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Valerio De Stefano, Bianca Rocca, Alberto Tosetto, Denise Soldati, Giovanna Petrucci, Eloise Beggiato, Irene Bertozzi, Silvia Betti, Giuseppe Carli, Monica Carpenedo, Daniele Cattaneo, Viviana Cavalca, Alfredo Dragani, Elena Elli, Guido Finazzi, Alessandra Iurlo, Giuseppe Lanzarone, Laura Lissandrini, Francesca Palandri, Chiara Paoli, Alessandro Rambaldi, Paola Ranalli, Maria Luigia Randi, Alessandra Ricco, Elena Rossi, Marco Ruggeri, Giorgina Specchia, Andrea Timillero, Linda Turnu, Nicola Vianelli, Alessandro M Vannucchi, Francesco Rodeghiero, Carlo Patrono
Once-daily (od), low-dose aspirin (75-100 mg) is recommended to reduce the thrombotic risk of patients with essential thrombocytemia (ET). This practice is based on data extrapolated from other high-risk patients and an aspirin trial in polycythemia vera, with the assumption of similar aspirin pharmacodynamics in the two settings. However, the pharmacodynamics of low-dose aspirin is impaired in ET, reflecting accelerated renewal of platelet cyclooxygenase (COX)-1. ARES is a parallel-arm, placebo-controlled, randomized, dose-finding, phase II trial enrolling 300 ET patients to address two main questions...
June 1, 2018: Blood Cancer Journal
Elisa Rumi, Emanuela Sant'Antonio, Emanuela Boveri, Daniela Pietra, Chiara Cavalloni, Elisa Roncoroni, Cesare Astori, Luca Arcaini
The 2016 WHO classification comprises two stages of primary myelofibrosis (PMF): early/prefibrotic primary myelofibrosis (pre-PMF) and overt fibrotic PMF (overt PMF). Diagnostic criteria rely on bone marrow morphology, fibrosis grade (0-1 in pre-PMF, 2-3 in overt PMF) and clinical features (leukoerythroblastosis, anaemia, leucocytosis, increased lactate dehydrogenase and palpable splenomegaly). An accurate differentiation from essential thrombocythemia (ET) is pivotal since the two entities show different clinical presentation and outcome, in terms of survival, leukemic evolution and rates of progression to overt myelofibrosis...
June 4, 2018: Expert Review of Hematology
Richard K Lubberich, Thomas Walenda, Tamme W Goecke, Klaus Strathmann, Susanne Isfort, Tim H Brümmendorf, Steffen Koschmieder, Wolfgang Wagner
BACKGROUND: Myeloproliferative neoplasms (MPN)-such as polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF)-are typically diseases of the elderly caused by acquired somatic mutations. However, it is largely unknown how the malignant clone interferes with normal hematopoiesis. In this study, we analyzed if serum of MPN patients comprises soluble factors that impact on hematopoietic stem and progenitor cells (HPCs). METHODS: CD34+ HPCs were cultured in medium supplemented with serum samples of PV, ET, or MF patients, or healthy controls...
2018: PloS One
Filipa Mousinho, Ana P Azevedo, Tatiana Mendes, Paula Sousa E Santos, Rita Cerqueira, Sónia Matos, Sónia Santos, Sância Ramos, João Faro Viana, Fernando Lima
Myeloproliferative neoplasms (MPNs) are classically divided into BCR RhoGEF and GTPase activating protein (BCR)-ABL proto‑oncogene 1 non‑receptor tyrosine kinase (ABL) positive chronic myeloid leukemia (CML) and BCR‑ABL negative MPNs, including essential thrombocythemia (ET). One of the major diagnostic criteria for ET is the absence of the philadelphia chromosome, thus when present it is almost indicative of CML. ET and CML are considered to be mutually exclusive; however, there are rare situations in which patients with ET present positive BCR‑ABL without the features of CML...
May 17, 2018: Molecular Medicine Reports
Margherita Maffioli, Ester Orlandi, Francesco Passamonti
This review focuses on the management of elderly patients with chronic myeloid leukemia and chronic myeloproliferative neoplasms, including polycythemia vera, essential thrombocythemia and primary myelofibrosis. Median age in these neoplasms is within the 6th decades of age. All new therapies can be done at any age without absolute contraindication. However, the selection of the precise therapy for the single patient is mandatory. For these reasons, an accurate definition of diagnosis and prognostication is necessary...
May 22, 2018: European Journal of Internal Medicine
Yan-Bo Nie, Meng Sun, Colin K He, Man-Kai Ju, Fu-Ling Zhou, San-Yun Wu, Yi Zhou, Li Liu, Hui Shen, Ting-Ting Huang, Pan Liu, Ying Xu, Liang Shao, Xue-Lan Zuo
Essential thrombocythemia (ET) is characterized by thrombotic and hemorrhagic events. The association of clinical characteristics of Chinese ET patients and additional sex combs like 1 (ASXL1) mutations in these patients has remained to be elucidated. In the present study, 72 newly diagnosed Chinese ET patients were enrolled to determine ASXL1 mutations. Mutations in ASXL1, Janus kinase (JAK)2, calreticulin (CALR) and myeloproliferative leukemia (MPL) genes were detected using Sanger sequencing, and data were statistically analyzed...
