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What are the therapeutic options for previously treated myelofibrosis?
Expert Rev Hematol
November 2024
Department of Haematology, Guy's and St Thomas' NHS Foundation Trust, London, UK.
Article Synopsis
- The JAK/STAT signaling pathway disruption is key in myelofibrosis (MF), and JAK inhibitors (JAKi) have become crucial for treatment, guiding a shift in how MF is managed.
- This review discusses treatment options for patients who don't respond to first-line JAKi, showcasing alternative JAKi and other strategies based on thorough research from various databases.
- Ruxolitinib's benefits may decline after a few years, leading to complications; newer JAKi provide alternatives, while stem cell transplants can offer a cure for some, highlighting the need for future research on innovative treatments and collaborations among different stakeholders.
The role of corticosteroids in the current treatment paradigm for myelofibrosis.
Expert Opin Pharmacother
October 2024
Hematology Unit, Azienda Ospedaliera Annunziata, Cosenza, Italy.
Article Synopsis
- Myelofibrosis (MF) is a blood disorder characterized by bone marrow fibrosis and splenomegaly, with potential therapeutic benefits from steroids due to their anti-inflammatory properties, particularly for managing anemia and cytopenias.
- Clinical studies are evaluated regarding the safety and effectiveness of steroids in MF, including their use alongside immunomodulatory agents and JAK inhibitors.
- Expert opinions suggest that while the role of steroids in MF treatment is not fully understood, they may offer benefits in alleviating anemia and improving patient outcomes, especially when combined with other treatments.
Janus kinase inhibitor monotherapy and combination therapies for myelofibrosis: what's the current standard of care?
Expert Rev Hematol
November 2024
Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Introduction: JAK inhibitors (JAKi) have changed the treatment paradigm of myelofibrosis (MF). Currently, 4 JAKis are approved in the US as monotherapy (mono) to treat patients with MF. JAKis are also being studied in combination (combo) with novel agents.
View Article and Find Full Text PDFIdiopathic multicentric Castleman disease with marrow fibrosis and extramedullary hematopoiesis.
Eur J Haematol
December 2024
Division of Hematology, Dalhousie University, Halifax, Nova Scotia, Canada.
Background: Idiopathic multicentric Castleman disease (iMCD) is a rare inflammatory disorder mediated by excessive proinflammatory cytokine signaling, most notably by interleukin 6 (IL-6). IL-6-induced extramedullary hematopoiesis (EMH) has been reported in murine models of iMCD. Herein we present four cases of iMCD with EMH in humans.
View Article and Find Full Text PDFRecent advances in JAK2 inhibition for the treatment of myelofibrosis.
Expert Opin Pharmacother
June 2024
Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
Introduction: Myelofibrosis (MF) is a BCR-ABL-negative myeloproliferative neoplasm characterized by splenomegaly, constitutional symptoms, cytopenias, a potential for leukemic transformation, and increased mortality. Patients who are ineligible for stem cell transplant rely on pharmacologic therapies of noncurative intent, whose cornerstone consists of JAK inhibitors (JAKi). While current JAKi are efficacious in controlling symptoms and splenic volume, none meaningfully reduce clonal burden nor halt disease progression, and patients oftentimes develop JAKi intolerant, relapsed, or refractory MF.
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