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Polycythemia vera

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https://www.readbyqxmd.com/read/29780645/sustained-regression-of-hydroxycarbamide-induced-actinic-keratoses-after-switching-to-anagrelide
#1
Georgios Gaitanis, Dora Gougopoulou, Eleni Kapsali, Ioannis D Bassukas
Hydroxycarbamide (HC) is the first-line treatment for certain myeloproliferative neoplasms, such as polycythemia vera and essential thrombocytosis (ET). In a subset of these patients long-term treatment with HC can result in the development of confluent actinic keratoses (AK) followed by invasive keratinocytic carcinomas ("squamous dysplasia"), preferentially on sun-exposed skin. Discontinuation or dose reduction of HC may result in partial improvement. A 59-year-old farmer after 14 years on HC (2 gr/d) and acetylsalicylic acid (100 mg/d) for ET, was referred for numerous, hyperkeratotic AK on face, scalp, and hands that could not be controlled with repeated ( N = 15) cryosurgery sessions in the previous 3 years...
2018: Case Reports in Dermatological Medicine
https://www.readbyqxmd.com/read/29772037/a-review-of-fabry-disease
#2
A Cinats, E Heck
The class of medications known as Janus kinase inhibitors block cytokine-mediated signaling via the Janus kinase-signal transducer and activator of transcription (JAK-STAT) pathway, which plays an important role in immunoregulation and normal cell growth. This class includes the drugs tofacitinib, approved for the treatment of rheumatoid arthritis, and ruxolitinib, approved for the treatment of myelofibrosis and polycythemia rubra vera. The most common adverse events (AEs) reported in patients taking tofacitinib are infections, whereas the most common AEs in patients taking ruxolitinib are anemia and thrombocytopenia...
May 2018: Skin Therapy Letter
https://www.readbyqxmd.com/read/29765288/serum-ferritin-as-a-biomarker-of-polycythemia-vera
#3
EDITORIAL
Stephen E Langabeer
No abstract text is available yet for this article.
April 2018: EJIFCC
https://www.readbyqxmd.com/read/29728092/cytoreductive-treatment-patterns-among-us-veterans-with-polycythemia-vera
#4
Shreekant Parasuraman, Jingbo Yu, Dilan Paranagama, Sulena Shrestha, Li Wang, Onur Baser, Robyn Scherber
BACKGROUND: Polycythemia vera (PV) is a myeloproliferative neoplasm associated with increased thrombotic and cardiovascular risk, which are key contributors to patient morbidity and mortality. The Veterans Health Administration (VHA) is the largest integrative health network in the United States. Available data concerning patients with PV in this population are limited. METHODS: This retrospective observational study evaluated the characteristics, management, and outcomes of patients with PV in the VHA Medical SAS® Dataset (October 1, 2005, to September 30, 2012)...
May 4, 2018: BMC Cancer
https://www.readbyqxmd.com/read/29721250/congenital-methemoglobinemia-misdiagnosed-as-polycythemia-vera-case-report-and-review-of-literature
#5
Dina Sameh Soliman, Mohamed Yassin
Methemoglobinemia is a rare overlooked differential diagnosis in patients presented with cyanosis and dyspnea unrelated to cardiopulmonary causes. Our patient is 29 year old Indian non-smoker male, his story started 6 months prior to presentation to our center when he had generalized fatigue and discoloration of hands. He presented with persistent polycythemia with elevated hemoglobin level. The patient was misdiagnosed in another center as polycythemia and treated with Imatinib. The diagnosis of PV was revisited and ruled out in view of negative JAK2, normal erythropoietin level and absence of features of panmyelosis...
