1,653 results match your criteria Polycythemia Secondary


Ruxolitinib versus best available therapy in inadequately controlled polycythaemia vera without splenomegaly (RESPONSE-2): 5-year follow up of a randomised, phase 3b study.

Lancet Haematol 2022 May 18. Epub 2022 May 18.

University Clinic for Haematology, Oncology, Haemostaseology and Palliative Care, Johannes Wesling Medical Centre Minden, University of Bochum, Minden, Germany.

Background: The phase 3b, randomised, open-label RESPONSE-2 study in patients with inadequately controlled polycythaemia vera without splenomegaly showed superiority of the Janus kinase (JAK) 1 and JAK2 inhibitor ruxolitinib versus best available therapy for the primary endpoint of haematocrit control at week 28. Here, we present secondary endpoints of the RESPONSE-2 study after 5 years of follow-up.

Methods: RESPONSE-2 was an open-label, randomised, phase 3b study done at 48 hospitals or clinics across 12 countries in Asia, Australia, Europe, and Canada. Read More

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Addition of navitoclax to ongoing ruxolitinib treatment in patients with myelofibrosis (REFINE): a post-hoc analysis of molecular biomarkers in a phase 2 study.

Lancet Haematol 2022 May 13. Epub 2022 May 13.

Guy's and St Thomas' NHS Foundation Trust, London, UK. Electronic address:

Background: Primary analyses of cohort 1a of the REFINE trial showed that addition of navitoclax to ruxolitinib induced a 35% or greater reduction in spleen volume (SVR) and reduced symptoms in patients with myelofibrosis no longer benefiting from ruxolitinib. Here, we report the exploratory post-hoc biomarker analyses from cohort 1a.

Methods: REFINE is a phase 2, multicentre, open-label trial designed to assess the activity and safety of navitoclax alone or in combination with ruxolitinib in patients with primary or secondary (post-polycythaemia vera or post-essential thrombocythaemia) myelofibrosis. Read More

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Pacritinib: First Approval.

Authors:
Yvette N Lamb

Drugs 2022 May 14. Epub 2022 May 14.

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

Pacritinib (VONJO™) is an orally administered, small molecule kinase inhibitor being developed by CTI BioPharma for the treatment of myelofibrosis and graft-versus-host disease. Pacritinib received its first approval in February 2022 in the USA for the treatment of adults with intermediate- or high-risk primary or secondary (post-polycythemia vera or post-essential thrombocythemia) myelofibrosis with a platelet count below 50 × 10/L. The accelerated approval was based on results from the randomized, active-controlled, phase III PERSIST-2 trial, in which spleen volume reduction was demonstrated in pacritinib recipients. Read More

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Impact of Individual Comorbidities on Survival of Patients with Myelofibrosis.

Cancers (Basel) 2022 May 9;14(9). Epub 2022 May 9.

Hematology Department, Hospital Universitario Virgen de la Victoria, 29010 Málaga, Spain.

The comorbidity burden is an important risk factor for overall survival (OS) in several hematological malignancies. This observational prospective study was conducted to evaluate the impact of individual comorbidities on survival in a multicenter series of 668 patients with primary myelofibrosis (PMF) or MF secondary to polycythemia vera (PPV-MF) or essential thrombocythemia (PET-MF). Hypertension (hazard ratio (HR) = 4. Read More

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Hemorrhages in polycythemia vera and essential thrombocythemia: epidemiology, description, and risk factors, learnings from a large cohort.

Thromb Haemost 2022 May 11. Epub 2022 May 11.

Institut de Cancérologie et d'Hématologie, CHU de Brest, Brest, France.

Background: The management of myeloproliferative neoplasms (MPN) is based on the reduction of thrombosis risk. The incidence, impact, and risk factors of bleedings have been less studied.

Method: All patients with polycythemia vera (n=339) or essential thrombocythemia (n=528) treated in our center are included in OBENE cohort (NCT02897297). Read More

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Clinicopathological characteristics of high-altitude polycythemia-related kidney disease in Tibetan inhabitants.

Kidney Int 2022 May 2. Epub 2022 May 2.

Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China; Renal Pathological Center, Institute of Nephrology, Peking University, Beijing, China; Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China; Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, China.

High-altitude polycythemia (HAPC) is a clinical syndrome that occurs in native inhabitants or long-term residents living at altitude. The kidney is one of the most affected organs. However, the clinical and kidney histopathological profiles of HAPC-related kidney disease have rarely been reported. Read More

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Selective versus complete fetoscopic coagulation of vascular equator: a matched comparative study.

