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Polycythemia vera

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https://www.readbyqxmd.com/read/28712096/myeloproliferative-neoplasms-translating-new-discoveries-into-better-outcomes-better-quality-of-life
#1
REVIEW
Leslie Padrnos, Ruben A Mesa
Despite the identification of JAK mutations and the development of targeted inhibitors, there remain significant unmet needs for patients with myeloproliferative neoplasms. Identification of the myeloproliferative neoplasm populations not currently benefiting from JAK inhibitor therapy highlights the therapeutic deficits still present in this heterogeneous stem cell malignancy. While JAK inhibition has provided significant benefits for patients with intermediate-2 or high-risk myelofibrosis and in patients with polycythemia vera in the second-line setting, JAK inhibitor monotherapy is not approved and not appropriate for all patients with myeloproliferative neoplasms...
July 15, 2017: Oncology (Williston Park, NY)
https://www.readbyqxmd.com/read/28702412/megakaryocytic-morphology-in-janus-kinase-2-v617f-positive-myeloproliferative-neoplasm
#2
Shuchi Ghai, Sharada Rai
CONTEXT: Alterations in megakaryocyte morphology are the hallmark of myeloproliferative neoplasms (MPNs). These neoplasm are also associated with Janus kinase 2 (JAK2) V617F mutation in nearly 95% patients with polycythemia vera (PV), 40% patients of essential thrombocythemia (ET) and 50% patients of myelofibrosis (MF). The utility of megakaryocyte morphology in these disorders in correlation with JAK2 V617F remains unresolved. AIMS: The aim of the study was to assess the morphology of megakaryocytes in bone marrow aspirates (BMAs) and bone marrow biopsies of patients of BCR-ABL negative MPNs with JAK2 V617F mutation...
April 2017: South Asian Journal of Cancer
https://www.readbyqxmd.com/read/28702402/in-the-era-of-next-generation-sequencing-is-the-cytogenetic-analysis-still-important-in-polycythemia-vera
#3
Prakas Kumar Mandal, S Kartthik
No abstract text is available yet for this article.
April 2017: South Asian Journal of Cancer
https://www.readbyqxmd.com/read/28700486/progression-of-primary-myelofibrosis-to-polycythemia-vera-a-case-report
#4
Yan Guo, Wenwei Xu, Lin Dong, Ning Huang, Kehong Bi
RATIONALE: This case report describes the progression of primary myelofibrosis (PMF) to polycythemia vera (PV), and discuss its potential mechanisms. PATIENT CONCERNS: The patient was admitted because of abdominal discomfort and enlarged spleen for 19 months. DIAGNOSIS: A case of PMF progressed to PV was retrospectively analyzed. There were 19 months between the diagnosis of PMF and PV. The JAK2 V617F mutation was positive before and after the diagnosis of PV; however, new chromosomal abnormalities were detected during the progression...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28699191/a-re-appraisal-of-the-benefit-risk-profile-of-hydroxyurea-in-polycythemia-vera-a-propensity-matched-study
#5
Tiziano Barbui, Alessandro M Vannucchi, Guido Finazzi, Maria Chiara Finazzi, Arianna Masciulli, Alessandra Carobbio, Arianna Ghirardi, Gianni Tognoni
The use of Hydroxyurea (HU) as first line therapy in polycythemia vera (PV) has been criticized because no solid demonstration that this drug prevents thrombosis or prolongs survival has been so far produced. Here we present the outcomes of a large cohort of patients with PV included in the European Collaborative Low-dose Aspirin (ECLAP) study. We selected 1,042 patients who, during the follow-up, had received only phlebotomy (PHL) or HU to maintain the hematocrit level<45%. To assure comparability, we conducted a propensity score matching analysis...