March 2, 2018: Hematology Reports
https://www.readbyqxmd.com/read/29717365/the-who-diagnostic-criteria-for-polycythemia-vera-role-of-red-cell-mass-versus-hemoglobin-hematocrit-level-and-morphology
#6
Ljubomir Jakovic, Mirjana Gotic, Heinz Gisslinger, Ivan Soldatovic, Dijana Sefer, Mila Tirnanic, Danijela Lekovic, Maja Perunicic Jovanovic, Martin Schalling, Bettina Gisslinger, Christine Beham-Schmid, Ingrid Simonitsch-Klupp, Jürgen Thiele
Regarding diagnosis of polycythemia vera (PV), discussion persists about hemoglobin (Hb) and/or hematocrit (Hct) threshold values as surrogate markers for red cell mass (RCM) and the diagnostic impact of bone marrow (BM) morphology. We performed a retrospective study on 290 patients with PV (151 males, 139 females; median age 65 years) presenting with characteristic BM features (initial biopsies, centralized evaluation) and endogenous erythroid colony (EEC) formations. This cohort included (1) a group of 229 patients when following the 2008 versus 256 patients diagnosed according to the 2016 World Health Organization (WHO) guidelines, all presented with increased RCM; (2) masked PV patients with low Hb (n = 143)/Hct (n = 45) recruited from the 2008 WHO cohort; (3) a cohort of 17 PV patients with elevated diagnostic Hb/Hct levels but low RCM; and (4) nine PV patients with increased RCM, opposing low Hb/Hct values...
May 1, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29685167/neutropenia-caused-by-hairy-cell-leukemia-in-a-patient-with-myelofibrosis-secondary-to-polycythemia-vera-a-case-report
#7
Andreas Hanssønn Habberstad, Hoa Thi Tuyet Tran, Ulla Randen, Signe Spetalen, Ingunn Dybedal, Geir E Tjønnfjord, Anders Erik Astrup Dahm
BACKGROUND: Polycythemia vera is a myeloproliferative disease that sometimes evolves to myelofibrosis, causing splenomegaly and neutropenia. In this case report, we describe a patient with polycythemia vera and unexplained neutropenia who later turned out to also have hairy cell leukemia. CASE PRESENTATION: A middle-aged Caucasian man with polycythemia vera presented to our hospital with chronic mouth ulcers. Later he developed leukopenia and pancytopenia. Bone marrow biopsies showed fibrosis...
April 24, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29683036/assessment-of-endothelial-dysfunction-with-flow-mediated-dilatation-in-myeloproliferative-disorders
#8
Abdulkerim Yildiz, Melike Güryildirim, Mehmet Sezgin Pepeler, Merve Yazol, Suna Özhan Oktar, Kadir Acar
BACKGROUND: Thrombosis is the most important cardiovascular complication of classical myeloproliferative disorders (MPDs). Endothelial dysfunction (ED) is known to play a major role in the mechanism of thrombophilia in MPDs. METHODS: Endothelial dysfunction and its associations with other parameters were investigated. A total of 18 patients with polycythemia vera (PV), 24 with essential thrombocytosis (ET), 7 with primary myelofibrosis (PMF), and 30 healthy patients as a control group were included in the study...
January 1, 2018: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29682367/myelodysplastic-syndrome-acute-myeloid-leukemia-arising-in-idiopathic-erythrocytosis
#9
Stephen E Langabeer, Eibhlin Conneally, Catherine M Flynn
The term "idiopathic erythrocytosis (IE)" is applied to those cases where a causal clinical or pathological event cannot be elucidated and likely reflects a spectrum of underlying medical and molecular abnormalities. The clinical course of a patient with IE is described manifesting as a persistent erythrocytosis with a low serum erythropoietin level, mild eosinophilia, and with evidence of a thrombotic event. The patient subsequently developed a myelodysplasic syndrome (MDS) and acute myeloid leukemia (AML), an event not observed in erythrocytosis patients other than those with polycythemia vera (PV)...
