Topic: Recent Advances in the Treatment of Polycythemia Vera (PV)
A Special Issue of Rare Disease and Orphan Drugs Journal
ISSN 2771-2893 (Online)
Submission deadline: 15 Dec 2024
Guest Editor(s)
Special Issue Introduction
Significant progress has been made in understanding the pathophysiology of Polycythemia Vera (PV). However, in underlying secondary polycythemia, also known as secondary erythrocytosis, aside from hypoxic conditions, the mechanisms remain incompletely understood. The impact of rheological perturbations on the oxygen supply and end-organ functions has also been studied to a limited extent.
The red blood mass is relatively constant and strictly regulated through complex mechanisms. RBCs are renewed approximately every 100 days, with old RBCs being sequestered by the spleen, liver, and bone marrow. In adults, RBCs are produced by the bone marrow, while during fetal life and the first month of life, the liver and spleen are active sites of erythropoiesis. Under normal conditions, erythropoiesis adapts to environmental factors, mainly oxygen concentration, partial pressure of oxygen, and cardiovascular function. In pathological conditions, the RBC mass can vary. Anemia is the most common disorder, which can be secondary to genetic defects or acquired dyserythropoiesis. In contrast, increased RBC mass, also known as polycythemia or erythrocytosis, is less frequent and can be inherited or caused by environmental conditions, organ dysfunctions, or epigenetic mechanisms. This increased RBC mass can result from oxygen reduction and improper regulation of erythropoiesis.
Advancements in the diagnostic strategy of PV have significantly improved disease management. The diagnosis of PV as a malignant or non-malignant disease can affect treatment decisions, as not all detected mutations necessitate specific chemotherapy treatments.
Cardiovascular or cerebrovascular complications, depending on the patient's age, may become the primary cause of death.
The various rheological abnormalities, increased RBC mass, and enhanced RBC adhesiveness to the endothelium may explain why the major morbidity or mortality in PV is associated with thrombosis and vascular occlusion. Reducing RBC mass generally benefits patients, but specifically targeting RBC adhesion could further reduce the thrombotic risk of a non-malignant disease while avoiding chemotherapy side effects and hemodynamic changes in older patients treated with erythro-pheresis.
Topics of Interest:
● Genetic and Molecular Insights: Studies on gene mutations, molecular pathways, and the genetic basis of PV.
● Diagnostic Techniques: Innovations in diagnostic methods, including biomarkers, imaging, and novel assays.
● Treatment Strategies: Advances in therapeutic approaches, including pharmacological treatments, phlebotomy, and bone marrow transplants.
● Clinical Trials and Case Studies: Reports on recent clinical trials, case studies, and patient management strategies.
● Pathophysiology and Epidemiology: Research on the underlying mechanisms and epidemiological trends of PV.
● Complications and Comorbidities: Investigations into the complications associated with PV and strategies for their management.
● Patient Quality of Life: Studies on the impact of PV on patient quality of life and approaches to improve patient outcomes.
We look forward to your valuable contributions to this Special Issue and to advancing the understanding and treatment of Polycythemia Vera.
The red blood mass is relatively constant and strictly regulated through complex mechanisms. RBCs are renewed approximately every 100 days, with old RBCs being sequestered by the spleen, liver, and bone marrow. In adults, RBCs are produced by the bone marrow, while during fetal life and the first month of life, the liver and spleen are active sites of erythropoiesis. Under normal conditions, erythropoiesis adapts to environmental factors, mainly oxygen concentration, partial pressure of oxygen, and cardiovascular function. In pathological conditions, the RBC mass can vary. Anemia is the most common disorder, which can be secondary to genetic defects or acquired dyserythropoiesis. In contrast, increased RBC mass, also known as polycythemia or erythrocytosis, is less frequent and can be inherited or caused by environmental conditions, organ dysfunctions, or epigenetic mechanisms. This increased RBC mass can result from oxygen reduction and improper regulation of erythropoiesis.
In terms of thrombotic risk, RBC abnormalities are major risk factors. An elevated platelet count may complicate the differentiation of PV from Essential Thrombocythemia. For management, simple and inexpensive treatments, such as aspirin and blood mass reduction, may suffice. Although chemotherapies, from hydroxyurea to newer anti-myeloproliferative drugs, have been used in the past, there are limited multi-center trials reported. Treatment for secondary polycythemia is typically described in recommendations or guidelines.
Advancements in the diagnostic strategy of PV have significantly improved disease management. The diagnosis of PV as a malignant or non-malignant disease can affect treatment decisions, as not all detected mutations necessitate specific chemotherapy treatments.
Cardiovascular or cerebrovascular complications, depending on the patient's age, may become the primary cause of death.
The various rheological abnormalities, increased RBC mass, and enhanced RBC adhesiveness to the endothelium may explain why the major morbidity or mortality in PV is associated with thrombosis and vascular occlusion. Reducing RBC mass generally benefits patients, but specifically targeting RBC adhesion could further reduce the thrombotic risk of a non-malignant disease while avoiding chemotherapy side effects and hemodynamic changes in older patients treated with erythro-pheresis.
Topics of Interest:
● Genetic and Molecular Insights: Studies on gene mutations, molecular pathways, and the genetic basis of PV.
● Diagnostic Techniques: Innovations in diagnostic methods, including biomarkers, imaging, and novel assays.
● Treatment Strategies: Advances in therapeutic approaches, including pharmacological treatments, phlebotomy, and bone marrow transplants.
● Clinical Trials and Case Studies: Reports on recent clinical trials, case studies, and patient management strategies.
● Pathophysiology and Epidemiology: Research on the underlying mechanisms and epidemiological trends of PV.
● Complications and Comorbidities: Investigations into the complications associated with PV and strategies for their management.
● Patient Quality of Life: Studies on the impact of PV on patient quality of life and approaches to improve patient outcomes.
We look forward to your valuable contributions to this Special Issue and to advancing the understanding and treatment of Polycythemia Vera.
Submission Deadline
15 Dec 2024
Submission Information
For Author Instructions, please refer to https://www.oaepublish.com/rdodj/author_instructions
For Online Submission, please login at https://oaemesas.com/login?JournalId=rdodj&IssueId=rdodj240820
Submission Deadline: 15 Dec 2024
Contacts: Cyndi Guan, Assistant Editor, assistant_editor@rdodjournal.com
