1,488 results match your criteria Polycythemia Secondary


The role of fedratinib for the treatment of patients with primary or secondary myelofibrosis.

Ther Adv Hematol 2020 15;11:2040620720925201. Epub 2020 May 15.

Division of Hematology and Oncology, UT Health San Antonio MD Anderson, Mail Code 8026, 6th Floor Urschel Tower, 7979 Wurzbach Rd, San Antonio, TX 78229-3900.

Myelofibrosis (MF) is a chronic myeloid neoplasm characterized by either primary myelofibrosis, or secondary MF following essential thrombocythemia or polycythemia vera. Historically, therapy has been symptom directed; however, in 2011, the first janus kinase inhibitor (JAK-i) - ruxolitinib - was approved for treatment. This medication was found to be effective in reduction of symptom burden and spleen size; however, the median duration of response is about 3 years. Read More

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http://dx.doi.org/10.1177/2040620720925201DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7232117PMC

Retrospective evaluation of 85 patients with urea cycle disorders: one center experience, three new mutations.

J Pediatr Endocrinol Metab 2020 May 24. Epub 2020 May 24.

Hacettepe University İhsan Doğramacı Children's Hospital, Ankara, Turkey.

Objectives Urea cycle disorders (UCDs) are rare hereditary diseases. This study was conducted to help identify the characteristics of UCDs in Turkey. Methods The primary outcome was to determine patient characteristics. Read More

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http://dx.doi.org/10.1515/jpem-2019-0413DOI Listing

Differential cytokine network profile in polycythemia vera and secondary polycythemia.

Sci Rep 2020 Apr 27;10(1):7032. Epub 2020 Apr 27.

Department of Clinical Analyses, Toxicology and Food Sciences, School of Pharmaceutical Sciences of Ribeirão Preto, University of São Paulo - USP, Ribeirão Preto - SP, Brazil.

Polycythemia vera (PV) is a clonal disorder resulting from neoplastic transformation of hematopoietic stem cells, while secondary polycythemia (SP) is a disease characterized by increased absolute red blood cell mass caused by stimulation of red blood cell production. Although the physiopathology of SP and PV is distinct, patients with these diseases share similar symptoms. The early differential diagnosis may improve the quality of life and decrease the disease burden in PV patients, as well as enable curative treatment for SP patients. Read More

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http://dx.doi.org/10.1038/s41598-020-63680-7DOI Listing

Type B Interrupted Aortic Arch with a Patent Ductus Arteriosus in an Adult Presenting with Secondary Polycythaemia.

Eur J Case Rep Intern Med 2020 26;7(4):001511. Epub 2020 Feb 26.

Department of Radiology & Imaging, Queen Elizabeth Hospital, Hong Kong, Hong Kong.

Interrupted aortic arch (IAA) is an extremely rare congenital cyanotic heart disease characterized by complete disruption between the ascending and descending aorta. A patent ductus arteriosus (PDA) or other collateral pathways provide blood flow to the distal descending aorta. Mortality is extremely high at early infancy, particularly after closure of the ductus arteriosus. Read More

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http://dx.doi.org/10.12890/2020_001511DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7162564PMC
February 2020

Obstructive Sleep Apnea Is Associated With Polycythemia in Hypogonadal Men on Testosterone Replacement Therapy.

J Sex Med 2020 Apr 16. Epub 2020 Apr 16.

Department of Urology, Glickman Urological and Kidney Institute, Cleveland Clinic Foundation, Cleveland, OH, USA.

Background: Polycythemia (erythrocytosis) is a known side effect of testosterone (T) replacement therapy (TRT) and appears to correlate with maximum T levels. There is also a well-established association between obstructive sleep apnea (OSA) and the development of polycythemia, which confers additional long-term cardiovascular morbidity. Synergy between TRT and OSA in the development of polycythemia remains poorly understood. Read More

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http://dx.doi.org/10.1016/j.jsxm.2020.03.006DOI Listing

Treatment and outcome in 370 cases with spontaneous or post-laser twin anemia polycythemia sequence managed in 17 different fetal therapy centers.

Ultrasound Obstet Gynecol 2020 Apr 14. Epub 2020 Apr 14.

Department of Pediatrics, Division of Neonatology, Leiden University Medical Center, Leiden, The Netherlands.

Objective: to investigate antenatal management and outcome in a large international cohort of spontaneous and post-laser twin anemia polycythemia sequence (TAPS).

Methods: Data of monochorionic twins diagnosed antenatally with TAPS collected in the TAPS Registry between 2014-2019 were included in this study. Antenatal diagnosis of TAPS was based on middle cerebral artery peak systolic velocity (MCA-PSV) > 1. Read More

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http://dx.doi.org/10.1002/uog.22042DOI Listing

The development of T-cell malignancies in patients with pre-existing myeloproliferative neoplasms: a report of three cases.

