keyword
MENU ▼
Read by QxMD icon Read
search

Myeloproliferative disease

keyword
https://www.readbyqxmd.com/read/28927137/atypical-chronic-myeloid-leukemia-with-isochromosome-x-p10-a-case-report
#1
Masahide Yamamoto, Sayaka Suzuki, Jun-Ichi Mukae, Keisuke Tanaka, Ken Watanabe, Gaku Oshikawa, Tetsuya Fukuda, Naomi Murakami, Osamu Miura
Atypical chronic myeloid leukemia (aCML) is a rare subtype of myelodysplastic/myeloproliferative neoplasm (MDS/MPN). Although recurrent chromosomal and genetic abnormalities are frequently observed in aCML, none are specific to this type of leukemia. The present study reported a case of aCML associated with i(X)(p10), a rare recurrent chromosomal abnormality of hematological malignancy. A 40-year-old female was referred to the Tokyo Medical and Dental University Hospital (Tokyo, Japan) due to slight leukocytosis and anemia...
September 2017: Oncology Letters
https://www.readbyqxmd.com/read/28914569/mutations-in-myeloproliferative-neoplasms-their-significance-and-clinical-use
#2
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/28903325/cxcl12-cxcr4-pathway-is-activated-by-oncogenic-jak2-in-a-pi3k-dependent-manner
#3
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/28902850/copy-number-variation-analysis-in-cytochromes-and-glutathione-s-transferases-may-predict-efficacy-of-tyrosine-kinase-inhibitors-in-chronic-myeloid-leukemia
#4
Alexander V Lavrov, Oksana A Ustaeva, Elmira P Adilgereeva, Svetlana A Smirnikhina, Ekaterina Y Chelysheva, Oleg A Shukhov, Yuriy V Shatokhin, Sergey V Mordanov, Anna G Turkina, Sergey I Kutsev
Chronic myeloid leukemia (CML) is a myeloproliferative disease characterized by the presence of BCR/ABL fusion gene in leukemic cells, which promotes uncontrolled cell proliferation. Up to 20% of CML patients show primary resistance or non-optimal response to tyrosine kinase inhibitor (TKI) therapy. We investigated the association between copy number variation (CNV) in glutathione S-transferases (GST) and cytochromes (CYP) and the response rate to TKI. We enrolled 47 patients with CML: 31 with an optimal response and 16 with failure at 6 months in accordance with European LeukemiaNet 2013 recommendations...
2017: PloS One
https://www.readbyqxmd.com/read/28881484/primary-cells-in-bcr-fgfr1-positive-8p11-myeloproliferative-syndrome-are-sensitive-to-dovitinib-ponatinib-and-dasatinib
#5
Niklas Landberg, Arta Dreimane, Marianne Rissler, Rolf Billström, Helena Ågerstam
OBJECTIVES: Translocations involving the Fibroblast growth factor receptor 1 (FGFR1) gene are associated with the 8p11 myeloproliferative syndrome (EMS), a rare neoplasm that following a usually short chronic phase progresses into acute myeloid or lymphoid leukemia. The treatment commonly involves chemotherapy and, if possible, allogeneic stem cell transplantation which is the only therapeutic option for long term survival. Given the aggressive course of EMS, we here evaluated tyrosine kinase inhibitors as treatment options to delay disease progression...
September 7, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28871936/xanthoma-like-skin-changes-in-an-elderly-woman-with-a-normal-lipid-profile
#6
Piotr Nockowski, Zdzisław Woźniak, Adam Reich, Joanna Maj
Dear Editor, An 83-year-old woman developed yellow-brownish infiltrates, nodules, and tumors mimicking xanthomas, mostly involving the periorbital and chest area within three months (Figure 1). She had no abnormalities in serum cholesterol or triglycerides levels. A detailed laboratory analysis revealed the presence of mild monoclonal gammopathy with a presence of immunoglobulin G (IgG) kappa light chains; however, according to hematologist consultation, it did not require medical intervention. Imaging assessment and ultrasound examination did not show any specific involvement of internal organs...