Am J Obstet Gynecol 2022 Apr 29. Epub 2022 Apr 29.

From the Department of Obstetrics and Maternal-Fetal Medicine, Hôpital Necker-Enfants Malades, AP-HP and EA7328, Université de Paris, Paris, France (Drs Bartin, Colmant, Bourgon, Ville, Stirnemann and Mrs Carrier).

Background: Complete coagulation of the vascular equator (i.e. 'Solomon' technique) has been suggested to reduce post-operative complications such as twin anemia polycythemia syndrome (TAPS) and recurrence of TTTS following fetoscopic laser coagulation of chorionic vessels for twin-twin transfusion syndrome (TTTS). Read More

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[Analysis of related factors in secondary erythrocytosis of obstructive sleep apnea hypopnea syndrome in Gansu province].

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2022 May;36(5):338-342

Department of Sleep Medicine Center,Gansu Provincial Hospital,Lanzhou,730000,China.

To analyze the related factors of secondary erythrocytosis of obstructive sleep apnea(OSA) in Gansu province. Polysomnography recording and analysis from January 2013 to January 2021, A total of 448 OSA patients of long-resident Han nationality in Gansu province. Hemoglobin(Hb) values were divided into group A(Hb 120-160 g/L) 41 cases, B(Hb 161-179 g/L) 142 cases, C(Hb 180-199 g/L) 152 cases, D(Hb 200-219 g/L) 79 cases, and E(Hb ≥220 g/L) 30 cases. Read More

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Thrombotic and Atherogenetic Predisposition in Polyglobulic Donors.

Biomedicines 2022 Apr 12;10(4). Epub 2022 Apr 12.

Department of Cybernetics and Biomedical Engineering, VŠB-Technical University of Ostrava, 17. listopadu 2172/15, 708 00 Ostrava-Poruba, Czech Republic.

This work analyses the results of research regarding the predisposition of genetic hematological risks associated with secondary polyglobulia. The subjects of the study were selected based on shared laboratory markers and basic clinical symptoms. (Janus Kinase 2) mutation negativity represented the common genetic marker of the subjects in the sample of interest. Read More

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Rapid progression of myelofibrosis in polycythemia vera patient carrying SRSF2 c.284C>A p.(Pro95His) and unique ASXL1 splice site c.1720-2A>G variant.

J Clin Lab Anal 2022 May 18;36(5):e24388. Epub 2022 Apr 18.

Department of Hematology and Bone Marrow Transplantation, Poznań University of Medical Sciences, Poznań, Poland.

Background: The prognosis in polycythemia vera (PV) is comparatively favorable, but individual myelofibrosis/leukemic progression risk is heterogeneous. About a quarter of patients progress to the fibrotic phase after 20 years.

Methods: Multiplex PCR, allele-specific qPCR, high-resolution melt analysis, and Sanger sequencing were used to detect BCR-ABL, JAK2, ASXL1, SRSF2, U2AF1, and IDH1/2 variants. Read More

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Patient with clinical celiac disease mimicking triple-negative essential thrombocythemia.

Clin Case Rep 2022 Apr 7;10(4):e05197. Epub 2022 Apr 7.

Department of Hematology and Oncology Hamad Medical Corporation Doha Qatar.

Platelets are acute-phase reactants, which can be elevated due to a secondary cause or less commonly because of a primary mechanism. Primary disorders include hematological conditions such as myelodysplastic syndrome, acute myeloid leukemia, chronic myeloid leukemia, polycythemia vera, and essential thrombocythemia (ET). Most ET patients have a mutation in the genes regulating thrombopoiesis, , , or genes. Read More

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Treatment of Focal and Segmental Glomerulosclerosis Secondary to High Altitude Polycythemia with Acetazolamide.

High Alt Med Biol 2022 Apr 8. Epub 2022 Apr 8.

Nephrology Division, Arzobispo Loayza National Hospital, Lima, Peru.

Vizcarra-Vizcarra, Cristhian A., Eduardo Chávez-Velázquez, Carmen Asato-Higa, and Abdías Hurtado-Aréstegui. Treatment of focal and segmental glomerulosclerosis secondary to high altitude polycythemia with acetazolamide. Read More

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High platelet-to-lymphocyte ratio may differentiate polycythemia vera from secondary polycythemia.