Ecancermedicalscience 2020 17;14:1011. Epub 2020 Feb 17.

Houston Methodist Cancer Center, 6445 Main Street, Outpatient Center, 24th Floor, Houston, TX 77030, USA.

Secondary acute myeloid leukaemia complicating the natural disease course of pre-existing Philadelphia chromosome-negative myeloproliferative neoplasms (PN-MPN) is well documented and associated with treatment challenges and significant morbidity. The incidence of a T-cell malignancy developing in patients with pre-existing PN-MPN is uncommon, with one case documented in the literature. We present two cases of angioimmunoblastic T-cell lymphoma (AITL) and one case of T-cell acute lymphoblastic leukaemia (T-ALL) that developed in patients with essential thrombocythemia (ET) and primary myelofibrosis (PMF), respectively. Read More

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http://dx.doi.org/10.3332/ecancer.2020.1011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7105335PMC
February 2020

Prevalence of polycythaemia with different formulations of testosterone therapy in transmasculine individuals.

Intern Med J 2020 Apr 1. Epub 2020 Apr 1.

Department of Endocrinology, Austin Health, Heidelberg, Victoria, Australia.

Background: Masculinising hormone therapy with testosterone is used to align an individual's physical characteristics with their gender identity. Testosterone therapy is typically administered via intramuscular or transdermal routes and polycythaemia is the most common adverse event.

Aims: To compare the risk of polycythaemia with different formulations of testosterone therapy in transmasculine individuals. Read More

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http://dx.doi.org/10.1111/imj.14839DOI Listing

Targeted Sequencing Improves DIPSS-Plus Prognostic Scoring in Myelofibrosis Patients Undergoing Allogeneic Transplantation.

Biol Blood Marrow Transplant 2020 Mar 16. Epub 2020 Mar 16.

Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, Washington; Department of Medicine, University of Washington, Seattle, Washington; Seattle Cancer Care Alliance, Seattle, Washington; Department of Pathology, University of Washington, Seattle, Washington. Electronic address:

Primary myelofibrosis (MF) and secondary MF developing after polycythemia vera or essential thrombocythemia are clonal disorders of hematopoiesis. Currently the sole therapy offering the potential of cure is hematopoietic cell transplantation (HCT). Several risk classification systems including clinical, hematologic, and mutational parameters have been proposed. Read More

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http://dx.doi.org/10.1016/j.bbmt.2020.03.007DOI Listing

Dissecting Primary Erythrocytosis Among Polycythemia Patients Referred to an Indian Armed Forces Hospital.

Indian J Hematol Blood Transfus 2020 Jan 8;36(1):187-191. Epub 2019 Jul 8.

2Department of Internal Medicine (Clinical Hematology Division), Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

Referrals for evaluation of polycythemia cases have increased since the hemoglobin (Hb) thresholds for diagnosis of Polycythemia Vera (PV) have been lowered by WHO. The current study enrolled patients of age > 18 years from the Indian Armed Forces or their family members with polycythemia from November 2016 to October 2018. After exclusion of secondary causes, 49 patients were diagnosed as Primary Erythrocytosis (PE). Read More

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http://dx.doi.org/10.1007/s12288-019-01155-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7042446PMC
January 2020

miR-146a rs2431697 identifies myeloproliferative neoplasm patients with higher secondary myelofibrosis progression risk.

Leukemia 2020 Feb 27. Epub 2020 Feb 27.

Hospital Morales Meseguer, Centro Regional de Hemodonación, IMIB-Arrixaca, Murcia, Spain.

Myelofibrosis (MF) occurs as part of the natural history of polycythemia vera (PV) and essential thrombocythemia (ET), and remarkably shortens survival. Although JAK2V617F and CALR allele burden are the main transformation risk factors, inflammation plays a critical role by driving clonal expansion toward end-stage disease. NF-κB is a key mediator of inflammation-induced carcinogenesis. Read More

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http://dx.doi.org/10.1038/s41375-020-0767-3DOI Listing
February 2020

Hematological disturbances in Down syndrome: single centre experience of thirteen years and review of the literature.

Turk J Pediatr 2019 ;61(5):664-670

Divisions of Hematology, Department of Pediatrics, Ondokuz Mayıs University School of Medicine, Samsun, Turkey.

Karakurt N, Uslu İ, Aygün C, Albayrak C. Hematological disturbances in Down syndrome: single centre experience of thirteen years and review of the literature. Turk J Pediatr 2019; 61: 664-670. Read More

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http://dx.doi.org/10.24953/turkjped.2019.05.004DOI Listing
January 2019

Thrombocytopenia in patients with myelofibrosis: management options in the era of JAK inhibitor therapy.

