1,564 results match your criteria Polycythemia Secondary

Allogeneic haematopoietic cell transplantation for myelofibrosis: a real-life perspective.

Br J Haematol 2021 Apr 21. Epub 2021 Apr 21.

Service d'Hématologie Clinique et Thérapie Cellulaire, Hôpital Saint-Antoine, Sorbonne Université, INSERM UMRs 938, Paris, France.

Myelofibrosis (MF) is a clonal stem cell neoplasm with heterogeneous clinical phenotypes and well-established molecular drivers. Allogeneic haematopoietic stem cell transplantation (HSCT) offers an important curative treatment option for primary MF and post-essential thrombocythaemia/polycythaemia vera MF or secondary MF. With a disease course that varies from indolent to highly progressive, we are now able to stratify risk of mortality through various tools including patient-related clinical characteristics as well as molecular genetic profile. Read More

View Article and Full-Text PDF

Multi-arm Trial of Inflammatory Signal Inhibitors (MATIS) for hospitalised patients with mild or moderate COVID-19 pneumonia: a structured summary of a study protocol for a randomised controlled trial.

Trials 2021 Apr 12;22(1):270. Epub 2021 Apr 12.

Imperial College, London and Imperial NHS Trust, London, UK.

Objectives: The primary objective of MATIS is to determine the efficacy of ruxolitinib (RUX) or fostamatinib (FOS) compared to standard of care (SOC) with respect to reducing the proportion of hospitalised patients progressing from mild or moderate to severe COVID-19 pneumonia. Secondary objectives, at 14 and 28 days, are to: Determine the efficacy of RUX or FOS to reduce mortality Determine the efficacy of RUX or FOS to reduce the need for invasive ventilation or ECMO Determine the efficacy of RUX or FOS to reduce the need for non-invasive ventilation Determine the efficacy of RUX or FOS to reduce the proportion of participants suffering significant oxygen desaturation Determine the efficacy of RUX or FOS to reduce the need for renal replacement therapy Determine the efficacy of RUX and FOS to reduce the incidence of venous thromboembolism Determine the efficacy of RUX and FOS to reduce the severity of COVID-19 pneumonia [graded by a 9-point modified WHO Ordinal Scale* Determine the efficacy of RUX or FOS to reduce systemic inflammation Determine the efficacy of RUX or FOS to the incidence of renal impairment Determine the efficacy of RUX or FOS to reduce duration of hospital stay Evaluate the safety of RUX and FOS for treatment of COVID-19 pneumonia.

Trial Design: A multi-arm, multi-stage (3-arm parallel-group, 2-stage) randomised controlled trial that allocates participants 1:1:1 and tests for superiority in experimental arms versus standard of care. Read More

View Article and Full-Text PDF

Congenital erythrocytosis.

Eur J Haematol 2021 Apr 11. Epub 2021 Apr 11.

Department of Hematology, Postgraduate Institute of Medical Education & Research, Chandigarh, India.

Erythrocytosis, or increased red cell mass, may be labeled as primary or secondary, depending on whether the molecular defect is intrinsic to the red blood cells/their precursors or extrinsic to them, the latter being typically associated with elevated erythropoietin (EPO) levels. Inherited/congenital erythrocytosis (CE) of both primary and secondary types is increasingly recognized as the cause in many patients in whom acquired, especially neoplastic causes have been excluded. During the past two decades, the underlying molecular mechanisms of CE are increasingly getting unraveled. Read More

View Article and Full-Text PDF

Immunoglobulin A Nephropathy with Erythrocytosis: An Unusual Association.

Cureus 2021 Feb 19;13(2):e13437. Epub 2021 Feb 19.

Ophthalmology, Teaching Hospital Jaffna, Jaffna, LKA.

Immunoglobulin A (IgA) nephropathy is the commonest type of glomerular nephritis worldwide and is one of the leading causes of chronic kidney disease. Erythrocytosis is known to be associated with IgA nephropathy and other renal manifestations. We report a case of a 35-year-old male with grade 3 hypertensive retinopathy, erythrocytosis, and heavy proteinuria with an impaired renal function who was diagnosed as a case of erythrocytosis with IgA nephropathy and secondary focal segmental glomerulosclerosis. Read More

View Article and Full-Text PDF
February 2021

Clinical and Molecular Attributes of Patients With BCR/ABL1-negative Myeloproliferative Neoplasms in India: Real-world Data and Challenges.

Clin Lymphoma Myeloma Leuk 2021 Feb 23. Epub 2021 Feb 23.

Molecular Genetics, Dayanand Medical College and Hospital, Ludhiana, Punjab, India.

