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http://dx.doi.org/10.1016/j.ejim.2022.09.002 | DOI Listing |
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 PDFEur J Intern Med
January 2023
Service and Central Laboratory of Hematology, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL), Rue du Bugnon 46, Lausanne CH-1011, Switzerland. Electronic address:
Am J Case Rep
July 2020
Department of Clinical Hematology, King George's Medical University, Lucknow, Uttar Pradesh, India.
BACKGROUND Priapism is rarely reported as a complication in patients with essential thrombocythemia at presentation. We could find very few such cases of essential thrombocythemia while searching the literature. A combined modality of treatment is used in the form of chemotherapy and procedures like repeated aspiration and instillation of phenylephrine to treat essential thrombocythemia presenting with recurrent priapism.
View Article and Find Full Text PDFAnn Hematol
April 2016
Jane Anne Nohl Division of Hematology, Keck School of Medicine, University of Southern California, 1441 Eastlake Avenue, MC9172, Los Angeles, CA, 90033, USA.
Pegylated interferon α-2a (Peg-IFN) has been shown to induce hematologic and molecular responses in patients with the Philadelphia-negative myeloproliferative neoplasms (MPNs), including polycythemia vera (PV) and essential thrombocythemia (ET). We describe a series of patients with long-standing MPNs among whom Peg-IFN was initiated when they developed anemia and increased bone marrow reticulin fibrosis suggestive of early transformation to post-ET (PET) or post-PV (PPV) myelofibrosis (MF). Six patients were treated with Peg-IFN for a mean duration of 33.
View Article and Find Full Text PDFHaematologica
May 2013
Cambridge Institute for Medical Research and Department of Haematology, University of Cambridge, Cambridge, UK.
Subclones homozygous for JAK2V617F are more common and larger in patients with polycythemia vera compared to essential thrombocythemia, but their role in determining phenotype remains unclear. We genotyped 4564 erythroid colonies from 59 patients with polycythemia vera or essential thrombocythemia to investigate whether the proportion of JAK2V617F -homozygous precursors, compared to heterozygous precursors, is associated with clinical or demographic features. In polycythemia vera, a higher proportion of homozygous-mutant precursors was associated with more extreme blood counts at diagnosis, consistent with a causal role for homozygosity in polycythemia vera pathogenesis.
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