May 2018: Experimental and Therapeutic Medicine
Francesco Passamonti, Barbara Mora, Daniela Barraco, Margherita Maffioli
PURPOSE OF REVIEW: The purpose of this review is to help doctors in the management of patients with post-polycythemia (PPV) and post-essential thrombocythemia (PET) myelofibrosis (MF) facing diagnostic criteria, prognostication, and treatment possibilities. RECENT FINDINGS: Diagnostic criteria of primary myelofibrosis (PMF) have been recently updated from the WHO classification. A clear-cut distinction between pre-fibrotic and overt PMF has been done. Concerning PPV and PET MF, the criteria come from 2008...
April 30, 2018: Current Hematologic Malignancy Reports
Carmelina Gurrieri, Bradford B Smith, Gregory A Nuttall, Rajiv K Pruthi, Sameh M Said, Mark M Smith
BACKGROUND: Essential thrombocythemia (ET) is a rare myeloproliferative disorder characterized by an unexplained thrombocytosis (>450 x109 /L) and associated vasomotor, thrombotic and hemorrhagic manifestations. While the literature detailing the perioperative management of patients with ET undergoing cardiac surgery is sparse, major perioperative complications have been reported, particularly in poorly controlled patients presenting with platelet counts ≥800 x 109 /L. The purpose of this study was to provide the experience at a large tertiary medical center in managing patients with ET undergoing cardiac surgery, and to summarize the available literature...
April 26, 2018: Annals of Thoracic Surgery
Ifeyinwa E Obiorah, Metin Ozdemirli
Myeloid sarcoma is an extramedullary tumor that consists of myeloblasts or immature myeloid cells. The neoplasm can occur in any part of the body, including the bone, periosteum, lymph nodes, skin, and soft tissue and they may occur de novo or in association with acute myeloid leukemia, myeloproliferative neoplasms and myelodysplastic syndromes. Most cases display a myelomonocytic or pure monoblastic morphology. Tumors with megakaryoblastic differentiation are extremely uncommon and may occur in association with transformation of a myeloproliferative disorder...
April 17, 2018: Hematology/oncology and Stem Cell Therapy
Nikhil Rabade, P G Subramanian, Rohan Kodgule, Goutham Raval, Swapnali Joshi, Shruti Chaudhary, Russel Mascarenhas, Prashant Tembhare, Sumeet Gujral, Nikhil Patkar
Introduction: Over the past decade, we have moved on from a predominantly morphological and clinical classification of myeloproliferative neoplasms (MPN) to a more evolved classification that accounts for the molecular heterogeneity that is unique to this subgroup of hematological malignancies. This usually incorporates mutations in Janus kinase 2 (JAK2), MPL, and calreticulin (CALR) genes. In this manuscript, we report the frequency of these mutations in a cohort of Indian patients at a tertiary cancer center...
April 2018: Indian Journal of Pathology & Microbiology
Jessica L Boklan, Alexandra M Walsh, Michelina C de la Maza, Leon L Su, Francisco A Nizzi, Kristian T Schafernak
A 10-year-old boy presented with spontaneous bruising and was found to have extreme thrombocytosis without neutrophilia/shift to immaturity, basophilia or eosinophilia. While the peripheral blood and bone marrow findings initially suggested essential thrombocythemia, BCR-ABL1 translocation was detected and chronic myeloid leukemia, chronic phase, was diagnosed. Apheresis for platelet depletion was performed as a bridge given the delayed effects of medical therapy.
April 17, 2018: Journal of Pediatric Hematology/oncology
Zhi-Peng He, Yong Wu
Myeloproliferative neoplasm(MPN) is clonal hematopoietic stem cell disorder characterized by abnormal proliferation and expansion of one or more myeloid lineages. BCR-ABL-negative MPN includes polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). The mutations of JAK2, CALR and MPL genes are involved in the pathogenesis of MPN that provided a more complete molecular diagnostic standard for MPN. More and more new mutated genes related to prognosis of MPN were discovered in the past few years, at same time it was found that cytokines were also involved in the genesis and development of MPN...
April 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
Eikan Mishima, Takehiro Suzuki, Yoichi Takeuchi, Kazumasa Seiji, Noriko Fukuhara, Kei Takase, Hideo Harigae, Takaaki Abe, Sadayoshi Ito
Myeloproliferative neoplasms (MPNs) with Janus kinase 2 (JAK2) mutation are associated with a high risk for occlusive vascular diseases. We report 2 cases of renovascular hypertension associated with JAK2 V617F mutation-positive MPNs and provide a literature review. In Case 1, a 63-year-old woman had resistant hypertension, massive proteinuria, and erythrocytosis. Evaluations revealed right renal artery stenosis causing renovascular hypertension and polycythemia vera with JAK2 V617F mutation. Renin-angiotensin system inhibitors and subsequent angioplasty controlled the blood pressure and the proteinuria resolved...
