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Francesca Palandri, Giuseppe Auteri, Michele Baccarani
Myelofibrosis (MF) is the most severe among the classical Philadelphia-negative myeloproliferative neoplasms that also include essential thrombocytemia and polycythemia vera. Myelofibrosis is characterized by numerous genetic lesions, often variously associated with each other, and by an aggressive clinical phenotype leading to severely reduced survival. Also, the inflammatory microenvironment plays a key role in disease initiation and progression. Because of the complexity of its pathogenesis and the variability of clinical features, MF is a disease that requires a personalized approach and remains orphan of curative treatments besides allogeneic transplantation...
August 10, 2016: Hematological Oncology
Cecilia Carubbi, Elena Masselli, Silvia Martini, Daniela Galli, Franco Aversa, Prisco Mirandola, Joseph E Italiano, Giuliana Gobbi, Marco Vitale
A deeper understanding of the molecular events driving megakaryocytopoiesis and thrombopoiesis is essential to regulate in vitro and in vivo platelet production for clinical applications. We previously documented the crucial role of PKCε in the regulation of human and mouse megakaryocyte maturation and platelet release. However, since several data show that different PKC isoforms fulfill complementary functions, we targeted PKCε and PKCδ, which show functional and phenotypical reciprocity, at the same time as boosting platelet production in vitro...
July 2016: Haematologica
G Tortorella, M Calzolari, A Tieghi, N Muià, A Piccin, L Gugliotta
No abstract text is available yet for this article.
January 15, 2016: International Journal of Cardiology
Ayfer Geduk, Elif B Atesoglu, Pinar Tarkun, Ozgur Mehtap, Abdullah Hacihanefioglu, Esra T Demirsoy, Canan Baydemir
INTRODUCTION: β-Catenin is a multifunctional protein that acts as a central effector molecule in the Wnt signaling pathway. Aberrant activation of the Wnt/β-catenin signaling pathway causes various diseases including cancer. In this study we evaluated β-catenin expression in bcr/abl-negative myeloproliferative neoplasms (MPNs). MATERIALS AND METHODS: The expression of β-catenin was evaluated in bone marrow using immunohistochemical methods in 66 patients with bcr/abl-negative myeloproliferative neoplasms (MPNs) and in 30 healthy control subjects...
December 2015: Clinical Lymphoma, Myeloma & Leukemia
Neikova K, V Dimitrova, R Dimitrov, A Savov
AIM: To analyze current practices in Bulgaria regarding antithrombotic medication (AM) during pregnancy, and to compare them with the ones recommended in literature. MATERIALS AND METHODS: In 84 pregnant women who were low dose aspirin (LDA) or/and low molecular weight heparin (LMWH) or unfractionated heparin (UH), data about AM were collected and analyzed. A descriptive analysis was performed of the indications for AM, its type, the applied doses and therapeutic regimens...
2015: Akusherstvo i Ginekologii︠a︡
A Alnajjar, H Al-Hussaini, M Al Sebayel, W Al-Kattan, H Elsiesy
Budd-Chiari syndrome is a rare condition caused by interrupted hepatic venous outflow in the hepatic veins, inferior vena cava, or right atrium. Reports from the literature have delineated on focal nodular hyperplasia (FNH)-like lesions in association with Budd-Chiari Syndrome. To our knowledge, there are no reports about true FNH lesions in patients with Budd-Chiari Syndrome. Focal nodular hyperplasia develops in disorders with aberrant circulation and vasculature. We report a case of Budd-Chiari syndrome in association with large solitary FNH in a 22-year-old man who was referred to our institution with sudden intermittent right upper quadrant abdominal pain, vomiting, diarrhea with pale stool, decreased appetite, dark urine, and abdominal distention for 15 days...
September 2015: Transplantation Proceedings
Mihaela Tevet, Razvan Ionescu, Cornel Dragan, Anca Roxana Lupu
BACKGROUND: A number of studies showed that the JAK2 V617F mutation increases the thrombotic risk in patients with myeloproliferative disorders (MPN) while others did not reveal this correlation, and it is unknown whether inherited thrombophilia is an additive risk factor in mutated subjects. Our aim was to clarify the contribution of JAK2 V617F to a hypercoagulable state, as well as its interaction with other thrombophilic factors in patients with thrombosis and myeloproliferative disorders...
March 2015: Mædica
Minodora Onisai, Irina Voican, Mihai Ciorascu, Viola Popov, Horia Bumbea, Mihaela Gaman, Alexandru Filipescu, Radu Vladareanu, Ana-Maria Vladareanu
Essential thrombocytemia--a classic myeloproliferative neoplasm characterized by persistent thrombocytosis--may associate both thrombotic and hemorrhagic events, as well as platelet dysfunction. Of all myeloproliferative neoplasms, essential thrombocytemia is more likely to be associated with pregnancy, because of a higher comparative incidence in younger patients. This association significantly increases the risk of pregnancy loss and of various pregnancy complications. We present a case of early severe preeclampsia with a critical and unusual evolution and life-threatening complications...