Leuk Lymphoma 2020 Feb 24:1-13. Epub 2020 Feb 24.

Hematology, Department of Medicine and Surgery, University of Insubria, Varese, Italy.

Myelofibrosis (MF), either appearing de novo (primary MF, PMF) or after a previous diagnosis of essential thrombocythemia or of polycythemia vera, is a progressive disease burdened by symptomatic splenomegaly, debilitating systemic symptoms, ineffective hematopoiesis, and overall reduced survival. Patients often present worsening cytopenias, including thrombocytopenia, secondary to progression of the disease as well as to cytoreductive treatment. Patients with MF and thrombocytopenia have few therapeutic options and there is limited information regarding the management of disease in these settings. Read More

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http://dx.doi.org/10.1080/10428194.2020.1728752DOI Listing
February 2020

Rare Presentation of Bilateral Central Retinal Vein Occlusion and Leukemic Retinopathy in a Young Adult Diagnosed with T-cell Acute Lymphoblastic Leukemia.

Cureus 2020 Jan 15;12(1):e6666. Epub 2020 Jan 15.

Emergency Medicine, Thomas Jefferson University, Philadelphia, USA.

Simultaneous bilateral central retinal vein occlusion (CRVO) is a rare presentation that warrants consideration of an underlying hyperviscosity state. Increased serum viscosity can lead to the hematologic emergency of leukostasis with resultant vascular obstruction and hypoxic tissue damage. The following case demonstrates the first case of bilateral CRVO in a young adult secondary to acute lymphoblastic leukemia (ALL). Read More

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http://dx.doi.org/10.7759/cureus.6666DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7021241PMC
January 2020

ASXL1 mutations, previous vascular complications and age at diagnosis predict survival in 85 WHO-defined polycythaemia vera patients.

Br J Haematol 2020 Feb 17. Epub 2020 Feb 17.

Department of Clinical Chemistry, Sahlgrenska University Hospital, Gothenburg, Sweden.

Polycythaemia vera (PV) patients have an overall comparatively favourable prognosis, but disease progression is very heterogeneous and life-threatening thrombosis and bleedings are frequent complications in untreated disease. Moreover, transformation to more severe secondary myelofibrosis and acute myeloid leukaemia can occur. The aim of this study was to identify gene mutations that could be used together with clinical data as prognostic markers to guide treatment decisions in PV patients. Read More

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http://dx.doi.org/10.1111/bjh.16450DOI Listing
February 2020

How I Manage Thrombotic/Thromboembolic Complications in Myeloproliferative Neoplasms.

Hamostaseologie 2020 Feb 12;40(1):47-53. Epub 2020 Feb 12.

Department of Medicine (Hematology, Oncology, Hemostaseology, and Stem Cell Transplantation), Faculty of Medicine, RWTH Aachen University, Aachen, Germany.

Patients with myeloproliferative neoplasms (MPNs), such as polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are at increased risk for arterial and venous thrombosis/thromboembolism. In particular, the risk of splanchnic venous thrombosis, such as portal vein thrombosis or Budd-Chiari syndrome, is significantly higher in patients with MPN than in the normal population. At the same time, MPN patients are at increased risk for severe bleeding. Read More

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http://dx.doi.org/10.1055/s-0040-1701474DOI Listing
February 2020

Neonatal Morbidity in Late Preterm Infants Associated with Intrauterine Growth Restriction.

Open Access Maced J Med Sci 2019 Nov 14;7(21):3592-3595. Epub 2019 Oct 14.

Service of Neonatology, University Hospital of Obstetrics and Gynecology "Koço Gliozheni", Tirana, Albania.

Aim: This study aims to compare the neonatal morbidity of Intrauterine growth restricted (IUGR) Late Preterm (LP) babies, to those born Late Preterm but evaluated as Appropriate for Gestational Age (AGA).

Methods: The study is a 2-year prospective one that used data from the Neonatal Intensive Care Unit (NICU) charts of LP neonates born in our tertiary maternity hospital "Koço Gliozheni" in Tirana. Congenital anomalies and genetical syndromes are excluded. Read More

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http://dx.doi.org/10.3889/oamjms.2019.832DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6986514PMC
November 2019

Venous thromboembolism associated with hyperhomocysteinemia, homozygosity for the methylenetetrahydrofolate reductase 677C>T gene variant, and secondary polycythemia.

Blood Coagul Fibrinolysis 2020 Jun;31(4):270-273

Department of Laboratory Medicine, Uji-Tokushukai Medical Center, Uji, Kyoto, Japan.