July 11, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28698883/anti-platelet-factor-4-heparin-antibody-formation-occurs-endogenously-and-at-unexpected-high-frequency-in-polycythemia-vera
#6
Sara C Meyer, Eva Steinmann, Thomas Lehmann, Patricia Muesser, Jakob R Passweg, Radek C Skoda, Dimitrios A Tsakiris
BACKGROUND: Myeloproliferative neoplasms (MPN) encounter thromboses due to multiple known risk factors. Heparin-induced thrombocytopenia (HIT) is a thrombotic syndrome mediated by anti-platelet factor 4 (PF4)/heparin antibodies with undetermined significance for thrombosis in MPN. We hypothesized that anti-PF4/heparin Ab might occur in MPN and promote thrombosis. METHODS: Anti-PF4/heparin antibodies were analyzed in 127 MPN patients including 76 PV and 51 ET. Screening, validation testing, and isotype testing of anti-PF4/heparin Ab were correlated with disease characteristics...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28688466/leukemic-transformation-in-myeloproliferative-neoplasms-a-literature-review-on-risk-characteristics-and-outcome
#7
REVIEW
Meera Yogarajah, Ayalew Tefferi
Myeloproliferative neoplasms (MPNs) operationally include essential thrombocythemia, polycythemia vera, primary myelofibrosis (PMF), and prefibrotic PMF. All 4 MPN variants might progress into blast-phase disease (MPN-BP). For essential thrombocythemia, reported risk factors for leukemic transformation include advanced age, extreme thrombocytosis, anemia, leukocytosis, and sequence variants/mutations involving TP53 and EZH2 (for expansion of gene symbols, see www.genenames.org); for polycythemia vera, advanced age, leukocytosis, abnormal karyotype, mutations involving SRSF2 and IDH2, and treatment with pipobroman, chlorambucil, or P32; and for PMF, increased blast percentage, thrombocytopenia, abnormal karyotype, triple-negative driver mutational status, and sequence variants/mutations involving SRSF2, RUNX1, CEBPA, and SH2B3...
July 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28685840/european-leukemianet-study-on-the-reproducibility-of-bone-marrow-features-in-masked-polycythemia-vera-and-differentiation-from-essential-thrombocythemia
#8
Hans Michael Kvasnicka, Attilio Orazi, Juergen Thiele, Giovanni Barosi, Carlos E Bueso-Ramos, Alessandro M Vannucchi, Robert P Hasserjian, Jean-Jacques Kiladjian, Umberto Gianelli, Richard Silver, Tariq I Mughal, Tiziano Barbui
The purpose of the study was to assess consensus and interobserver agreement among an international panel of six hematopathologists regarding characterization and reproducibility of bone marrow (BM) histologic features used to diagnose early stage myeloproliferative neoplasms, in particular differentiation of so-called masked/prodromal polycythemia vera (mPV) from JAK2-mutated essential thrombocythemia (ET). The six members of the hematopathology panel evaluated 98 BM specimens independently and in a blinded fashion without knowledge of clinical data...
July 7, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28680586/pulmonary-extra-medullary-hematopoiesis-and-pulmonary-hypertension-from-underlying-polycythemia-vera-a-case-series
#9
Inderjit Singh, Geoffrey Mikita, Daniel Green, Cristobal Risquez, Abraham Sanders
Myeloproliferative neoplasia (MPN)-associated pulmonary hypertension (PH) is included in group five of the most recent clinical classification of PH.(1) The MPNs are a heterogeneous group of disorders that includes disorders with primary expression of a myeloid phenotype and disorders characterized by expression of the Janus Kinase 2 (JAK2) mutation, p.V617F. The latter includes essential thrombocytosis, polycythemia vera, and idiopathic myelofibrosis.(2) Pulmonary extra-medullary hematopoiesis (EMH) refers to the presence of hematopoietic precursor cells in the lung...