October 2015: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
Serena Rupoli, Gaia Goteri, Paola Picardi, Giorgia Micucci, Lucia Canafoglia, Anna Rita Scortechini, Irene Federici, Federica Giantomassi, Lidia Da Lio, Antonio Zizzi, Elisa Honorati, Pietro Leoni
BACKGROUND: Vascular events represent the most frequent complications of thrombocytemias. We aimed to evaluate their risk in the WHO histologic categories of Essential Thrombocytemia (ET) and early Primary Myelofibrosis (PMF). METHODS: From our clinical database of 283 thrombocytemic patients, we selected those with available bone marrow histology performed before any treatment, at or within 1 year from diagnosis, and reclassified the 131 cases as true ET or early PMF, with or without fibrosis, according to the WHO histological criteria...
2015: Diagnostic Pathology
Zouhayr Souirti, Ouafae Messouak, Faouzi Belahsen
Cerebral venous thrombosis (CVT) is a rare origin of stroke, the clinical presentation and etiologies vary. The prognosis is shown to be better than arterial thrombosis. Magnetic Resonance Imaging (MRI) and MR Venograpgy (MRV) are currently important tools for the diagnosis. We studied 30 cases of CVT diagnosed in the department of neurology at the University Hospital of Fez (Morocco). Patients diagnosed with CVT signs between January 2003 and October 2007 were included in the study. Cerebral CT-scan was performed in 27 cases (90%) while the MRI examination was done in 18 patients (67%); and most patients (90%) received anticoagulant therapy...
2014: Pan African Medical Journal
Naoufal Hjira, Rachid Frikh, Abderrahmane ALBouzidi, Mohammed Boui
Cerebral venous thrombosis (CVT) is a rare origin of stroke, the clinical presentation and etiologies vary. The prognosis is shown to be better compared to arterial thrombosis. Magnetic Resonance Imaging (MRI) and MR Venograpgy (MRV) are currently important tools for the diagnostic. We studied 30 cases of CVT diagnosed in the department of neurology of the University Hospital of Fez (Morocco). Patients diagnosed with CVT signs between January 2003 and October 2007 were included in the study. Cerebral CT-scan was performed in 27 cases (90%) while the MRI examination was done in 18 patients (67%); and most patients (90%) received anticoagulant therapy...
2014: Pan African Medical Journal
Rodolphe Besancenot, Damien Roos-Weil, Carole Tonetti, Hadjer Abdelouahab, Catherine Lacout, Florence Pasquier, Christophe Willekens, Philippe Rameau, Yann Lecluse, Jean-Baptiste Micol, Stefan N Constantinescu, William Vainchenker, Eric Solary, Stéphane Giraudier
Megakaryopoiesis is a 2-step differentiation process, regulated by thrombopoietin (TPO), on binding to its cognate receptor myeloproliferative leukemia (MPL). This receptor associates with intracytoplasmic tyrosine kinases, essentially janus kinase 2 (JAK2), which regulates MPL stability and cell-surface expression, and mediates TPO-induced signal transduction. We demonstrate that JAK2 and MPL mediate TPO-induced proliferation arrest and megakaryocytic differentiation of the human megakaryoblastic leukemia cell line UT7-MPL...
September 25, 2014: Blood
Pierre Fontana, Alessandro Casini, Françoise Boehlen, Jean-Luc Reny
Aspirin is a cornerstone in the prevention of ischemic events and guidelines usually recommend a once-daily dosing. This dosing is based at least in part on the platelet renewal rate, which is of only 10-15% a day. A once-daily dosing is now challenged by several studies demonstrating that when platelet turnover is increased, such as in patients with diabetes or essential thrombocytemia, the inhibition of platelet function provided by aspirin is no longer homogeneous between dosing with a significant recovery of platelet function within a day that is blunted by a twice-daily dosing...
January 15, 2014: Revue Médicale Suisse
F Bilgir, O Bilgir, M Calan, F Sari
AIM: It is known that thrombocytosis is closely related to vascular complications and particularly thrombosis in essential thrombocytemia (ET) cases. The aim of this study is to investigate a possible correlation between adhesion molecules and vascular attacks as well as the relation of these molecules to the platelet count. METHODS: The study group consisted of 30 ET patients and 30 healthy controls subjects. Serum intracellular adhesion molecule-1 (ICAM-1), serum vascular adhesion molecule-1 (VCAM-1) and serum E-selectin levels were determined by ELISA method according to manufacturer's instructions...