: The development of pulmonary embolism/deep vein thrombosis (DVT) in the extremities is influenced by various risk factors. Hyperhomocysteinemia is one such risk factor, which may be associated with vitamin B12/folate deficiency, or the methylenetetrahydrofolate reductase gene variant, 677C>T. Here, we report a 47-year-old male who developed pulmonary embolism/DVT, associated with hyperhomocysteinemia (plasma homocysteine: 71. Read More

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http://dx.doi.org/10.1097/MBC.0000000000000897DOI Listing

Thrombosis among 1537 patients with JAK2 -mutated myeloproliferative neoplasms: Risk factors and development of a predictive model.

Cancer Med 2020 Mar 28;9(6):2096-2105. Epub 2020 Jan 28.

Department of Hematology, The Second Hospital of Tianjin Medical University, Tianjin, China.

To explore the risk factors of thrombosis in patients with JAK2 -mutated myeloproliferative neoplasms (MPNs), a cohort of 1537 Chinese patients with JAK2 -mutated MPN was retrospectively analyzed. The Kaplan-Meier method and multivariate Cox analysis were used to study the risk factors of thrombosis in patients with JAK2 -mutated MPN. Among the 1537 MPN patients, 931, 468, and 138 had polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), respectively. Read More

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http://dx.doi.org/10.1002/cam4.2886DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7064115PMC

Polycythemia with Renal Cell Carcinoma and Normal Erythropoietin Level.

Case Rep Urol 2019 11;2019:3792514. Epub 2019 Dec 11.

Department of Hematology and Oncology, Texas Tech University Health Sciences Center, Lubbock, TX, USA.

A 61-year-old obese Caucasian male with past medical history of smoking, hypertension, chronic obstructive pulmonary disease (COPD), and sleep apnea presented to the hematology clinic with polycythemia. Despite the newly-diagnosed polycythemia, the patient denied any significant symptoms or history of blood clots. Further evaluation with computerized tomography (CT) and ultrasound showed a large renal mass suspicious for renal cell carcinoma of the right kidney. Read More

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http://dx.doi.org/10.1155/2019/3792514DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6942735PMC
December 2019

Polycythaemia Vera Presenting As A Porta Hepatis Mass.

J Ayub Med Coll Abbottabad 2019 Oct-Dec;31(4):627-628

Aga Khan University Hospital, Karachi, Pakistan.

Polycythaemia Vera (PV) is a myeloproliferative disorder in which bone marrow has increased production of red blood cells, white blood cells and platelets. The hallmarks of the disease are veno-occlusive events, secondary to increased blood viscosity. Polycythaemia Vera rarely presents with portal vein thrombosis below age of 55 years especially in absence of any chronic liver disease. Read More

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Tobacco smoking causes secondary polycythemia and a mild leukocytosis among heavy smokers in Taif City in Saudi Arabia.

Saudi J Biol Sci 2020 Jan 18;27(1):407-411. Epub 2019 Nov 18.

Laboratory Technology Department, Turabah University College, Taif University, 5137, Unit No. 1, At Taif 26522 - 7360, Saudi Arabia.

Tobacco smoking is a common risk factor of cardiovascular diseases, cancers and heart health problems. In Taif, the number of secondary polycythemia patients is increasing dramatically and most of those patients are heavy smokers. Therefore, this study is an attempt to understand the pathophysiological mechanism behind that problem. Read More

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http://dx.doi.org/10.1016/j.sjbs.2019.11.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6933161PMC
January 2020

Ruxolitinib and interferon-α2 combination therapy for patients with polycythemia vera or myelofibrosis: a phase II study.

Haematologica 2019 Dec 26. Epub 2019 Dec 26.

Department of Hematology, Zealand University Hospital, Roskilde, Denmark.

We report the final two-year end-of-study results from the first clinical trial investigating combination treatment with ruxolitinib and low-dose pegylated interferon-α2 (PEG-IFNα2). The study included 32 patients with polycythemia vera (PV) and 18 with primary- or secondary myelofibrosis (MF); 46 patients were previously intolerant or refractory to PEG-IFNα2. The primary outcome was efficacy, based on hematological parameters, quality of life measurements, and JAK2 V617F allele burden. Read More

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http://dx.doi.org/10.3324/haematol.2019.235648DOI Listing
December 2019

Prevalence of placental dichotomy, fetal cardiomegaly and starry-sky liver in twin anemia polycythemia sequence.

Ultrasound Obstet Gynecol 2019 Dec 19. Epub 2019 Dec 19.

Division of Fetal Medicine, Department of Obstetrics, Leiden University Medical Center, The Netherlands.

Objective: To investigate the prevalence of additional ultrasound markers including placental dichotomy, cardiomegaly, and starry-sky liver in pregnancies with twin anemia polycythemia sequence (TAPS).

