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http://dx.doi.org/10.1182/blood.2024024199 | DOI Listing |
Ann Hematol
January 2025
Hematology and Hematopoietic Stem Cell Transplant Center, Department of Medicine and Surgery, University of Naples Federico II, Via S. Pansini 5, Naples, 80131, Italy.
Splenomegaly is a quite common clinical feature of Philadelphia (Ph) negative chronic myeloproliferative neoplasms (MPNs) and its presence may, in some cases, drives treatment decision. Most importantly, palpable splenomegaly is a minor criterion for both pre-fibrotic/early primary myelofibrosis and primary myelofibrosis (PMF) diagnosis, even if clinical assessment by physical examination is poorly reliable and accurate. On the other hand, despite the International Working Group-Myeloproliferative Neoplasms Research and Treatment and European LeukemiaNet guidelines defined spleen response criteria by palpation, they also recognized the highly subjective nature of spleen size assessment by physical examination, and recommended objective confirmation of volume reduction via computed tomography or magnetic resonance imaging (MRI).
View Article and Find Full Text PDFIntern Med
January 2025
Department of Internal Medicine 1, Shimane University Faculty of Medicine, Japan.
We herein report a 56-year-old man with severe hypocalcemia during ruxolitinib therapy for myelofibrosis transitioning from JAK2 mutation-positive polycythemia vera. Blood transfusions were administered every one to two weeks for ruxolitinib-induced anemia. Blood tests revealed hypocalcemia with low TRACP-5b, 25-hydroxyvitamin D (25 (OH) D), and 1,25-dihydroxyvitamin D (1,25 (OH) D) levels within the lower reference range.
View Article and Find Full Text PDFChildren (Basel)
December 2024
Department of Pediatrics, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan 704302, Taiwan.
Introduction: Polycythemia is a rare condition that can be either primary or secondary. We report a case of an adolescent with progressive hydronephrosis-induced polycythemia and low erythropoietin levels, along with a thorough literature review.
Report Of A Case: A 17-year-old girl with epilepsy had progressively elevated hemoglobin levels and low erythropoietin levels.
Med J Armed Forces India
December 2024
Professor & Senior Advisor, (Anaesthesia & Critical Care), Command Hospital (Eastern Command), Kolkata, West Bengal, India.
Eisenmenger syndrome (ES) is a rare congenital heart disease (CHD) that occurs in persons with large intracardiac anatomical defects which may go undetected for many years, consequently results in reduced life expectancies and high mortality in the third and fourth decade of life. Pregnancy in patients with CHD and pulmonary arterial hypertension (PAH) is presaged with challenges, and perioperative management of such cases remains a topic of debate. Our case had both, atypical presentation, not diagnosed until her third trimester of third pregnancy and a quick progression to a severe stage with PAH and CorPulmonale.
View Article and Find Full Text PDFSaudi Med J
December 2024
From the Department of Preventive Medicine (Alsharif, Alharazi), Directorate of Health Affairs; from the Hematology Department (Mansory, Badawi), Faculty of Medicine, King Abdulaziz University; and from the Hematology Research Unit (Mansory, Badawi), King Fahd Medical Research Center, King Abdulaziz University, and from the Blood Transfusion Services Unit (Badawi), King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia.
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.
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