Causal relationship between 731 immune cells and the risk of myeloproliferative neoplasms: A 2-sample bidirectional Mendelian randomization study.

Myeloproliferative neoplasms (MPN) are chronic hematological disorders marked by the abnormal proliferation of bone marrow cells. The most commonly encountered forms are polycythemia vera (PV), primary myelofibrosis (PMF), and essential thrombocythemia (ET). These disorders are generally associated with increases in blood components, which can lead to conditions like splenomegaly, thrombosis, bleeding tendencies, and a heightened risk of progressing to acute leukemia.

Characteristics, blood counts, treatments, and clinical outcomes of 5871 patients with polycythemia vera treated in US community practices.

Objective: This study aimed to describe clinical characteristics-including blood counts and pharmacologic cytoreductive treatment patterns-and outcomes after 6 months of hydroxyurea (HU) treatment among patients with polycythemia vera (PV) in US community practices.

Methods: This retrospective observational study included adult patients with a PV diagnosis (1JAN2008-31JAN2020) and ≥2 postdiagnosis visits in the iKnowMed electronic health record database (US Oncology Network and non-Network clinics). Suboptimal HU response required ≥1 criterion after ≥3 months of treatment: white blood cell count (WBC) >10 × 10/L, platelet count >400 × 10/L, and/or hematocrit >45%.

Dysarthria and Altered Mental Status Following Electroconvulsive Therapy (ECT) in a Patient With Polycythemia Vera: A Case Report.

Although rare, neurological complications following electroconvulsive therapy (ECT) can have significant consequences. Recognizing patient populations that may be at high risk of neurological complications following ECT can reduce the likelihood of these patients developing such complications. We report a case of a patient with a history of polycythemia vera presenting with a worsening episode of depression with psychotic features who demonstrated a significant change in neurological status following ECT, suspected to be due to cerebral ischemia.

High Pulmonary Hypertension Risk by Echocardiogram Shortens Survival in Polycythemia Vera.

Pulmonary Hypertension (PH) is a known complication of myeloproliferative neoplasms (MPN) with estimated prevalence as high as 50%. Patients with Polycythemia Vera (PV) report a wide spectrum of symptoms that significantly overlap with those reported by patients with PH. Yet, it is not known how PH affects outcomes and survival in patients with PV.

Biological Markers of Myeloproliferative Neoplasms in Children, Adolescents and Young Adults.

Myeloproliferative neoplasms (MPNs) are clonal hematopoietic cancers characterized by hyperproliferation of the myeloid lineages. These clonal marrow disorders are extremely rare in pediatric patients. MPN is reported to occur 100 times more frequently in adults, and thus research is primarily focused on this patient group.

Coexistence of tubulocystic renal cell carcinoma and polycythemia vera: A rare case report.

Tubulocystic Renal Cell Carcinoma (TC-RCC) and Polycythemia Vera (PV) are both infrequent medical conditions. TC-RCC was recognized as a distinct subtype of kidney cancer by the World Health Organization in 2016, while PV is a rare myeloproliferative neoplasm distinguished by the excessive production of red blood cells. The coexistence of these two conditions is exceptionally uncommon and lacks comprehensive documentation.

JAK2-V617F mutation among blood donors: A meta-analysis.

Objectives: To systematically review evidence on the prevalence of the JAK2V617F (JAK2) mutation and polycythemia vera (PV) among all blood donors, focusing on those with elevated hematocrit. Although blood donors are generally healthy, considering a preclinical stage of myeloproliferative neoplasm, especially in those with polycythemia, is crucial. Evidence on managing these donors is limited.

Polycythemia vera with acute coronary syndrome and bleeding as initial presentation: A case report and literature review.

Polycythemia vera (PV) is a chronic myeloproliferative disorder characterized by increased red blood cell mass, leading to a heightened risk for thrombosis and hemorrhage. While thrombotic complications such as stroke, deep vein thrombosis, and pulmonary embolism are commonly associated with PV, coronary artery syndromes, as the initial presentation, are rare. Here, we present the case of a 73-year-old male who presented with severe chest pain and was diagnosed with non-ST-elevation myocardial infarction (NSTEMI).

Molecular profiling in MPN: who should have it and why?

Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs) are a group of blood cancers that result from somatic mutations in hematopoietic stem cells, causing constitutive activation of JAK-STAT signaling pathways with consequent overproduction of 1 or more myeloid lineages. The initiating event in MPN pathogenesis is a genetic mutation, and consequently molecular profiling is central to the diagnosis, risk stratification, and, increasingly, monitoring of therapy response in persons with MPN. In this review we summarize current approaches to molecular profiling of classical MPNs (essential thrombocythemia, polycythemia vera, and myelofibrosis), using illustrative clinical case histories to demonstrate how genetic analysis is already fully integrated into MPN diagnostic classification and prognostic risk stratification.

Pegylated interferon: the who, why, and how.

Interferon alpha (IFN-α) is a fascinating molecule with many biological properties yet to be fully understood. Among these properties, several have demonstrated usefulness for targeting malignant cells, including hematopoietic cells from patients with myeloproliferative neoplasms. Indeed, IFN-α has been used for decades across all myeloproliferative neoplasms, but only recently a new form, ropegIFN-α2b, was approved to treat patients with polycythemia vera.

A rare case of spinal myeloid sarcoma.

Background: Myeloid sarcoma (MS), a rare extramedullary tumor composed of myeloid blast cells, is classified by the World Health Organization as a subtype of acute myeloid leukemia (AML). Isolated, primary, or nonleukemic MS has an incidence of 2/1,000,000 adults and constitutes only 0.7% of all AML cases.

Essential Thrombocytosis in a Postmenopausal Female a Rare Cause of Aquagenic Pruritus.

Essential thrombocytosis (ET) is a hematopoietic stem cell disorder in which the overproduction of platelets without a definable cause occurs. Its incidence is 1-2 per 100,000 population with female preponderance. These myeloproliferative disorders are associated with many dermatological manifestations such as pruritus and erythromelalgia.

Arterial Stiffness as a New Predictor of Clinical Outcome in Patients with Polycythemia Vera.

Thrombotic adverse events and disease progression are crucial in Polycythemia Vera (PV), as it stands as the leading cause of mortality. The pulse wave velocity (PWV) is a valuable indicator of arterial aging and often plays a significant independent role in contributing to cardiovascular adverse events (CV-AEs). The aim of this study was to examine the relationship between PWV and critical vascular function parameters, with the goal of identifying new predictive factors of vascular damage and exploring a potential connection with disease progression.

Pharmacokinetics and Pharmacodynamics of Rusfertide, a Hepcidin Mimetic, Following Subcutaneous Administration of a Lyophilized Powder Formulation in Healthy Volunteers.

Background And Objective: Hepcidin, an endogenous peptide hormone, binds to ferroportin and is the master regulator of iron trafficking. Rusfertide, a synthetic peptide, is a potent hepcidin mimetic. Clinical studies suggest rusfertide may be effective in the treatment of polycythemia vera.

A higher V617F allele burden may be a risk factor for hemorrhagic events in younger patients with polycythemia vera.

Objectives: Hemorrhagic events are a rare but potentially fatal complication in patients with polycythemia vera (PV).

Methods: We analyzed the characteristics of hemorrhagic events in 267 patients with PV.

Results: A median follow-up of 4.