An analysis of the results of determination of erythropoietin in the blood plasma of patients with polycythemia vera and data on the kinetics of erythroid cell proliferation led to a conclusion that erythrocytapheresis more than bloodletting stimulated the production of erythropoietin and the cell proliferative potential. Activation of regenerative processes probably determined by deinhibition of the normal clone of bone marrow erythroid cells, can account for the mechanism of a therapeutic effect of erythrocytapheresis in patients with polycythemia vera.
Download full-text PDF |
Source |
---|
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 PDFJNCI Cancer Spectr
January 2025
Section on Medical Neuroendocrinology National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, 20892, MD, USA.
Head and neck paragangliomas (HNPGLs) are typically slow-growing, hormonally inactive tumors of parasympathetic paraganglia. Inactivation of prolyl-hydroxylase domain-containing 2 protein causing indirect gain-of-function of hypoxia-inducible factor-2α (HIF-2α), encoded by EPAS1, was recently shown to cause carotid body hyperplasia. We previously described a syndrome with multiple sympathetic paragangliomas caused by direct gain-of-function variants in EPAS1 (Pacak-Zhuang syndrome, PZS) and developed a corresponding mouse model.
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 PDFBackground: Due to environmental hypoxia on the high-altitude local residents often exhibit a compensative increase in hemoglobin concentration to maintain the body's oxygen supply. In certain people, the number of red blood cells continues to grow, resulting in high altitude polycythemia (HAPC) which is characterized by headache, disorientation, sleeplessness, and bone discomfort. HAPC is often associated with multiple complications, of which lower extremity arteriosclerosis obliterans (LEASO) is rare.
View Article and Find Full Text PDFAnn Hematol
January 2025
Department of Medicine, School of Clinical Medicine, LKS Faculty of Medicine, the University of Hong Kong, Hong Kong, China.
Polycythemia vera (PV) is characterized by clonal hematopoietic stem or progenitor cells with constitutively active somatic mutation(s) in the Janus kinase 2 gene. Phlebotomy (Phl) and aspirin are often used alone for low-risk PV patients. However, data from the Low-PV study demonstrated that Phl and aspirin may not be adequate for patients.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!