic (overt) PMF have emphasized the need for a reappraisal of the risk of thrombosis and hemorrhage in patients with MF...
August 14, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28775247/spinal-abscess-caused-by-salmonella-bacteremia-in-a-patient-with-primary-myelofibrosis
#13
Shehab Fareed, Abdulqadir J Nashwan, Sulieman Abu Jarir, Ahmed Husain, Dina Sameh Suliman, Friyal Ibrahim, Abbas Moustafa, Muhammad S Akhter, Mohamed A Yassin
BACKGROUND In Primary Myelofibrosis (PMF; a clonal disorder arising from the neoplastic transformation of early hematopoietic stem cells) patients, spinal cord compression (SCC) is a common complication or even a presentation symptom due to extramedullary hematopoiesis (EMH). However, a case of SCC caused by a spinal abscess is unusual. To the best of our knowledge, this is the first case report of this rare condition. CASE REPORT We are reporting the case of a 50-year-old male with primary myelofibrosis and long-standing splenomegaly with back pain as a presenting symptom who was found to have spinal cord compression...
August 4, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28770949/treatment-of-patients-with-primary-myelofibrosis-using-dasatinib
#14
Q-L Song, B Zhang, Y Xu, R-X Xia, X-H Lu, Z-X Pei, Q-W Xu, W-Y Li, Z-D Li
OBJECTIVE: Primary myelofibrosis (PMF) is a chronic clonal myeloproliferative neoplasm. It is associated with a poor prognosis, with a median survival time of approximately five years. Thus far, there are no specific targeted drugs for PMF. In this study, we evaluated the efficacy and safety of dasatinib, a second-generation tyrosine kinase inhibitor, in six PMF patients. PATIENTS AND METHODS: From June 1, 2015 to February 29, 2016, six patients with PMF in our department were enrolled into this trial...
July 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28766534/-clinical-features-of-essential-thrombocythemia-and-primary-myelofibrosis-depending-on-the-molecular-characteristics-of-disease
#15
A L Melikyan, I N Subortseva, A B Sudarikov, A M Kovrigina, E A Gilyazitdinova, T I Kolosheinova, A O Abdullaev, S A Treglazova
The aim of the present paper was to evaluate the clinical features and risk of thrombotic events (TE) in patients with essential thrombocythemia (ET) and primary myelofibrosis (PMF), depending on the molecular characteristics of disease. Clinical data and laboratory parameters were analyzed in 50 ET patients and 50 PMF ones who had been followed up at the Department for Standardization of Treatments, National Research Center for Hematology, Ministry of Health of the Russian Federation, from February 2015 to September 2016...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28747560/germline-variations-at-jak2-tert-hbs1l-myb-and-mecom-and-the-risk-of-myeloproliferative-neoplasms-in-taiwanese-population
#16
Yi-Hao Chiang, Yu-Cheng Chang, Huan-Chau Lin, Ling Huang, Chun-Chia Cheng, Wei-Ting Wang, Hung-I Cheng, Nai-Wen Su, Caleb Gon-Shen Chen, Johnson Lin, Yi-Fang Chang, Ming-Chih Chang, Ruey-Kuen Hsieh, Wen-Chien Chou, Ken-Hong Lim, Yuan-Yeh Kuo
Germline variations at JAK2, TERT, HBS1L-MYB and MECOM have been found to associate with myeloproliferative neoplasms (MPNs) in European populations. Whether these germline variations are associated with MPNs in Taiwanese population is obscure. Here we aimed to evaluate the association of five germline variations (JAK2 46/1 haplotype tagged by rs12343867, JAK2 intron 8 rs12339666, TERT rs2736100, HBS1L-MYB rs9376092 and MECOM rs2201862) and the risk of MPNs in Taiwanese population. A total of 178 MPN patients (109 essential thrombocythemia, 54 polycythemia vera and 15 primary myelofibrosis) were enrolled into this study...