Introduction: Classic BCR/ABL1-negative myeloproliferative neoplasms (MPNs) are characterized by clinical and genetic heterogeneity and include 4 distinct constituents. Very little data on clinical presentation and epidemiology of the same is available from the Indian setting.

Patients And Methods: Patients referred to Hematology-Oncology from January 2018 to August 2020 with suspected MPNs were included in the analysis and prospectively followed-up. Read More

View Article and Full-Text PDF
February 2021

Thrombocytosis in children and adolescents-classification, diagnostic approach, and clinical management.

Ann Hematol 2021 Mar 12. Epub 2021 Mar 12.

Department of Pediatric Oncology, Hematology and Immunology and Hopp Children's Cancer Research Center (KiTZ), Heidelberg University and German Cancer Research Center (DKFZ), Heidelberg, Germany.

Secondary thrombocytosis is a frequent secondary finding in childhood infection and inflammation. Primary hereditary thrombocytosis may be caused by germline mutations within the genes encoding key regulators of thrombopoiesis, i.e. Read More

View Article and Full-Text PDF

Gene expression profile correlates with molecular and clinical features in patients with myelofibrosis.

Blood Adv 2021 Mar;5(5):1452-1462

Center for Genome Research.

Myelofibrosis (MF) belongs to the family of classic Philadelphia-negative myeloproliferative neoplasms (MPNs). It can be primary myelofibrosis (PMF) or secondary myelofibrosis (SMF) evolving from polycythemia vera (PV) or essential thrombocythemia (ET). Despite the differences, PMF and SMF patients are currently managed in the same way, and prediction of survival is based on the same clinical and genetic features. Read More

View Article and Full-Text PDF

Sex-based approach for the clinical impact of polycythaemia on cardiovascular outcomes in the general population.

Eur J Prev Cardiol 2020 Oct 4. Epub 2020 Oct 4.

Division of Cardiology, Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, 211 Eonju-ro Gangnam-gu, Seoul 06273, Republic of Korea.

Aims: Although the adverse cardiovascular effect of anaemia has been well described, the effect of polycythaemia on the cardiovascular outcomes of the general population remain unclear. The primary objective is to identify the association between polycythaemia and major adverse cardiovascular events (MACE), and the secondary objective is to identify the specific haemoglobin concentration more associated with an increased risk for MACE.

Methods And Results: This was a retrospective cohort study, 451 107 subjects were enrolled who underwent national health examinations from the Korean National Sample Cohort. Read More

View Article and Full-Text PDF
October 2020

Secondary Polycythaemia as an Unusual Cause of Falsely Elevated Prothrombin Time and International Normalized Ratio.

Kathmandu Univ Med J (KUMJ) 2020 Apr-Jun;18(70):203-204

Department of Haematology, Dhulikhel Hospital, Kathmandu University Hospital, Kathmandu University School of Medical Sciences, Dhulikhel, Kavre, Nepal.

Elevated Prothrombin time is common in conditions such as liver dysfunction and use of Vitamin K antagonists. Polycythaemia is among the uncommon causes of elevated prothrombin time. Elevated hematocrit greater than 55% leads to a decrease in plasma of the blood sample, thereby reducing the coagulating factors available. Read More

View Article and Full-Text PDF
February 2021

Bone marrow megakaryocytic activation predicts fibrotic evolution of Philadelphia-negative myeloproliferative neoplasms.

Haematologica 2020 11 19. Epub 2020 Nov 19.

Department of Life Sciences and Public Health, Universita Cattolica del Sacro Cuore, Largo F. Vito 1, 00168 Rome, Italy; Fondazione Policlinico Universitario A. Gemelli IRCCS, Largo A. Gemelli 8, 00168, Rome.

Philadelphia-negative chronic myeloproliferative neoplasms (MPNs) have been traditionally considered as indistinctly slowly progressing conditions; recent evidence proves that a subset of cases have a rapid evolution, so that MPNs' prognosis needs to be personalized. We identified a new morphological parameter, defined as Megakaryocytic Activation (M-ACT) based on the coexistence of megakaryocytic emperipolesis, megakaryocytes (MK) clusters formation and evidence of arrangement of collagen fibers around the perimeter of MK. We retrospectively analyzed the bone marrow biopsy of two MPNs cohorts of patients with polycythemia (PV) (n=64) and non-PV patients [including essential thrombocythemia (ET), and early/prefibrotic primary myelofibrosis (PMF)] (n=222). Read More

View Article and Full-Text PDF
November 2020

Polycythemia Vera and Essential Thrombocythemia Patients Exhibit Unique Serum Metabolic Profiles Compared to Healthy Individuals and Secondary Thrombocytosis Patients.