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/29676359/molecular-genetics-of-bcr-abl1-negative-myeloproliferative-neoplasms-in-india
#10
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
https://www.readbyqxmd.com/read/29665937/-current-understanding-of-myeloproliferative-neoplasm-related-gene-mutations-and-cytokine-review
#11
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
https://www.readbyqxmd.com/read/29660912/increased-plasma-viscosity-in-plasma-cell-dyscrasia-and-whole-blood-viscosity-in-polycythemia-vera
#12
Dong Woo Shin, Ja-Yoon Gu, Jun Sik Kim, Jae-Seol Jung, Dong-Yeop Shin, Youngil Koh, Inho Kim, Hyun Kyung Kim
BACKGROUND: Although hyperviscosity syndrome in plasma cell dyscrasia (PCD) and thrombosis in myeloproliferative neoplasm (MPN) are major causes of morbidity and mortality, blood viscosity measurements are often underutilized. OBJECTIVE: This study aimed to characterize whether whole blood viscosity (WBV) or plasma viscosity (PV) could be predictive of hyperviscosity syndrome in PCD and could be elevated in subgroups of MPN. METHODS: A total of 75 patients with hematologic diseases: PCD (n = 26), MPN (n = 25) including polycythemia vera (P...
April 13, 2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29656438/renovascular-hypertension-associated-with-jak2-v617f-positive-myeloproliferative-neoplasms-treated-with-angioplasty-2-cases-and-literature-review
#13
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 14, 2018: Journal of Clinical Hypertension
https://www.readbyqxmd.com/read/29650801/the-role-of-jak2-inhibitors-in-mpn-seven-years-after-approval
#14
Francesco Passamonti, Margherita Maffioli
Myeloproliferative neoplasms (MPNs) include essential thrombocythemia, polycythemia vera (PV) and primary myelofibrosis (PMF). 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 myelofibrosis (MF) and of PV inadequately controlled by or intolerant of hydroxyurea...
April 12, 2018: Blood
https://www.readbyqxmd.com/read/29622658/value-of-cytogenetic-abnormalities-in-post-polycythemia-vera-and-post-essential-thrombocythemia-myelofibrosis-a-study-of-the-mysec-project
#15
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
https://www.readbyqxmd.com/read/29611379/gata1-expression-in-bcr-abl1-negative-myeloproliferative-neoplasms
#16
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
https://www.readbyqxmd.com/read/29611054/molecular-evidence-of-jak2-p-v617f-mutated-pure-erythroid-leukemia-arising-from-polycythemia-vera
#17
Alisha D Ware, Jacqueline Birkness, Amy S Duffield, Christopher D Gocke
No abstract text is available yet for this article.
April 2, 2018: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29599206/impact-of-hydroxycarbamide-and-interferon-%C3%AE-on-red-cell-adhesion-and-membrane-protein-expression-in-polycythemia-vera
#18
Mégane Brusson, Maria De Grandis, Sylvie Cochet, Sylvain Bigot, Mickaël Marin, Marjorie Leduc, François Guillonneau, Patrick Mayeux, Thierry Peyrard, Christine Chomienne, Caroline Le Van Kim, Bruno Cassinat, Jean-Jacques Kiladjian, Wassim El Nemer
Polycythemia vera is a chronic myeloproliferative neoplasm characterized by the JAK2V617F mutation, elevated blood cell counts and a high risk of thrombosis. Although the red cell lineage is primarily affected by JAK2V617F, the impact of mutated JAK2 on circulating red blood cells is poorly documented. Recently, we showed that in polycythemia vera erythrocytes had abnormal expression of several proteins including Lu/BCAM adhesion molecule and proteins from the endoplasmic reticulum, mainly calreticulin and calnexin...
March 29, 2018: Haematologica
https://www.readbyqxmd.com/read/29575945/current-approaches-to-challenging-scenarios-in-myeloproliferative-neoplasms
#19
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
https://www.readbyqxmd.com/read/29556018/pegylated-interferon-alfa-2a-and-hydroxyurea-in-polycythemia-vera-and-essential-thrombocythemia-differential-cellular-and-molecular-responses
#20
Tsewang Tashi, Sabina Swierczek, Soo Jin Kim, Mohamed E Salama, Jihyun Song, Nahla Heikal, Katherine Y King, Kim Hickman, Scott Litton, Josef T Prchal
No abstract text is available yet for this article.
February 28, 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
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