July 2017: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/28866671/autoimmune-myelofibrosis-clinical-features-course-and-outcome
#7
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/28863787/venous-thromboembolism-and-hyperhomocysteinemia-as-first-manifestation-of-pernicious-anemia-a-case-series
#8
W Ammouri, Z Mezalek Tazi, H Harmouche, M Maamar, M Adnaoui
BACKGROUND: Hyperhomocysteinemia has been suspected of favoring thrombosis. Several case-control studies and even a meta-analysis have confirmed a link between venous thrombosis and hyperhomocysteinemia. Homocysteine is due to genetic and acquired factors (poor diet in folate and vitamin B12, older age, renal impairment, thyroid diseases, and malignancies) induced by the intake and the concentrations of vitamin B9 or B12 in the majority of cases. CASES PRESENTATION: We report the cases of four Moroccan patients who presented with acute vein thrombosis of different sites: a 34-year-old man, a 60-year-old man, a 58-year-old man, and a 47-year-old woman...
September 2, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28860000/pre-engraftment-bloodstream-infections-after-allogeneic-hematopoietic-cell-transplantation-the-impact-of-t-repleted-transplant-from-haploidentical-donors
#9
Malgorzata Mikulska, Anna Maria Raiola, Federica Galaverna, Elisa Balletto, Maria Lucia Borghesi, Riccardo Varaldo, Francesca Gualandi, Livia Giannoni, Giordana Pastori, Daniele Roberto Giacobbe, Alessio Signori, Valerio Del Bono, Claudio Viscoli, Andrea Bacigalupo, Emanuele Angelucci
Bloodstream infections (BSI) are frequent and important infectious complications after hematopoietic cell transplantation (HCT). The aim of this study was to analyze the incidence, risk factors and outcome of pre-engraftment BSI after allogeneic HCT. A retrospective analysis of data from 553 consecutive patients transplanted in years 2010-2016 was performed. Sixty percent of patients received T-repleted unmanipulated haploidentical bone marrow with high dose post-transplant cyclophosphamide. BSI rate was 30%; among isolated 213 pathogens, 54% were Gram-positive, 43% Gram-negative and 3% fungi...
August 28, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28859112/mathematical-modelling-as-a-proof-of-concept-for-mpns-as-a-human-inflammation-model-for-cancer-development
#10
Morten Andersen, Zamra Sajid, Rasmus K Pedersen, Johanne Gudmand-Hoeyer, Christina Ellervik, Vibe Skov, Lasse Kjær, Niels Pallisgaard, Torben A Kruse, Mads Thomassen, Jesper Troelsen, Hans Carl Hasselbalch, Johnny T Ottesen
The chronic Philadelphia-negative myeloproliferative neoplasms (MPNs) are acquired stem cell neoplasms which ultimately may transform to acute myelogenous leukemia. Most recently, chronic inflammation has been described as an important factor for the development and progression of MPNs in the biological continuum from early cancer stage to the advanced myelofibrosis stage, the MPNs being described as "A Human Inflammation Model for Cancer Development". This novel concept has been built upon clinical, experimental, genomic, immunological and not least epidemiological studies...
2017: PloS One
https://www.readbyqxmd.com/read/28846072/mice-expressing-krasg12d-in-hematopoietic-multipotent-progenitor-cells-develop-neonatal-myeloid-leukemia
#11
Stefan P Tarnawsky, Rebecca J Chan, Mervin C Yoder
Juvenile myelomonocytic leukemia (JMML) is a pediatric myeloproliferative neoplasm that bears distinct characteristics associated with abnormal fetal development. JMML has been extensively modeled in mice expressing the oncogenic KrasG12D mutation. However, these models have struggled to recapitulate the defining features of JMML due to in utero lethality, nonhematopoietic expression, and the pervasive emergence of T cell acute lymphoblastic leukemia. Here, we have developed a model of JMML using mice that express KrasG12D in multipotent progenitor cells (Flt3Cre+ KrasG12D mice)...