Wien Klin Wochenschr 2022 Apr 7. Epub 2022 Apr 7.

University Hospital Dubrava, Zagreb, Croatia.

Discriminating polycythemia vera (PV) from secondary polycythemia (SP) is crucial due to the inherent risk of thrombosis in PV and different treatment approaches. The majority of PV patients have subnormal serum erythropoietin levels and harbor Janus kinase 2 (JAK2) mutations; however, serum erythropoietin levels may be normal in approximately one third of PV patients and mutational analysis is costly and requires access to specialized laboratories. Recently, neutrophil-to-lymphocyte (NLR) and platelet-to-lymphocyte ratios (PLR) emerged as rapidly available biomarkers to identify PV patients under an increased risk of thrombosis and death. Read More

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Deimplementation of Polycythemia Screening in Asymptomatic Infants in a Level 1 Nursery.

Pediatr Qual Saf 2022 Mar-Apr;7(2):e533. Epub 2022 Mar 30.

Department of Pediatrics, Medical University of South Carolina, Charleston, SC.

Polycythemia (venous hematocrit >65%) is rare in healthy newborns (incidence: 0.4%-5%), with serious outcomes (stroke, bowel ischemia) of unknown incidence in asymptomatic infants. No national guidelines address screening or management of asymptomatic infants with polycythemia. Read More

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Prescription Patterns and Testosterone Concentrations Achieved With AndroForte 5% Testosterone Cream in Transgender and Gender Diverse Individuals.

J Sex Med 2022 Mar 30. Epub 2022 Mar 30.

Department of Endocrinology, Austin Health, Heidelberg, Victoria, Australia; Department of Medicine (Austin Health), University of Melbourne, Heidelberg, Victoria, Australia.

Background: Masculinizing hormone therapy with testosterone is used to align an individual's physical characteristics with their gender identity in trans and gender diverse individuals. Standard testosterone doses and formulations recommended for hypogonadal cisgender men are typically administered. 100 mg AndroForte 5% testosterone cream is the recommended starting dose in hypogonadal cisgender men but there are no data evaluating the use of AndroForte 5% testosterone cream in gender-affirming hormone therapy regimens. Read More

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Role of JAK inhibitors in myeloproliferative neoplasms: current point of view and perspectives.

Int J Hematol 2022 May 29;115(5):626-644. Epub 2022 Mar 29.

Department of Experimental and Clinical Medicine, University of Florence, CRIMM, Center of Research and Innovation of Myeloproliferative Neoplasms, Azienda Ospedaliero-Universitaria Careggi, Largo Brambilla, 3 pad 27B, 50134, Florence, Italy.

Classic Philadelphia-negative myeloproliferative neoplasms (MPN) include polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF), classified as primary (PMF), or secondary to PV or ET. All MPN, regardless of the underlying driver mutation in JAK2/CALR/MPL, are invariably associated with dysregulation of JAK/STAT pathway. The discovery of JAK2V617F point mutation prompted the development of small molecules inhibitors of JAK tyrosine kinases (JAK inhibitors-JAKi). Read More

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Low-risk polycythemia vera and essential thrombocythemia: management considerations and future directions.

Ann Hematol 2022 May 28;101(5):935-951. Epub 2022 Mar 28.

Division of Hematology and Medical Oncology, Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, One Gustave L Levy Place, Box 1079, New York, NY, 10029, USA.

Thrombotic events are a distinctive feature of the myeloproliferative neoplasms (MPNs) polycythemia vera (PV) and essential thrombocythemia (ET). Patients with these MPNs may also experience a poor quality of life secondary to symptom burden, as well as progression of disease to acute leukemia or myelofibrosis. Over the years, various risk stratification methods have evolved in order to attempt to predict thrombotic risk, which is the largest contributor of morbidity and mortality in these patients. Read More

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Safety and effectiveness of ruxolitinib in the real-world management of polycythemia vera patients: a collaborative retrospective study by pH-negative MPN latial group.

Ann Hematol 2022 Jun 22;101(6):1275-1282. Epub 2022 Mar 22.

Hematology, Department of Translational and Precision Medicine, Az. Policlinico Umberto I-Sapienza University, Via Benevento 6, 00161, Rome, Italy.

Ruxolitinib is approved for polycythemia vera (PV) patients after failure to previous cytoreductive therapy, based on durable results observed in phase 3 trials. We report a multicenter retrospective study demonstrating the efficacy and safety of ruxolitinib in real-life setting. Eighty-three patients were evaluated. Read More

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Impact of Umbilical Cord Milking on Hematological Parameters in Preterm Neonates With Placental Insufficiency.