July 12, 2017: Oncotarget
https://www.readbyqxmd.com/read/28745329/involvement-of-maf-spp1-axis-in-the-development-of-bone-marrow-fibrosis-in-pmf-patients
#17
S Ruberti, E Bianchi, P Guglielmelli, S Rontauroli, G Barbieri, L Tavernari, T Fanelli, R Norfo, V Pennucci, G C Fattori, C Mannarelli, N Bartalucci, B Mora, L Elli, M A Avanzini, C Rossi, S Salmoiraghi, R Zini, S Salati, Z Prudente, V Rosti, F Passamonti, A Rambaldi, S Ferrari, E Tagliafico, A M Vannucchi, R Manfredini
Primary Myelofibrosis (PMF) is a myeloproliferative neoplasm characterized by hyperplastic megakaryopoiesis and myelofibrosis. We recently described the upregulation of MAF in PMF CD34+ hematopoietic progenitor cells (HPCs) compared to healthy donor. Here we demonstrated that MAF is also upregulated in PMF compared to Essential Thrombocytemia (ET) and Polycytemia Vera (PV) HPCs. MAF overexpression and knockdown experiments shed some light into the role of MAF in PMF pathogenesis, by demonstrating that MAF favours the megakaryocyte and monocyte/macrophage commitment of HPCs and leads to the increased expression of proinflammatory and profibrotic mediators...
July 12, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28737134/nephrotic-syndrome-in-primary-myelofibrosis-with-renal-extramedullary-hematopoiesis-and-glomerulopathy-in-the-jak-inhibitor-era
#18
Rachele Del Sordo, Rachele Brugnano, Carla Covarelli, Gioia Fiorucci, Franca Falzetti, Giorgio Barbatelli, Emidio Nunzi, Angelo Sidoni
Primary myelofibrosis (PMF) is an uncommon form of myeloproliferative neoplasm (MPN) characterized by a proliferation of predominantly megakaryocytes and granulocytes in the bone marrow that, in fully-developed disease, is associated with reactive deposition of fibrous connective tissue, extramedullary hematopoiesis (EMH), and splenomegaly. Kidney involvement is rare and clinically presents with proteinuria, nephrotic syndrome, and renal insufficiency. Renal damage can be due to EMH and glomerulopathy. Renal EMH presents three patterns: infiltration of the interstitium with possible renal failure caused by functional damage of parenchyma and vessels, infiltration of capsule and pericapsular adipose tissue, and sclerosing mass-like lesions that can cause hydronephrosis and hydroureter with obstructive uropathy and renal failure...
July 24, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28731851/the-frequency-of-occurrence-of-fish-shaped-red-blood-cells-in-different-haematologic-disorders
#19
Christoph Robier, Carolin Körber, Franz Quehenberger, Manfred Neubauer, Albert Wölfler
BACKGROUND: Red blood cells (RBC) resembling the silhouette of a fish are rarely observed in peripheral blood (PB) smears. In this study, we determined the frequency of occurrence of fish-shaped RBC in different haematologic diseases. METHODS: We examined PB smears of patients with iron deficiency anaemia (IDA) (n=23), β-thalassaemia minor (BTM) (n=30), sickle cell disease (SCD) (n=7), autoimmune haemolytic anaemia (AIHA) (n=13), microangiopathic haemolytic anaemia (MAHA) (n=11), hereditary sphaerocytosis (HS) (n=4), hereditary elliptocytosis (HE) (n=3), vitamin B12 and folate deficiency (n=15), anaemia in liver disease (LD) (n=17), myelodysplastic syndrome (MDS) (n=15), acute myeloid leukaemia (AML) (n=29), chronic myeloid leukaemia (CML) (n=18), primary myelofibrosis (PMF) (n=12), chronic myelo-monocytic leukaemia (CMML) (n=15) and 21 healthy controls by light microscopy for the occurrence of fish-shaped erythrocytes...
July 21, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28728747/endothelial-to-mesenchymal-transition-in-bone-marrow-and-spleen-of-primary-myelofibrosis
#20
Benedetta Gaia Erba, Cristian Gruppi, Monica Corada, Federica Pisati, Vittorio Rosti, Niccolo' Bartalucci, Jean-Luc Villeval, Alessandro Maria Vannucchi, Giovanni Barosi, Alessandra Balduini, Elisabetta Dejana
Primary myelofibrosis is characterized by the development of fibrosis in the bone marrow that contributes to ineffective hematopoiesis. Bone marrow fibrosis is the result of a complex and not yet fully understood interaction among megakaryocytes, myeloid cells, fibroblasts, and endothelial cells. Here, we report that >30% of the endothelial cells in the small vessels of the bone marrow and spleen of patients with primary myelofibrosis have a mesenchymal phenotype, which is suggestive of the process known as endothelial-to-mesenchymal transition (EndMT)...
August 2017: American Journal of Pathology
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