Methods: All monochorionic twins antenatally diagnosed with TAPS at our center between 2006-2019 were retrospectively reviewed for the presence of placental dichotomy, cardiomegaly in the donor and a starry-sky liver in the recipient. TAPS was diagnosed based on delta middle cerebral artery - peak systolic velocity > 0. Read More

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http://dx.doi.org/10.1002/uog.21948DOI Listing
December 2019

Inaccurate point-of-care blood glucose measurement in a dog with secondary erythrocytosis.

J Vet Emerg Crit Care (San Antonio) 2020 Jan 16;30(1):81-85. Epub 2019 Dec 16.

Department of Veterinary Clinical Sciences, Iowa State University, Ames, IA.

Background: Point-of-care (POC) portable blood glucose meters (PBGMs) are convenient and inexpensive tools for assessing patient blood glucose concentrations. They are often used to quickly diagnose hypoglycemia or collect serial glucose readings in diabetic patients. However, POC meters have been previously identified in human and veterinary literature to be inaccurate when utilized in patients with abnormal HCT. Read More

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http://dx.doi.org/10.1111/vec.12909DOI Listing
January 2020

Myeloproliferative and lymphoproliferative disorders: State of the art.

Hematol Oncol 2020 Apr 27;38(2):121-128. Epub 2019 Dec 27.

UOC Ematologia Aziendale, Azienda Usl Toscana Nord Ovest, Pisa, Italy.

Myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), are clonal disorders complicated mainly by vascular events and transformation to myelofibrosis (for PV and ET) or leukemia. Although secondary malignancies, in particular, lymphoproliferative disorders (LPNs), are rare, they occur at a higher frequency than found in the general population, and there has been recent scientific discussion regarding a hypothetical relationship between treatment with JAK inhibitors in MPN and the risk of development of LPN. This has prompted increased interest regarding the coexistence of MPN and LPN. Read More

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http://dx.doi.org/10.1002/hon.2701DOI Listing
April 2020
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Successful treatment of pulmonary embolism in a patient with polycythemia vera by rheolytic thrombectomy.

J Cardiovasc Thorac Res 2019 1;11(4):325-327. Epub 2019 May 1.

Health Sciences University, Sultan Abdülhamid Han Training and Research Hospital, Department of Cardiology, Istanbul, Turkey.

Acute pulmonary embolism (APE) may lead to life-threatening conditions such as cardiac death and congestive heart failure. Thus, a proper diagnosis and management play a crucial role to prevent such complications. Moreover, APE is a rare clinical onset of chronic myeloproliferative disease. Read More

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http://dx.doi.org/10.15171/jcvtr.2019.54DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6891036PMC

Nocturnal Mean Oxygen Saturation Is Associated with Secondary Polycythemia in Young Adults with Obstructive Sleep Apnea, Especially in Men.

Nat Sci Sleep 2019 5;11:377-386. Epub 2019 Dec 5.

Department of Respiratory and Critical Care Medicine, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, People's Republic of China.

Objective: Whether the severity of obstructive sleep apnea (OSA) contributes to clinical polycythemia is uncertain, especially in young adults. This study aimed to assess the correlation between untreated OSA and polycythemia, controlling for multiple confounders, and to observe the difference in both genders.

Methods: All participants underwent nocturnal polysomnography. Read More

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http://dx.doi.org/10.2147/NSS.S226143DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6901050PMC
December 2019

Management of macular edema with branch retinal vein occlusion in a case of secondary polycythemia.

GMS Ophthalmol Cases 2019 20;9:Doc38. Epub 2019 Nov 20.

Shri Bhagawan Mahavir Vitreoretinal Services, Sankara Nethralaya Hospital, Chennai, India.

We report a case of polycythemia with an ocular complication of branch retinal vein occlusion associated with macular edema that was managed by anti-vascular endothelial growth factor (VEGF) and systemic management. A 43-year-old, one-eyed male, a known case of polycythemia presented with complaints of decreased vision in the right eye. He underwent comprehensive eye evaluation, fundus photography and optical coherence tomography at the baseline visit and post intravitreal ranibizumab 1-, 3-, 4- and 11-month follow-up. Read More

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http://dx.doi.org/10.3205/oc000127DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6883382PMC
November 2019

Risk of disease transformation and second primary solid tumors in patients with myeloproliferative neoplasms.

Blood Adv 2019 11;3(22):3700-3708

Department of Internal Medicine.

This study aimed to elucidate patterns of disease transformation to secondary myelofibrosis (SMF) or secondary acute myeloid leukemia (SAML) and the development of second primary malignancies in South Korean patients with BCR-ABL1-negative myeloproliferative neoplasms (MPNs). By using nationwide public health care insurance claims data, we identified and analyzed 7454 patients with MPNs who were newly diagnosed with essential thrombocythemia (ET), polycythemia vera (PV), or primary myelofibrosis (PMF) from 2008 to 2016 and used the data to appropriately trace the disease course. Transformation to SMF or SAML was rare in patients with ET and PV, but patients with PMF had an 8-year cumulative incidence of SAML of 21. Read More

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http://dx.doi.org/10.1182/bloodadvances.2019000655DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6880910PMC
November 2019

An unusual case of pulmonary arterio-venous fistula (PAVF).