December 2013: Panminerva Medica
Miguel Gallardo, Santiago Barrio, Marisol Fernandez, Alberto Paradela, Alicia Arenas, Oscar Toldos, Rosa Ayala, Enriqueta Albizua, Ana Jimenez, Santiago Redondo, Rosa Maria Garcia-Martin, Florinda Gilsanz, Juan Pablo Albar, Joaquin Martinez-Lopez
JAK-STAT signaling through the JAK2V617F mutation is central to the pathogenesis of myeloproliferative neoplasms (MPN). However, other events could precede the JAK2 mutation. The aim of this study is to analyze the phenotypic divergence between polycytemia vera (PV) and essential thrombocytemia (ET) to find novel therapeutics targets by a proteomic and functional approach to identify alternative routes to JAK2 activation. Through 2D-DIGE and mass spectrometry of granulocyte protein from 20 MPN samples, showed differential expression of HSP70 in PV and ET besides other 60 proteins...
2013: Molecular Cancer
O Y Mishcheniuk, O M Kostukevich, I V Dmytrenko, V V Sholoyko, I M Prokopenko, Z V Martina, G V Pilipenko, S V Klymenko
AIM: The aim of this study was to examine the JAK2 V617F, the G1691A allele of factor V, and the G20210A prothrombin gene mutation status, and their predictive value for thrombosis in patients with Ph-negative myeloproliferative neoplasms (MPN) in Ukraine, with special emphasize to patient exposed to ionizing radiation due to the Chernobyl accident. MATERIALS AND METHODS: There were 198 patients with Ph-negative MPN included in the study. Of these, 45 patients had experienced radiation exposure due to the Chernobyl accident...
September 2013: Experimental Oncology
Mandy Brecqueville, Jérôme Rey, Raynier Devillier, Arnaud Guille, Rémi Gillet, José Adélaide, Véronique Gelsi-Boyer, Christine Arnoulet, Max Chaffanet, Marie-Joelle Mozziconacci, Norbert Vey, Daniel Birnbaum, Anne Murati
Myelofibrosis is a myeloproliferative neoplasm that occurs de novo (primary myelofibrosis) or results from the progression of polycythemia vera or essential thrombocytemia (hereafter designated as secondary myelofibrosis or post-polycythemia vera/ essential thrombocythemia myelofibrosis). To progress in the understanding of myelofibrosis and to find molecular prognostic markers we studied 104 samples of primary and secondary myelofibrosis at chronic (n=68) and acute phases (n=12) from 80 patients, by using array-comparative genomic hybridization and sequencing of 23 genes (ASXL1, BMI1, CBL, DNMT3A, EZH2, IDH1/2, JAK2, K/NRAS, LNK, MPL, NF1, PPP1R16B, PTPN11, RCOR1, SF3B1, SOCS2, SRSF2, SUZ12, TET2, TP53, TRPS1)...
January 2014: Haematologica
Essohana Padaro, Kossi Agbetiafa, Irénée Messanh Delagnon Kueviakoe, Yao Layibo, Koffi Amegbor, Ahoefa Vovor, Stéphane Giraudier, Ibrahima Sanogo, Akueté Yvon Segbena
The goal of our study is to document the prevalence of change JAK2V617F among patients reached of myeloproliferative syndromes (MPS) in Togo in order to evaluate frequencies. This descriptive study included 15 patients followed with the CHU Campus for a SMP. The research of JAK2 change by PCR was carried out with the APHP Henri Mondor of Creteil (France). During the study period, 15 patients followed for MPS (9 cases of polycythemia Vera, 5 cases of essential thrombocytemia and a case of primitive myelofibrosis) profited from the research of JAK2 change...
October 1, 2012: Annales de Biologie Clinique
János László Iványi, Eva Marton, Márk Plander
INTRODUCTION: Treatment of immune thrombocytopenia is sometimes difficult and needs personal setting. According to evidence-based guidelines, corticosteroids are suggested for first-line treatment. In case of corticosteroid ineffectiveness, second-line therapeutic options (splenectomy, immunosuppressive drugs and, recently, thrombopoietin-mimetics) may result in beneficial therapeutic effect. AIMS: The aim of the authors was to examine the clinicopathological data, disease course, treatment results, and the effectiveness of novel drugs in patients with immune thrombocytopenia...
October 14, 2012: Orvosi Hetilap
A O Abdullaeva, O A Glinshchikova, S A Suslova, N Kh Shadieva, L Iu Kolosova, L M Meshcheriakova, M V Vakhrusheva, A B Sudarikov
The mutation V617F of gene JAK2 is detected in 95% of patients with genuine polycythemia, in 50% of patients with essential thrombocytemia and idiopathic myelofibrosis. The mutation V617F can be applied as a molecular marker of response to treatment in patients with chronic myeloproliferative diseases associated with this mutation. The technique of quantitative evaluation of V617F (sensitivity up to 0.01%) using polymerase chain reaction is described. This method can be applied to assess the minimal residual disease in patients with chronic myeloproliferative diseases...
July 2012: Klinicheskaia Laboratornaia Diagnostika
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