August 28, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28845953/vasculopathies-cutaneous-necrosis-and-emergency-in-dermatology
#12
Constanza Martinez-Mera, Javier Fraga, Tania M Capusan, Maria Herrero-Moyano, Almudena Godoy-Trapero, Mar Llamas-Velasco
The most of the emergency in dermatology comprise a variety of entities that most are benign in course. However the vasculopathies and vasculitis are not common,but they could represent the 1,9% and 4,4% according to some studies of emergency dermatology department. They become an important diseases which have to identified early to established an appropiate managment and treatment. Some of them are well known as the leukocitoclastic vasculitis, Schölein-Henoch, panarteritis nodosa, ANCA associated vasculitis, giant cell arteritis, cryoglobulinemic vasculitis and antiphospholipid syndrome...
July 28, 2017: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://www.readbyqxmd.com/read/28828906/mpn10-score-and-survival-of-molecularly-annotated-myeloproliferative-neoplasm-patients
#13
Yasser H ElNahass, Hossam K Mahmoud, Mervat M Mattar, Omar A Fahmy, Mohamed A Samra, Raafat M Abdelfattah, Fatma A ElRefaey, Hala M Fahmy, Gamal M Fathy, Ahmed Abdulgawad, Mohamed AbdelKader, Hend N Elleithy, Mona Gamil, Mohamed Talaat, Heba A Nader, Wafaa H ElMetnawy
JAK2, CALR, MPL and triple-negative mutational status has a direct impact on symptom severity and disease burden assessed by MPN10 score in myeloproliferative neoplasms (MPNs). Among 93 patients; median MPN10 score was 48 (5-76) in JAK2 mutants versus 25 (4-80) in JAK2 negative (p < .001); 22.5 (4-65) in CALR mutants versus 35 (5-80) in CALR negative (p < .050) and 21 (10-48) in triple negative versus 40 (4-80) in JAK2/CALR/MPL mutants (p < .001). At three years, progression free and overall survival of JAK2-positive versus JAK2-negative patients were 62% versus 100% (p < ...
August 22, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28822056/patient-reported-outcomes-in-myelodysplastic-syndromes-and-mds-mpn-overlap-syndromes-stepping-onto-the-stage-with-changing-times
#14
REVIEW
Sagar S Patel, Aaron T Gerds
Quality of life (QOL) and symptom burden are important measures captured by patient-reported outcomes (PROs). Myelodysplastic and myelodysplastic/myeloproliferative (MDS/MPN) neoplasm overlap syndromes are notable for significant morbidity and mortality, including a wide spectrum of physical and psychosocial effects. Thus, the development and application of PROs can provide meaningful information to facilitate communication and assist in follow up care. Disease-specific measures can more accurately reflect the full breadth of functional restrictions and symptoms...
August 18, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28821362/membranous-nephropathy-and-intrarenal-extramedullary-hematopoiesis-in-a-patient-with-myelofibrosis
#15
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/28812191/improved-outcome-of-childhood-acute-myeloid-leukemia-in-an-eastern-european-country-lithuanian-experience
#16
Igne Kairiene, Ramune Pasauliene, Nadezda Lipunova, Goda Vaitkeviciene, Lina Rageliene, Jelena Rascon
The reported treatment outcomes of children treated for cancer in Eastern European countries are inferior to those in Northern/Western Europe. We hypothesized that recent survival rates could be comparable to the current standards and performed a population-based analysis of treatment outcome of childhood acute myeloid leukemia (AML) in Lithuania, a small Eastern European country. Children < 18 years old who were treated for AML from 2000 to 2013 were included (n = 54). Estimates of 5-year event-free (EFS5y) and overall survival (OS5y) rates were analyzed...
