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Myeloproliferative Neoplasms: Challenging Dogma.
J Clin Med
November 2024
Hematology Division, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA.
Myeloproliferative neoplasms, polycythemia vera, essential thrombocytosis, and primary myelofibrosis are a unique group of clonal hematopoietic stem cell neoplasms that share somatic, gain-in-function driver mutations in 2, , and . As a consequence, these disorders exhibit similar phenotypic features, the most common of which are the ceaseless production of normal erythrocytes, myeloid cells, platelets alone or in combination, extramedullary hematopoiesis, myelofibrosis, and a potential for leukemic transformation. In the case of polycythemia vera and essential thrombocytosis, however, prolonged survival is possible.
View Article and Find Full Text PDFA Case of Renal Artery Thrombosis With Concurrent Adrenal Hemorrhage in Polycythemia Vera.
Cureus
September 2024
Nephrology, University of Iowa Hospitals and Clinics, Iowa, USA.
Article Synopsis
- * Diagnosis typically involves checking for high hemoglobin levels, low erythropoietin, and the presence of a specific mutation (JAK2) to confirm the disease.
- * A case study of a 68-year-old woman with PV showed how she developed renal artery thrombosis leading to acute kidney injury, requiring careful management of anticoagulation to balance the risks of both thrombosis and bleeding.
Response, complications and risk of leukemic transformation of phosphorus-32p treatment in philadelphia-negative chronic myeloproliferative syndromes.
Rev Esp Med Nucl Imagen Mol (Engl Ed)
September 2024
Servicio de Hematología y Hemoterapia, Hospital Universitario de Álava, Osakidetza, Vitoria-Gasteiz, Álava, Spain.
Article Synopsis
- The study looked at how well Phosphorus-32 P treatment worked for 17 older patients with a specific type of blood cancer that is not caused by a gene called Philadelphia.
- Most patients improved after treatment, and some had to get multiple doses because the first one wasn't enough.
- The researchers found that there were some side effects, like anemia, but there were no serious problems like developing leukemia.
Characterization of myeloproliferative neoplasms based on genetics only and prognostication of transformation to blast phase.
Leukemia
December 2024
MLL Munich Leukemia Laboratory, Munich, Germany.
Article Synopsis
- Myeloproliferative neoplasms (MPN) are diverse blood disorders with abnormal blood cell production and a risk of progressing to severe phases, such as blast phase.
- The WHO 2022 categorization recognizes distinct MPNs like chronic myeloid leukemia and others, but diagnosing these conditions can be tricky due to overlapping features and missing clinical information.
- A new model based on 12 genetic markers has been created to help accurately classify MPN patients; it shows that genetic mutations can change as the disease progresses, emphasizing the need for enhanced genetic testing at both diagnosis and during disease advancement.
Impact of Phlebotomy on Quality of Life in Low-Risk Polycythemia Vera.
J Clin Med
August 2024
Faculty of Science, Medicine and Health, University of Wollongong, Wollongong, NSW 2522, Australia.
Polycythemia vera is an indolent myeloproliferative disorder that predisposes patients to venous and arterial thrombosis and can transform into myelofibrosis and acute myeloid leukemia. Consistent phlebotomy prevents life-threatening cerebrovascular and coronary artery disease and prolongs survival in low-risk polycythemia vera (patients under 60 years without thrombosis). However, despite its effectiveness in preventing serious complications, phlebotomy does not necessarily enhance the quality of life (QoL).
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