Front Pediatr 2021 4;9:827219. Epub 2022 Mar 4.

Neonatal Intensive Care Unit, Mansoura University Children's Hospital, Mansoura, Egypt.

Background: Data is still lacking about the expediency of umbilical cord milking (UCM) in preterm neonates born to mothers with placental insufficiency (PI).

Objective: To study the effect of UCM in preterm neonates who had ante-natal evidence of placental insufficiency on peripheral blood cluster of differentiation 34 (CD34) percentage, hematological indices, and clinical outcomes.

Methods: Preterm neonates, <34 weeks' gestation, born to mothers with evidence of placental insufficiency that underwent UCM (PI+UCM group) were compared with historical controls whose umbilical stumps were immediately clamped [PI+ICC (immediate cord clamping) group] in a case-control study. Read More

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Potential limitations of diagnostic standard codes to distinguish polycythemia vera and secondary erythrocytosis.

Sci Rep 2022 03 18;12(1):4674. Epub 2022 Mar 18.

Section of Benign Hematology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd. Suite 1464, Houston, TX, 77030, USA.

Red cell overproduction is seen in polycythemia vera (PV), a bone marrow myeloproliferative neoplasm characterized by trilinear cell proliferation (WBC, platelets), as well as in secondary erythrocytosis (SE), a group of heterogeneous disorders characterized by elevated EPO gene transcription. We aimed to verify the concordance of the International Classification of Diseases (ICD) code-based diagnosis of "polycythemia" or "erythrocytosis" with the true clinical diagnosis of these conditions. We retrospectively reviewed the electronic medical records (January 1, 2005, to December 31, 2016) of adult patients with ICD codes of polycythemia and/or erythrocytosis who had testing done for the presence of the JAK2V617F mutation. Read More

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Testosterone concentrations and prescription patterns of 1% testosterone gel in transgender and gender diverse individuals.

Ther Adv Endocrinol Metab 2022 11;13:20420188221083512. Epub 2022 Mar 11.

Department of Medicine, Austin Health, The University of Melbourne, 145 Studley Road, Heidelberg, VIC 3084, Australia.

Background: Masculinising hormone therapy with testosterone is used to align an individual's physical characteristics with their gender identity. Standard testosterone doses and formulations recommended for hypogonadal cisgender men are typically administered, although there are currently limited data evaluating the use of 1% testosterone gel in gender-affirming hormone therapy regimens.

Objectives: The objective of the study was to assess the prescription patterns and serum total testosterone concentrations achieved with 1% testosterone gel in trans and gender diverse individuals. Read More

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Unintentional Dual Benefit: Improvement in Secondary Polycythemia in a Patient Receiving Chemotherapy for Metastatic Clear Cell Renal Cell Carcinoma.

Eur J Case Rep Intern Med 2022 18;9(2):003125. Epub 2022 Feb 18.

St. Joseph's University Medical Center, Paterson, NJ, USA.

Complete blood counts are frequently collected from cancer patients, but laboratory findings may be misleading. Secondary polycythemia can occur in renal cell carcinoma (RCC) due to erythropoietin (EPO) stimulation. Therefore, complete blood counts should be closely monitored to prevent complications such as thrombosis. Read More

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February 2022

[Fatal primary influenza virus pneumonia in a patient with secondary myelofibrosis during ruxolitinib therapy].

Rinsho Ketsueki 2022 ;63(2):108-110

Department of Hematology, Tokyo Metropolitan Geriatric Hospital.

In recent years, fatal cases of primary influenza virus pneumonia have been rare. A 67-year-old woman with secondary myelofibrosis, who had been diagnosed with polycythemia vera 25 years prior, died of primary influenza virus pneumonia. She was immunocompromised due to the underlying disease and ruxolitinib therapy, but she was not vaccinated against influenza. Read More

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Myelofibrotic and leukemic transformation in 2016 WHO-defined Philadelphia-negative myeloproliferative neoplasm.

Blood Res 2022 Mar;57(1):59-68

Division of Hematology/Oncology, Department of Internal Medicine, Daejeon, Korea.

Background: Information on myelofibrotic and leukemic transformations in Korean Philadelphia chromosome- negative myeloproliferative neoplasms (Ph MPNs) is limited.