Cancers (Basel) 2021 Jan 27;13(3). Epub 2021 Jan 27.

Drug Discovery Unit, Instituto de Investigación Sanitaria La Fe, 46026 Valencia, Spain.

Most common myeloproliferative neoplasms (MPNs) include polycythemia vera (PV) and essential thrombocythemia (ET). Accurate diagnosis of these disorders remains a clinical challenge due to the lack of specific clinical or molecular features in some patients enabling their discrimination. Metabolomics has been shown to be a powerful tool for the discrimination between different hematological diseases through the analysis of patients' serum metabolic profiles. Read More

View Article and Full-Text PDF
January 2021

Bone Marrow and Peripheral Blood Findings in Patients Infected by SARS-CoV-2.

Am J Clin Pathol 2021 04;155(5):627-637

James Homer Wright Pathology Laboratories of the Massachusetts General Hospital and the Department of Pathology, Harvard Medical School, Boston, MA, USA.

Objectives: Coronavirus disease 19 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is associated with diverse clinical, including hematologic, abnormalities. We describe peripheral blood and bone marrow findings in deceased and living patients with COVID-19.

Methods: We examined bone marrows from 20 autopsies and 2 living patients with COVID-19 using H&E-stained slides and immunohistochemical stains. Read More

View Article and Full-Text PDF

A focal extramedullary hematopoiesis of the spleen in a patient with essential thrombocythemia presenting with a complicated postoperative course: a case report.

Surg Case Rep 2021 Jan 26;7(1):33. Epub 2021 Jan 26.

Division of Gastroenterological, Hepato-Biliary-Pancreatic, Transplantation and Pediatric Surgery, Department of Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano, 390-8621, Japan.

Background: Extramedullary hematopoiesis is a compensatory response occurring secondary to inadequate bone marrow function and is occasionally observed in essential thrombocythemia (ET). This disease usually presents as multifocal masses in the paravertebral or intra-abdominal region; however, formation of a focal mass in the liver or spleen is rare. In addition, ET is characterized by increased platelet count and shows a tendency toward thrombosis and, occasionally, bleeding. Read More

View Article and Full-Text PDF
January 2021

Prevention of recurrent thromboembolism in myeloproliferative neoplasms: review of literature and focus on direct oral anticoagulants.

Postgrad Med 2021 Feb 9:1-9. Epub 2021 Feb 9.

Division of Hematology, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (MF) are stem cell clonal neoplasms characterized by expansion of late myeloid cells. Thrombosis risk is elevated in MPNs and contributes significantly to morbidity and mortality. Current consensus guidelines make no specific recommendations regarding anticoagulant choice for the treatment of venous thromboembolism (VTE) in MPNs, with most evidence supporting the use of vitamin K antagonists (VKAs) for secondary prophylaxis. Read More

View Article and Full-Text PDF
February 2021

Effects of delayed cord clamping in intrauterine growth-restricted neonates: a randomized controlled trial.

Eur J Pediatr 2021 Jun 21;180(6):1701-1710. Epub 2021 Jan 21.

Department of Neonatology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, 249203, India.

The time of cord clamping in intrauterine growth-restricted (IUGR) neonates remains an area of uncertainty. This assessor-blinded randomized controlled trial compared the effects of delayed cord clamping (DCC) with early cord clamping (ECC) on the systemic blood flow (SBF) and cerebral hemodynamics in IUGR neonates of gestational age ≥28 weeks, not requiring resuscitation. Eligible newborns were randomized to DCC (cord clamping after 60 s; n=55) or ECC (cord clamping within 30 s; n=55) group immediately after delivery. Read More

View Article and Full-Text PDF

Portal Vein Thrombosis Secondary to Occult Polycythemia Vera.

Eur J Case Rep Intern Med 2020 2;7(12):002003. Epub 2020 Nov 2.

Hospital do SAMS, Lisbon, Portugal.

Portal vein thrombosis (PVT) is an uncommon finding in patients without cirrhosis. The underlying x\aetiology is challenging and the condition has a wide differential diagnosis. We present a case of PVT in an anaemic patient with chronic iron and folic acid deficiency masking underlying polycythemia vera (PV). Read More

View Article and Full-Text PDF
November 2020

Extrinsic tricuspid valve compression due to an aortic aneurysm causing significant right to left shunt via a patent foramen ovale: a case report.

Rizwan Ahmed

Eur Heart J Case Rep 2020 Dec 21;4(6):1-5. Epub 2020 Nov 21.

