: Recent advances in the prognostic scheme and treatment of primary and secondary myelofibrosis (MF) have resulted in an overwhelming amount of clinical information to assimilate. The authors believe a comprehensive review that summarizes the most recent published literature, could serve as guidelines for the practicing hematologist.: The authors provide a summary of landmark articles regarding epidemiology, symptoms, and pathogenesis of disease. The authors conducted a systematic literature review to answer questions regarding differences between primary myelofibrosis (PMF) and secondary myelofibrosis (SMF), appropriate use and selection of the current risk-stratification models, early versus late treatment of MF and current practices in allogeneic hematopoietic stem cell transplantation (allo-HCT) for MF. The authors conclude the article with their clinical opinion based on their experience and literature review. The purpose of this article is to identify current practices, address any variation, identify and investigate conflicting results and produce statements to guide decision-making.: In this section, the authors advocate for and provide examples of a standardized way of incorporating future discoveries in the pathogenesis and risk-stratification models of MF. They also discuss the importance of using only one risk-stratification model for PMF and one for SMF and their reasoning for early instead of late treatment of MF.
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http://dx.doi.org/10.1080/17474086.2020.1691519 | DOI Listing |
Zhonghua Yi Xue Za Zhi
January 2025
Department of Hematology, the Second Hospital of Tianjin Medical University, Tianjin300211, China.
To investigate the effect of ten-eleven translocation methylcytosine dioxygenase 2 (TET2) gene mutations on the secondary myelofibrosis (SMF) of JAK2 myeloproliferative neoplasms (MPN) patients. A retrospective collection was conducted on MPN patients with JAK2 mutation detected by second-generation sequencing in the Department of Hematology, the Second Hospital of Tianjin Medical University. TET2JAK2 MPN patients were selected as the mutant group, and TET2JAK2 MPN patients matched for age and gender were selected as the non-mutant group.
View Article and Find Full Text PDFOncol Ther
January 2025
Hematology, Department of Translational and Precision Medicine, Policlinico Umberto I-Sapienza University, Via Benevento 6, 00161, Rome, Italy.
Introduction: Myelofibrosis (MF) is often characterized by a multifactorial anemia determined, in part, by bone marrow (BM) fibrosis, extramedullary erythropoiesis and splenomegaly. Ruxolitinib (RUX) is the first-in-class janus kinase 2 (JAK2) inhibitor approved for treatment of MF, proved to reduce spleen volume and decrease symptom burden. The red cell distribution width (RDW) is the measure of erythrocyte volume variability (anisocytosis).
View Article and Find Full Text PDFBlood Adv
January 2025
Univ Angers, Nantes Université, CHU Angers, Inserm, CNRS, CRCI2NA, F-49000, Angers, France, ANGERS, France.
Patients with essential thrombocythemia (ET) have a chronic evolution with a risk of hematological transformation associated with a dismal outcome. Since patients with resistance or intolerance have an adverse prognosis, it is important to identify which patient will respond to first-line treatment. We therefore aim to describe the association between additional mutations and response to first-line treatment in patients with ET.
View Article and Find Full Text PDFLeuk Res Rep
December 2024
Internal Medicine Department, Institut Jules Bordet - Hôpital Universitaire de Bruxelles (H.U.B.), Université Libre de Bruxelles (ULB), Brussels, Belgium.
We describe the case of a female patient with May-Hegglin syndrome who developed peripheral T-cell lymphoma not otherwise specified. The patient presents with systemic lupus erythematous phenotype and myelofibrosis secondary to T-cell lymphoma. Peripheral T-cell lymphoma not otherwise specified, represents 25 % of all peripheral T-cell lymphoma.
View Article and Find Full Text PDFJ Adv Pract Oncol
May 2024
Levine Cancer Institute, Atrium Health, Charlotte, North Carolina.
Myelofibrosis is a myeloproliferative neoplasm characterized by the buildup of fibrous scar tissue in the bone marrow occurring secondary to the secretion of inflammatory cytokines, leading to cytopenias, dysfunctional hematopoiesis, and constitutional symptoms. One of the pathologic mechanisms that underlies myelofibrosis is aberrant activation of the Janus kinase (JAK)-STAT pathway. Targeting the JAK-STAT pathway via JAK inhibition can lead to significant improvements in spleen volume reduction and symptom improvement in intermediate- and high-risk myelofibrosis.
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