J Family Med Prim Care 2019 Oct 31;8(10):3408-3411. Epub 2019 Oct 31.

Department of Medicine, Tata Main Hospital, Jamshedpur, Bistupur, Jharkhand, India.

Pulmonary Arteriovenous Fistula (PAVF) is an abnormal communication between the pulmonary veins and pulmonary arteries. Most individuals may have the condition since birth (congenital occurrence), but it can also be an acquired condition. Most individuals with Pulmonary Arteriovenous Fistula also have a hereditary haemorrhagic telangiectasia. Read More

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http://dx.doi.org/10.4103/jfmpc.jfmpc_580_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6857369PMC
October 2019

Risk factors for infections and secondary malignancies in patients with a myeloproliferative neoplasm treated with ruxolitinib: a dual-center, propensity score-matched analysis.

Leuk Lymphoma 2020 Mar 12;61(3):660-667. Epub 2019 Nov 12.

Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Ruxolitinib is a JAK1/2 inhibitor approved for the treatment of myelofibrosis (MF) and polycythemia vera (PV). Recent data have suggested the possibility of increased infectious and secondary malignancy rates in patients treated with ruxolitinib. We conducted a dual-center, retrospective study of 202 myeloproliferative neoplasm (MPN) patients receiving ruxolitinib and a control cohort of 73 ruxolitinib-naïve MPN patients. Read More

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http://dx.doi.org/10.1080/10428194.2019.1688323DOI Listing

The Temporal Sequence and the Differences in Somatic Mutation Acquisition Determines Clinical Behaviors of JAK2-Positive Myeloproliferative Neoplasms.

Anticancer Res 2019 Nov;39(11):6273-6282

Cancer Research Institute, Seoul National University Hospital, Seoul, Republic of Korea.

Background/aim: We have yet to understand why JAK2-positive myeloproliferative neoplasms (MPN) patients manifest different phenotypes despite harboring JAK2 mutations and what drives secondary transformations.

Patients And Methods: Using targeted sequencing, we analyzed mutational status of 17 polycythemia vera (PV), 16 essential thrombocythemia (ET), 8 primary myelofibrosis (PMF) patients who tested positive for JAK by polymerase chain reaction.

Results: The somatic mutations in JAK2 influence the clinical behavior of the disease. Read More

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http://dx.doi.org/10.21873/anticanres.13837DOI Listing
November 2019

Mycobacterial Infections With Ruxolitinib: A Retrospective Pharmacovigilance Review.

Clin Lymphoma Myeloma Leuk 2020 Jan 26;20(1):18-23. Epub 2019 Aug 26.

Houston Methodist Cancer Center/Weill Cornell Medicine, Houston, TX. Electronic address:

Background: Ruxolitinib is a selective Janus kinase inhibitor (JAKI) 1/2 approved for the treatment of myelofibrosis (MF) and polycythemia vera (PV). These patients may be at risk for developing opportunistic infections. We assessed the number of patients that developed typical (Mycobacterium tuberculosis [MTB]) and atypical mycobacterial infections (AMI) while on treatment with ruxolitinib by utilizing the United States Food and Drug Administration (FDA) adverse events reporting system (FAERS). Read More

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http://dx.doi.org/10.1016/j.clml.2019.08.008DOI Listing
January 2020
4 Reads

Acute ischaemic stroke in secondary polycythaemia due to complex congenital cyanotic heart disease.

BMJ Case Rep 2019 Oct 30;12(10). Epub 2019 Oct 30.

Department of Neurology, Christian Medical College & Hospital Ludhiana, Ludhiana, India

A 65-year-old woman presented to the emergency department with an acute onset of left-sided hemiparesis and slurred speech for 6 hours. Physical examination was notable for clubbing and peripheral cyanosis. Brain MRI showed an acute infarct involving the right gangliocapsular region. Read More

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http://dx.doi.org/10.1136/bcr-2019-231261DOI Listing
October 2019
1 Read

A Kindred with a β-Globin Base Substitution [β89(F5)Ser→Arg (AG>AG); : c.270T>G] Resulting in Hemoglobin Vanderbilt.

Hemoglobin 2019 Jul - Sep;43(4-5):273-276. Epub 2019 Oct 28.

Division of Hematology, Stanford Cancer Institute, Stanford University School of Medicine, Stanford, CA, USA.