March 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28674362/the-amelioration-of-myelofibrosis-with-thrombocytopenia-by-a-jak1-2-inhibitor-ruxolitinib-in-a-post-polycythemia-vera-myelofibrosis-patient-with-a-jak2-exon-12-mutation
#10
Kazuhiko Ikeda, Koki Ueda, Takahiro Sano, Kazuei Ogawa, Takayuki Ikezoe, Yuko Hashimoto, Soji Morishita, Norio Komatsu, Hitoshi Ohto, Yasuchika Takeishi
Less than 5% of patients with polycythemia vera (PV) show JAK2 exon 12 mutations. Although PV patients with JAK2 exon 12 mutations are known to develop post-PV myelofibrosis (MF) as well as PV with JAK2V617F, the role of JAK inhibitors in post-PV MF patients with JAK2 exon 12 mutations remains unknown. We describe how treatment with a JAK1/2 inhibitor, ruxolitinib, led to the rapid amelioration of marrow fibrosis, erythrocytosis and thrombocytopenia in a 77-year-old man with post-PV MF who carried a JAK2 exon 12 mutation (JAK2H538QK539L)...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28673391/the-development-and-use-of-janus-kinase-2-inhibitors-for-the-treatment-of-myeloproliferative-neoplasms
#11
REVIEW
Gabriela S Hobbs, Sarah Rozelle, Ann Mullally
Following the discovery of the JAK2V617F mutation, Janus kinase (JAK) 2 inhibitors were developed as rationally designed therapy in myeloproliferative neoplasms (MPNs). Although JAK2 inhibitors have clinical efficacy in MPN, they are not clonally selective for the JAK2V617F-mutant cells. Because activated JAK-signal transducer and activator of transcription (STAT) signaling is a common feature of MPN, JAK2 inhibitors are efficacious regardless of the specific MPN phenotypic driver mutation. The Food and Drug Administration approved the JAK1/JAK2 inhibitor, ruxolitinib, for the treatment of myelofibrosis and polycythemia vera...
August 2017: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/28669446/inflammation-and-myeloproliferative-neoplasms
#12
REVIEW
Federico Lussana, Alessandro Rambaldi
Myeloproliferative neoplasms (MPN) include three main entities: Polycythemia Vera (PV), Essential Thrombocythemia (ET), and Myelofibrosis (MF). MPN represent a unique model of the relationship between the clonal development of a hematologic malignancy and chronic inflammation. The neoplastic clone is the main driver of this inflammatory reaction as demonstrated by the curative effect of allogeneic stem cell transplantation which leads not only to a complete restore of the hematopoiesis, but also to regression of bone marrow fibrosis...
June 29, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28668372/-two-cases-of-myeloid-sarcomas-with-review-of-literature
#13
P Mottaz, F Becot-Bretez, R Jeanneau, G Vignon, A Bertin, F Carrere, P F Augereau, P Aucher, F Lellouche
INTRODUCTION: Myeloid sarcomas are uncommon proliferations of immature myeloid cells occurring in any extramedullary organ. We report here two cases of myeloid sarcomas in patients with, respectively, a polycythemia vera and a myelodysplastic syndrome. CASE REPORTS: The first is an 81-year-old woman who presented with osteolytic lesions. Diagnosis has been highlighted using anatomopathological study after bone marrow biopsy, but it was delayed because of a very localized basin lesion and few positive myeloid markers...
June 28, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28660085/dermatomyositis-associated-with-myelofibrosis-following-polycythemia-vera
#14
Naomi Fei, Sarah Sofka
Dermatomyositis (DM) is a unique inflammatory myopathy with clinical findings of proximal muscle weakness, characteristic rash, and elevated muscle enzymes. The association of DM and malignancy, most commonly adenocarcinoma, is well known. There have been few case reports of primary myelofibrosis associated with DM. We present the case of a 69-year-old male with a history of polycythemia vera (PV) who developed proximal muscle weakness, dysphagia, and rash. He was found to have elevated creatinine kinase and skin biopsy was consistent with DM...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28655032/age-related-macular-degeneration-in-patients-with-chronic-myeloproliferative-neoplasms
#15
Marie Bak, Torben Lykke Sørensen, Esben Meulengracht Flachs, Ann-Dorthe Zwisler, Knud Juel, Henrik Frederiksen, Hans Carl Hasselbalch
Importance: It has been suggested that systemic inflammation increases the risk of age-related macular degeneration (AMD). Given that chronic immune modulation is present in patients with myeloproliferative neoplasms (MPNs), the risk of AMD in these patients may be increased. Objective: To compare the risk of AMD in patients with MPNs with the risk of AMD in matched controls from the general population. Design, Setting, and Participants: A nationwide population-based cohort study using Danish registers was conducted of all patients in Denmark who received a diagnosis between January 1, 1994, and December 31, 2013, of essential thrombocythemia, polycythemia vera, myelofibrosis, or unclassifiable MPNs...