April 2018: Journal of Clinical Hypertension
Francesco Passamonti, Margherita Maffioli
Myeloproliferative neoplasms (MPNs) include essential thrombocythemia, polycythemia vera (PV), and primary myelofibrosis (MF). Phenotype-driver mutations of JAK2 , CALR , and MPL genes are present in MPNs and can be variably combined with additional mutations. Driver mutations entail a constitutive activation of the JAK2/STAT pathway, the key signaling cascade in MPNs. Among JAK2 inhibitors (JAKis), ruxolitinib (RUX) has been approved for the treatment of intermediate and high-risk MF and for PV inadequately controlled by or intolerant of hydroxyurea...
May 31, 2018: Blood
Barbara Mora, Toni Giorgino, Paola Guglielmelli, Elisa Rumi, Margherita Maffioli, Alessandro Rambaldi, Marianna Caramella, Rami Komrokji, Jason Gotlib, Jean-Jacques Kiladjian, Francisco Cervantes, Timothy Devos, Francesca Palandri, Valerio De Stefano, Marco Ruggeri, Richard T Silver, Giulia Benevolo, Francesco Albano, Chiara Cavalloni, Daniela Barraco, Michele Merli, Daniela Pietra, Rosario Casalone, Tiziano Barbui, Giada Rotunno, Mario Cazzola, Alessandro Maria Vannucchi, Francesco Passamonti
No abstract text is available yet for this article.
April 5, 2018: Haematologica
Naery Yang, Sholhui Park, Min Sun Cho, Miae Lee, Ki Sook Hong, Yeung Chul Mun, Chu Myong Seong, Hee Jin Huh, Jungwon Huh
BACKGROUND: This study aimed to determine GATA1 expression levels to better characterize subgroups in BCR/ABL1-negative myeloproliferative neoplasms (MPNs). METHODS: This study enrolled 49 patients diagnosed as having BCR/ABL1-negative MPN on the basis of the 2016 World Health Organization classification : nine polycythemia vera (PV), 17 essential thrombocythemia (ET), 12 prefibrotic primary myelofibrosis (prePMF), and 11 overt primary myelofibrosis (PMF). Relevant clinical and laboratory data were retrieved from the medical records...
July 2018: Annals of Laboratory Medicine
Dian Zhou, Wei Chen, Hai Cheng, Jian-Lin Qiao, Li-Li Zhu, Zhen-Yu Li, Kai-Lin Xu
Essential thrombocythemia (ET) is an uncommon chronic myeloproliferative disorder with no cure. Patients with ET are at risk of different complications, and currently there are no optimal prognostic standards to predict severe post-diagnosis complications such as thrombosis and hemorrhage. In this study, we retrospectively analyzed the full set of clinical data from 150 Chinese patients with ET enrolled from 2013 to 2016. We discovered that neutrophil-to-lymphocyte ratio (NLR), along with other known clinical parameters such as age, leukocyte count, incidence of thrombotic events is higher in patients with JAK2 V617F mutation...
March 27, 2018: Leukemia Research
Veronika Buxhofer-Ausch, Michael Steurer, Siegfried Sormann, Ernst Schloegl, Wolfgang Schimetta, Bettina Gisslinger, Martin Schalling, Maria Theresa Krauth, Jürgen Thiele, Reinhard Ruckser, Günther Gastl, Heinz Gisslinger
OBJECTIVES: Risk of thrombosis is significantly enhanced by both elevated platelet (PLT) and white blood cell (WBC) counts according to a retrospective analysis of a large anagrelide- registry in thrombocythemic MPN patients. We were interested in the impact of elevated WBC counts on thrombosis risk in patients where PLT counts were reduced below the calculated cut off of 574.5 G/L by treatment with anagrelide. METHODS: Cox regression analysis and Kaplan Meier plot were applied on all patients in the registry with optimized PLT counts...
March 30, 2018: European Journal of Haematology
Eran Zimran, Ronald Hoffman, Marina Kremyanskaya
The Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs) including polycythemia vera, essential thrombocythemia and primary myelofibrosis are clonal hematological malignancies that originate at the level of the hematopoietic stem cell, and are characterized by excessive proliferation of cells belonging to one or more of the myeloid lineages. Central to the pathogenesis of the MPNs is constitutive activation of the JAK/STAT signaling pathway due to a family of driver mutations affecting JAK2, CALR or MPL...
June 2018: Expert Review of Anticancer Therapy
J Marcoval
No abstract text is available yet for this article.
March 19, 2018: Actas Dermo-sifiliográficas
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