August 15, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28796790/mesenchymal-stromal-cells-msc-from-jak2-myeloproliferative-neoplasms-differ-from-normal-msc-and-contribute-to-the-maintenance-of-neoplastic-hematopoiesis
#17
Teresa L Ramos, Luis Ignacio Sánchez-Abarca, Beatriz Rosón-Burgo, Alba Redondo, Ana Rico, Silvia Preciado, Rebeca Ortega, Concepción Rodríguez, Sandra Muntión, Ángel Hernández-Hernández, Javier De Las Rivas, Marcos González, José Ramón González Porras, Consuelo Del Cañizo, Fermín Sánchez-Guijo
There is evidence of continuous bidirectional cross-talk between malignant cells and bone marrow-derived mesenchymal stromal cells (BM-MSC), which favors the emergence and progression of myeloproliferative neoplastic (MPN) diseases. In the current work we have compared the function and gene expression profile of BM-MSC from healthy donors (HD-MSC) and patients with MPN (JAK2V617F), showing no differences in the morphology, proliferation and differentiation capacity between both groups. However, BM-MSC from MPN expressed higher mean fluorescence intensity (MIF) of CD73, CD44 and CD90, whereas CD105 was lower when compared to controls...
2017: PloS One
https://www.readbyqxmd.com/read/28791654/pharmacotherapy-of-myelofibrosis
#18
REVIEW
Douglas Tremblay, Bridget Marcellino, John Mascarenhas
Myelofibrosis (MF) is a myeloproliferative neoplasm that is pathologically characterized by bone marrow myeloproliferation, reticulin and collagen fibrosis, and extramedullary hematopoiesis. Constitutive activation of the Janus associated kinase (JAK)-signal transducers and activators of transcription signaling pathway with resultant elevation in pro-inflammatory cytokine levels is the pathogenic hallmark of MF. JAK inhibitors, namely ruxolitinib, have been successful in alleviating symptoms and reducing splenomegaly, but therapy-related myelosuppression has led to the further development of highly selective JAK2 inhibitors...
August 8, 2017: Drugs
https://www.readbyqxmd.com/read/28781891/vascular-occlusions-following-ocular-surgical-procedures-a-clinical-observation-of-vascular-complications-after-ocular-surgery
#19
Charlotte Fischer, Anne Bruggemann, Annette Hager, Josep Callizo Planas, Johann Roider, Hans Hoerauf
BACKGROUND: Ocular vascular occlusions following intraocular procedures are a rare complication. We report a case series of patients with retinal vascular occlusions or anterior ischemic optic neuropathy (AION) after anterior and posterior segment surgery and demonstrate possible risk factors. METHODS: Observational case series. RESULTS: In ten patients, vascular occlusions were observed within ten weeks after intraocular surgery: branch retinal arterial occlusion (BRAO) (n = 2), central retinal artery occlusion (CRAO) (n = 2), central retinal vein occlusion (CRVO) (n = 1), branch retinal vein occlusion (BRVO) (n = 1), anterior ischemic optic neuropathy (AION) (n = 3), and combined central artery and vein occlusion (n = 1)...
2017: Journal of Ophthalmology
https://www.readbyqxmd.com/read/28780729/the-impact-of-myeloproliferative-neoplasms-mpns-on-patient-quality-of-life-and-productivity-results-from-the-international-mpn-landmark-survey
#20
Claire N Harrison, Steffen Koschmieder, Lynda Foltz, Paola Guglielmelli, Tina Flindt, Michael Koehler, Jonathan Mathias, Norio Komatsu, Robert N Boothroyd, Amber Spierer, Julian Perez Ronco, Gavin Taylor-Stokes, John Waller, Ruben A Mesa
Myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET) are myeloproliferative neoplasms (MPNs) associated with high disease burden, reduced quality of life (QOL), and shortened survival. To assess how MPNs affect patients, we conducted a global MPN Landmark survey. This online survey of patients with MPNs and physicians was conducted in Australia, Canada, Germany, Japan, Italy, and the United Kingdom. The survey measured MPN-related symptoms and the impact of MPNs on QOL and the ability to work as well as disease-management strategies...
August 5, 2017: Annals of Hematology
keyword
keyword
21407
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"