Institute of Cardiac Sciences, Sheikh Khalifa Medical City, Abu Dhabi, United Arab Emirates.

Background: Aortic aneurysms are known to cause compression of adjacent structures including the tracheobronchial tree, oesophagus, and recurrent laryngeal nerve. Extremely rarely, they can lead to compression of the tricuspid valve (TV) annulus. We describe a case where aortic aneurysm caused TV annulus compression and persistent right-to-left shunt through a patent foramen ovale (PFO). Read More

View Article and Full-Text PDF
December 2020

[Development of skin squamous cell carcinoma on the scalp in a hydroxycarbamide-treated polycythemia vera patient].

Rinsho Ketsueki 2020 ;61(12):1670-1672

Department of Hematology/Oncology, University of Yamanashi.

Hydroxycarbamide is a widely used cytoreductive agent for treating polycythemia vera and essential thrombocythemia. Although hydroxycarbamide is usually well tolerated by most patients for long periods, some patients experience mucosal or cutaneous adverse events. Furthermore, a series of case report have indicated an association of hydroxycarbamide use with the development of non-melanoma skin cancer, especially in western countries. Read More

View Article and Full-Text PDF
February 2021

Perinatal outcomes of pregnancies complicated by twin anemia-polycythemia sequence: a systematic review and meta-analysis.

Ultrasound Obstet Gynecol 2021 Jan 11. Epub 2021 Jan 11.

Twin Trust Centre for Research and Clinical Excellence, St George's University Hospitals NHS Foundation Trust, Blackshaw Road, London, SW17 0RE, UK.

Objective: To report the perinatal outcome in monochorionic diamniotic (MC) twin pregnancies complicated by twin anemia polycythemia sequence (TAPS).

Methods: Medline, Embase and Cochrane Library databases were searched. Inclusion criteria were non-anomalous MCDA twin pregnancies with a diagnosis of TAPS. Read More

View Article and Full-Text PDF
January 2021

Diagnostic Performance of Erythropoietin Levels in Polycythemia Vera: Experience at a Comprehensive Cancer Center.

Clin Lymphoma Myeloma Leuk 2021 Apr 5;21(4):224-229. Epub 2020 Nov 5.

Section of Benign Hematology, The University of Texas MD Anderson Cancer Center, Houston, TX. Electronic address:

Introduction: Considering the evolving diagnostic criteria of polycythemia vera (PV), we analyzed the utility of serum erythropoietin (EPO) as a predictive marker for differentiating polycythemia vera (PV) from other etiologies of erythrocytosis.

Patients And Methods: We conducted a retrospective study after a review of electronical medical records from January 2005 to December 2016 with diagnosis of erythrocytosis using International Classification of Disease-specific codes. To evaluate the diagnostic performance of EPO levels and JAK2-V617F mutation, we constructed a receiver-operated characteristic curve of sensitivity versus 1-specificity for serum EPO levels and JAK2-V617F mutation as predictive markers for differentiating PV from other causes of erythrocytosis. Read More

View Article and Full-Text PDF

Acute normovolemic hemodilution for a patient with secondary polycythemia undergoing aortic valve replacement due to severe aortic stenosis - A case report.

Anesth Pain Med (Seoul) 2020 Apr 29;15(2):181-186. Epub 2020 Apr 29.

Department of Anesthesiology and Pain Medicine, Ulsan University Hospital, Ulsan, Korea.

Background: A high hematocrit level in patients with erythrocytosis is linked with increased blood viscosity and increased risk of thromboembolism. Therefore, it is necessary to adequately lower the hematocrit level before performing a high-risk surgery.

Case: A 67-year-old male was scheduled for aortic valve replacement due to severe aortic stenosis. Read More

View Article and Full-Text PDF

Might a high hemoglobin mass be involved in non-cardiogenic pulmonary edema? The case of the chronic maladaptation to high-altitude in the Andes.

Med Hypotheses 2021 Jan 24;146:110418. Epub 2020 Nov 24.

School of Biological Sciences & Engineering. Yachay Tech University, San Miguel de Urcuquí, Ecuador. Electronic address:

Exposure to hypoxic environments when ascending at high altitudes may cause life-threatening pulmonary edema (HAPE) due to a rapid accumulation of extracellular fluid flooding in the pulmonary alveoli. In Andeans, high-altitude adaptation occurs at the expense of being more prone to chronic mountain sickness: relative hypoventilation, excess pulmonary hypertension, and secondary polycythemia. Because HAPE prevalence is high in the Andes, we posit the hypothesis that a high hemoglobine mass may increase HAPE risk. Read More

View Article and Full-Text PDF
January 2021

Clinical insights into the origins of thrombosis in myeloproliferative neoplasms.