Methods: This study retrospectively analyzed transformations in patients diagnosed with essential thrombocythemia (ET), polycythemia vera (PV) prefibrotic/early primary myelofibrosis (pre-PMF), or overt primary myelofibrosis (PMF) based on the 2016 World Health Organization criteria between January 1996 and December 2020 at Chungam National University Hospital, Daejeon, Korea.

Results: A total of 351 patients (144 with ET, 131 with PV, 45 with pre-PMF, and 31 with PMF; 204 men and 147 women) with a median age of 64 years (range, 15‒91 years) were followed for a median of 4. Read More

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The Case of the Red Extremities.

Clin Pract Cases Emerg Med 2022 Feb;6(1):83-84

Mayo Clinic, Department of Emergency Medicine, Phoenix, Arizona.

Case Presentation: A 37-year-old man with severe obstructive sleep apnea presented to the emergency department with burning pain, redness and swelling in his hands and feet, worsening for several weeks. Pertinent laboratory studies revealed polycythemia.

Discussion: Erythromelalgia is a clinical diagnosis characterized by painful burning, erythema, warmth, and edema usually involving the distal extremities. Read More

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February 2022

CLINICAL CASE OF GAISBÖCK SYNDROME CAUSED BY HOOKAH AND TOBACCO SMOKING: "ZEBRA" OR "HORSE"?

Wiad Lek 2022 ;75(1 pt 2):324-326

UKRAINIAN MILITARY MEDICAL ACADEMY, KYIV, UKRAINE.

The role of smoking, in particular hookah smoking, in the development of hypoxia and, as its consequence, - polycythemia with subsequent development of hypertension is often underestimated; modification of this risk factor can lead to the normalization of blood pressure, hemoglobin and hematocrit levels, as well as red blood cell count. A case of a 31 years old man, Caucasian, applied to the General Therapeutic Clinic of the National Military Medical Clinical Center "Main Military Clinical Center" (Kyiv, Ukraine), with hypertension and secondary polycythemia associated with cigarette and hookah smoking is presented. Read More

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February 2022

Importance of Sequencing , and Genes to Confirm the Diagnosis of High Oxygen Affinity Hemoglobin.

Genes (Basel) 2022 01 12;13(1). Epub 2022 Jan 12.

Biology Division, Department of Biological Hematology, Dijon Hospital, 21000 Dijon, France.

High oxygen affinity hemoglobin (HOAH) is the main cause of constitutional erythrocytosis. Mutations in the genes coding the alpha and beta globin chains (, and ) strengthen the binding of oxygen to hemoglobin (Hb), bringing about tissue hypoxia and a secondary erythrocytosis. The diagnosis of HOAH is based upon the identification of a mutation in , or in specialized laboratories. Read More

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January 2022

Secondary Polycythemia in Men Receiving Testosterone Therapy Increases Risk of Major Adverse Cardiovascular Events and Venous Thromboembolism in the First Year of Therapy.

J Urol 2022 06 20;207(6):1295-1301. Epub 2022 Jan 20.

University of Miami, Department of Urology, Miami, Florida.

Purpose: An unsafe hematocrit threshold for men receiving testosterone therapy (TT) has never been tested. This study seeks to determine whether secondary polycythemia among men receiving TT confers an increased risk of major adverse cardiovascular events (MACE) and venous thromboembolic events (VTE).

Materials And Methods: Using a multi-institutional database of 74 million patients, we identified 2 cohorts of men with low testosterone (total testosterone <350 ng/dl) who received TT and subsequently either developed polycythemia (5,887) or did not (4,2784). Read More

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Polycythemia secondary to renal cysts.

J Nephrol 2022 Jan 11. Epub 2022 Jan 11.

Division of Nephrology and Hypertension, Mayo Clinic, Scottsdale, AZ, USA.

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January 2022

Testosterone therapy and secondary erythrocytosis.

Int J Impot Res 2022 Jan 6. Epub 2022 Jan 6.

Dalhousie University Department of Urology, Halifax, NS, Canada.

Secondary erythrocytosis is one of the most common adverse events associated with testosterone therapy (TT). Upon encountering this, clinicians will often either adjust TT dosing, stop therapy, order a phlebotomy, or recommend a combination of these. Despite this, the evidence for secondary polycythemia causing harm during TT is scarce, and the hematocrit-based cutoffs present in multiple guidelines appear to be arbritrarily chosen. Read More

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January 2022