June 22, 2017: JAMA Ophthalmology
https://www.readbyqxmd.com/read/28622623/a-phase-2-study-of-momelotinib-a-potent-jak1-and-jak2-inhibitor-in-patients-with-polycythemia-vera-or-essential-thrombocythemia
#16
Srdan Verstovsek, Stephane Courby, Martin Griesshammer, Ruben A Mesa, Carrie Baker Brachmann, Jun Kawashima, Julia D Maltzman, Lixin Shao, Yan Xin, Daniel Huang, Ashish Bajel
Momelotinib is a potent inhibitor of JAK1 and JAK2 that demonstrated efficacy in patients with primary and secondary myelofibrosis. This phase 2, open-label, randomized study evaluated the efficacy and safety of oral once-daily momelotinib (100mg and 200mg) for the treatment of polycythemia vera (PV) and essential thrombocythemia (ET). The primary endpoint for PV was overall response rate (ORR), defined as the proportion of patients with hematocrit <45%, white blood cell count <10×10(9)/L, platelet count ≤400×10(9)/L, and resolution of palpable splenomegaly, each lasting ≥4 weeks...
May 30, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28601551/patients-with-post-essential-thrombocythemia-and-post-polycythemia-vera-differ-from-patients-with-primary-myelofibrosis
#17
Lucia Masarova, Prithviraj Bose, Naval Daver, Naveen Pemmaraju, Kate J Newberry, Taghi Manshouri, Jorge Cortes, Hagop M Kantarjian, Srdan Verstovsek
Prognostic scoring systems for primary myelofibrosis (PMF) are not accurate in patients with post-essential thrombocythemia and post-polycythemia vera myelofibrosis (PET-MF; PPV-MF). Given the paucity of data describing the clinical characteristics, disease course and outcomes of these patients, we sought to describe and compare the clinical characteristics and outcomes of 755 patients with PMF, 181 with PPV-MF, and 163 with PET-MF referred to our institution between 1984 and 2013. The median follow-up was 31 months, and 56% (n=616) patients had died...
June 2, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28596255/thrombotic-thrombocytopenic-purpura-associated-with-pegylated-interferon-alfa-2a-use-in-a-patient-with-polycythemia-vera
#18
Radhika Gangaraju, Soo J Kim, Jing-Fei Dong, Sabina Swierczek, Josef T Prchal
Pegylated interferon alfa-2a (pegIFNa) is being increasingly used for treatment of myeloproliferative neoplasms; however, its side effects, including autoimmune complications, are not unusual. We report on a 47-year-old woman with polycythemia vera (PV) treated with pegIFNa and in complete hematologic remission who developed thrombotic thrombocytopenic purpura (TTP). To our knowledge, thrombotic microangiopathy has been reported as a side effect of interferon (IFN) use in patients with hepatitis and chronic myeloid leukemia, but not in those with PV...
June 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/28589759/leukemic-transformation-in-patients-with-myeloproliferative-neoplasms-a-population-based-retrospective-study
#19
Rajesh Shrestha, Smith Giri, James O Armitage, Vijaya Raj Bhatt
AIM: This study determined the epidemiology of developing leukemic transformation in patients with myeloproliferative neoplasms (MPN). METHODS: We utilized the Surveillance, Epidemiology and End Results 13 database to identify 83 cases of leukemic transformation in MPN (n = 9335). RESULTS: The 5-year cumulative incidence of leukemic transformation was higher in male versus female (2.17 vs 1.09%, p < 0.001), and in myelofibrosis (2.19%; 95% CI: 1...
June 7, 2017: Future Oncology
https://www.readbyqxmd.com/read/28585070/thromboses-and-hemorrhages-are-common-in-mpn-patients-with-high-jak2v617f-allele-burden
#20
Irene Bertozzi, Giulia Bogoni, Giacomo Biagetti, Elena Duner, Anna Maria Lombardi, Fabrizio Fabris, Maria Luigia Randi
The most common causes of morbidity and mortality in myeloproliferative neoplasms (MPN) are thrombotic and hemorrhagic complications. The JAK2V617F mutation, commonly found in MPN, correlates with several clinical and laboratory characteristics even if the relevance of JAK2V617F allele burden in the natural history of these diseases is unclear. In this study we searched, a relation between thrombotic and hemorrhagic complications and JAK2V617F allele burden level in MPN patients. We evaluated 253 consecutive MPN [121 essential thrombocythemia (ET), 124 polycythemia vera (PV), and 8 primary myelofibrosis (PMF)] patients in whom the JAK2V617F allele burden was available, all studied and followed (median 8...
June 6, 2017: Annals of Hematology
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