Blood 2021 Mar;137(9):1145-1153

Division of Hematology and Medical Oncology and.

Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are hematopoietic stem cell disorders that are defined by activating mutations in signal transduction pathways and are characterized clinically by the overproduction of platelets, red blood cells, and neutrophils, significant burden of disease-specific symptoms, and high rates of vascular events. The focus of this review is to critically reevaluate the clinical burden of thrombosis in MPNs, to review the clinical associations among clonal hematopoiesis, JAK2V617F burden, inflammation, and thrombosis, and to provide insights into novel primary and secondary thrombosis-prevention strategies. Read More

View Article and Full-Text PDF

Austrian recommendations for the management of essential thrombocythemia.

Wien Klin Wochenschr 2021 Jan 19;133(1-2):52-61. Epub 2020 Nov 19.

Department of Internal Medicine I, Division of Hematology and Blood Coagulation, Medical University of Vienna, Vienna, Austria.

According to the World Health Organization (WHO) classification, essential (primary) thrombocythemia (ET) is one of several Bcr-Abl negative chronic myeloproliferative neoplasms (MPN). The classical term MPN covers the subcategories of MPN: ET, polycythemia vera (PV), primary myelofibrosis (PMF), and prefibrotic PMF (pPMF). ET is marked by clonal proliferation of hematopoietic stem cells, leading to a chronic overproduction of platelets. Read More

View Article and Full-Text PDF
January 2021

Erythromelalgia Caused by Polycythemia Vera Combined with Primary Aldosteronism.

Eur J Case Rep Intern Med 2020 13;7(11):001852. Epub 2020 Aug 13.

Postgraduate Clinical Training Centre, Shimane University Hospital, Shimane, Japan.

A 61-year-old woman with a history of primary aldosteronism caused by unilateral hyperplasia of the adrenal gland presented with a 2-week history of redness and severe pain in the right thumb and thenar regions. She had initially visited a dermatologist and was diagnosed with cellulitis and treated with cefditoren pivoxil for 5 days, but there was no improvement. The pain worsened and was accompanied by a burning sensation. Read More

View Article and Full-Text PDF

Hypertryptasemia and Mast Cell-Related Disorders in Severe Osteoporotic Patients.

Mediators Inflamm 2020 20;2020:5785378. Epub 2020 Oct 20.

Endocrinology and Diabetology Service, IRCCS Istituto Ortopedico Galeazzi, Milan, Italy.

Purpose: Systemic mastocytosis (SM) is characterized by a clonal proliferation of neoplastic mast cells (MCs) in one or more extracutaneous organs including the bone marrow (BM). SM is often associated with osteoporosis (OP) and fractures. Hypertryptasemia usually occurs in SM. Read More

View Article and Full-Text PDF
October 2020

[Polycythemia vera as a cause for progressive cognitive impairment].

Dtsch Med Wochenschr 2020 11 3;145(22):1625-1627. Epub 2020 Nov 3.

Johanniter-Kliniken Bonn, Bonn, Klinik für Geriatrie mit Neurologie und Tagesklinik.

History: A 82-years old woman was admitted with a progressive cognitive decline for further investigations and treatment.

Findings And Diagnosis: In the computed tomography of the brain findings of subcortical artherosclerotic encephalopathy (SAE) were present. Laboratory findings revealed elevated hemoglobin-levels (19. Read More

View Article and Full-Text PDF
November 2020

Polycythaemia following treatment of lymphoplasmacytic lymphoma.

BMJ Case Rep 2020 Oct 30;13(10). Epub 2020 Oct 30.

Clinical Hematology, King George's Medical University, Lucknow, India.

A 61-year-old man presented to the department of clinical haematology in February 2016 with symptomatic anaemia, generalised lymphadenopathy and hepatomegaly. Routine investigations showed severe anaemia with the presence of lymphoplasmacytoid cells in the peripheral smear, and bone marrow examination with IHC and serum protein electrophoresis confirmed diagnosis of lymphoplasmacytic lymphoma. The patient received supportive transfusion therapy and combination chemotherapy. Read More

View Article and Full-Text PDF
October 2020

Generalized Chorea Due to Secondary Polycythemia Responding to Phlebotomy.

J Mov Disord 2021 Jan 31;14(1):89-91. Epub 2020 Oct 31.

Department of Transfusion Medicine, Sree Chitra Tirunal Institute of Medical Sciences and Technology, Kerala, India.

View Article and Full-Text PDF
January 2021