High oxygen affinity hemoglobins (Hbs), characterized by a decreased ability to release oxygen to the tissues and a left-shifted oxygen dissociation curve, are a rare cause of secondary erythrocytosis. Here, we report a base substitution in the β-globin gene at codon 89 (AG>AG) in a kindred with familial erythrocytosis resulting in Hb Vanderbilt, a high oxygen affinity variant. Read More

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http://dx.doi.org/10.1080/03630269.2019.1680382DOI Listing

The red blood cell count and the erythrocyte sedimentation rate in the diagnosis of polycythaemia vera.

Eur J Haematol 2020 Jan 22;104(1):46-54. Epub 2019 Nov 22.

Department of Hematology, Zealand University Hospital, Roskilde, Denmark.

Background: Iron deficiency in polycythaemia vera (PV) may impact the validity of the haematocrit (HCT), since HCT is red blood cell count (RBC) × mean corpuscular volume (MCV).

Objectives: To investigate (a) the effect of microcytosis on HCT, (b) the erythrocyte sedimentation rate (ESR) as a possible additional diagnostic marker for PV.

Material And Methods: This study included 182 subjects: 39 with PV, 27 with essential thrombocythemia (ET) and 116 suspected of myeloproliferative neoplasm (MPN) with a secondary cause for either thrombocytosis or erythrocytosis. Read More

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http://dx.doi.org/10.1111/ejh.13334DOI Listing
January 2020
3 Reads

Beyond Hemoglobin: When and How to Work Up Possible Polycythemia Vera.

Clin Med Res 2020 Mar 3;18(1):11-20. Epub 2019 Oct 3.

Biomedical Informatics Research Center, Marshfield Clinic Research Institute, Marshfield, Wisconsin.

Background: World Health Organization (WHO) 2017 diagnostic criteria for hemoglobin levels in polycythemia vera (PV) were lowered from 185 g/L to 165 g/L for men and from 165 g/L to 160 g/L for women, but these cutoffs were not designed for screening.

Objectives: The primary aim of this study was to assess the value of laboratory and clinical parameters in deciding whether to further pursue a diagnosis of PV. A secondary aim was to explore the diagnostic utility of bone marrow morphology. Read More

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http://dx.doi.org/10.3121/cmr.2019.1483DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7153800PMC
March 2020
1 Read

Clinical Spectrum and Complications of Polycythemia, in Patients presenting at Tertiary Care Centre at Goa.

J Assoc Physicians India 2019 Oct;67(10):20-24

Associate Professor, Dept. of Medicine, Goa Medical College, Goa.

Background: Polycythemia is characterized by rise in hemoglobin and hematocrit, either as a result of hematopoietic clonal expansion (Vera) or secondary to hypoxic stimuli (secondary polycythemia).It is of great importance to detect early and identify the type of polycythemia and also asses the thrombotic risk so that timely and appropriate treatment can be given. The present study aims to characterize the different presentations and complications of polycythemia, evaluate genetic factors and differences between the two categories of polycythemia in ethnic Goan subjects. Read More

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October 2019
3 Reads

Fedratinib: First Approval.

Authors:
Hannah A Blair

Drugs 2019 Oct;79(15):1719-1725

Springer Nature, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.

Fedratinib (INREBIC) is a JAK2-selective inhibitor that has been developed as an oral treatment for myelofibrosis. In August 2019, fedratinib received its first global approval in the USA for the treatment of adult patients with intermediate-2 or high-risk primary or secondary (post-polycythemia vera or post-essential thrombocythemia) myelofibrosis. Phase III clinical development for myelofibrosis is ongoing worldwide. Read More

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http://dx.doi.org/10.1007/s40265-019-01205-xDOI Listing
October 2019
1 Read

Concurrent heterozygous Von-Hippel-Lindau and transmembrane-protein-127 gene mutation causing an erythropoietin-secreting pheochromocytoma in a normotensive patient with severe erythrocytosis.

J Hypertens 2020 Feb;38(2):340-346

Cardiorenal Research Unit, Centro Ipertensione Arteriosa e Studio Malattie Cardiorenali, SS Fisiopatologia Medica, Clinica e Terapia Medica, Dipartimento di Medicina e Chirurgia, University of Parma, Parma.

Background: Mutations of genes related to Krebs cycle enzymes, kinases or to pseudohypoxic signaling pathways, including Von-Hippel-Lindau (VHL) and transmembrane-protein-127 predispose to pheochromocytoma and paraganglioma development. Homozygous loss of function mutation of VHL (VHL 598C>T) gene can associate with polycythemia because of an altered hypoxia sensing.

Patient: A 19-year-old normotensive man presented with headache, fatigue associated with severe erythrocytosis (hematocrit 76%), high hemoglobin (25. Read More

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http://dx.doi.org/10.1097/HJH.0000000000002253DOI Listing
February 2020
2 Reads

Comparative phenotypic profiling of the JAK2 inhibitors ruxolitinib, fedratinib, momelotinib, and pacritinib reveals distinct mechanistic signatures.

PLoS One 2019 27;14(9):e0222944. Epub 2019 Sep 27.

Eurofins Discovery, Phenotypic Services, Burlingame, California, United States of America.

Janus kinase-signal transducers and activators of transcription (JAK-STAT) signaling is critical to multiple cellular processes, including survival, differentiation, and proliferation. JAK-STAT signaling dysregulation has been noted in inflammatory disorders, and aberrant JAK2 pathway activation has been implicated in myelofibrosis and polycythemia vera. Moreover, 4 therapeutic JAK2 inhibitors (ruxolitinib, fedratinib, momelotinib, and pacritinib) have either been approved or are in advanced clinical development for myelofibrosis. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0222944PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6764664PMC
April 2020
3 Reads

The role of a low erythropoietin level for the polycythemia vera diagnosis.

Blood Cells Mol Dis 2020 02 7;80:102355. Epub 2019 Sep 7.

Hematology and Oncology Division, Henry Ford Cancer Institute, Henry Ford Health System, Detroit, MI, United States of America.

A low erythropoietin (EPO) level is a minor diagnostic criterion for polycythemia vera (PV). Controversies exist regarding the diagnostic value of a low EPO level when considering increasing availability of advanced molecular testing. We assessed the role of low EPO level for PV diagnosis in the context of positive JAK2 mutation status as well as other diagnostic parameters. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10799796193031
Publisher Site
http://dx.doi.org/10.1016/j.bcmd.2019.102355DOI Listing
February 2020
3 Reads

Inappropriate Secondary Erythrocytosis in a Dog With Renal Sarcoma.

Top Companion Anim Med 2019 Sep 15;36:9-11. Epub 2019 May 15.

Department of Small Animal Medicine and Surgery, College of Veterinary Medicine, University of Georgia, Athens, GA, USA.

A 7-year-old mixed breed dog was evaluated for erythrocytosis with an initial hematocrit of 82.3%. Abdominal ultrasound revealed a 6 cm mass on the cranial pole of the right kidney. Read More

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http://dx.doi.org/10.1053/j.tcam.2019.05.002DOI Listing
September 2019
3 Reads

A journey through infectious risk associated with ruxolitinib.

Br J Haematol 2019 Nov 29;187(3):286-295. Epub 2019 Aug 29.

Department of Haematology Oncology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.

Ruxolitinib has proved to be effective for the treatment of patients with myelofibrosis (either primary or secondary) and polycythaemia vera, and its approval led to a significant change in the current treatment algorithm. Despite its efficacy and beyond its well described haematological toxicity, a peculiar immunosuppressive effect emerged as our clinical experience grew, both within and outside of a clinical trial setting. Definite and negative interactions with multiple pathways of the immune system of patients have been reported so far, involving both adaptive and innate immune responses. Read More

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http://dx.doi.org/10.1111/bjh.16174DOI Listing
November 2019
2 Reads

UNUSUAL CAUSE OF BRANCH RETINAL ARTERY OCCLUSION: POLYCYTHEMIA IN A TRANSGENDER MAN FROM UNREGULATED TESTOSTERONE USE.

Retin Cases Brief Rep 2019 Aug 28. Epub 2019 Aug 28.

National Healthcare Group Eye Institute, Tan Tock Seng Hospital, Singapore.

Purpose: We report a transgender patient with branch retinal artery occlusion who had secondary polycythemia from unregulated testosterone injections and review the literature on the mechanisms of supraphysiologic and standard doses of testosterone causing a hypercoagulable state.

Methods: Case report.

Results: A 45-year-old Chinese transgender man with no medical history presented with a 1-week history of a scotoma in his left eye vision. Read More

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http://dx.doi.org/10.1097/ICB.0000000000000924DOI Listing
August 2019
13 Reads

Management of advanced phase myeloproliferative neoplasms.

Clin Adv Hematol Oncol 2019 Jul;17(7):405-411

Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, New York.

The BCR-ABL1-negative myeloproliferative neoplasms (MPNs), including polycythemia vera, essential thrombocythemia, and primary myelofibrosis, can evolve into a form of secondary acute myeloid leukemia termed MPN in blast phase (MPN-BP). MPN in accelerated phase (MPN-AP), which is defined by 10% to 19% myeloid blasts in the peripheral blood or bone marrow, is a precursor to MPN-BP. Alternative definitions of MPN-AP exist based on studies identifying clinical variables that portend a poor prognosis and high risk for progression to MPN-BP. Read More

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July 2019
7 Reads