Publications by authors named "Dupriez B"
Article Synopsis
- A phase 2 trial investigated the effectiveness of ruxolitinib in improving disease-free survival (DFS) in myelofibrosis patients prior to transplantation, involving 76 participants.
- Of the patients, 64 had donors, with significant transplantation success: 92% proceeded to transplant after ruxolitinib treatment, and 31% showed a partial response beforehand.
- After one year, overall survival was 68% and DFS was 55%, with better outcomes linked to HLA-matched sibling donors compared to unrelated donors.
Article Synopsis
- The study looked at how different gene mutations affect the health of people with myelofibrosis, a type of blood disease.
- Researchers analyzed 479 patients and grouped them based on specific mutations to see how these groups relate to worsening conditions or death.
- They found that mutations in certain genes like TP53 and high-risk genes made it more likely for patients to get worse or die, while a mutation in the ASXL1 gene alone didn’t have a significant negative impact.
Article Synopsis
- The study focuses on the long-term outcomes of 62 patients with myeloproliferative neoplasm-associated myelofibrosis treated with pegylated interferon-α2a, highlighting improvements in survival rates and mutational patterns.
- The median follow-up was 58 months, and nearly half of the patients were alive at the time of analysis, with those receiving pegylated interferon showing better survival compared to historical cohorts.
- Next-generation sequencing revealed that many patients had non-driver mutations, with the presence of additional mutations linked to poorer survival, reinforcing the potential benefits of pegylated interferon in this patient population.
Article Synopsis
- A retrospective study analyzed the real-life effects of eltrombopag in 46 patients with relapsed/refractory aplastic anemia who were not candidates for transplantation or antithymocyte globulin treatment.
- The study found that by the last follow-up, nearly half of the patients were still on the medication, with 49% achieving transfusion independence at various time points.
- The results showed significant hematologic improvements, with about 64% of patients experiencing response in at least one blood lineage and some achieving notable trilineage responses.
Article Synopsis
- Primary myelofibrosis (PMF) is a serious blood disorder characterized by bone marrow fibrosis, which disrupts normal blood cell production.
- Although mutations like JAK2V617F are found in many PMF patients, the exact cause of the disease is still unclear.
- Research focusing on bone marrow mesenchymal stromal cells (BM-MSC) aims to uncover how these cells contribute to the disrupted environment that affects hematopoietic stem and progenitor cell function in PMF.
Article Synopsis
- Primary myelofibrosis is a type of blood cancer that can lead to leukemia, characterized by abnormal blood cell production and changes in bone marrow structure.
- This study found that bone marrow mesenchymal stromal cells (MSC) in patients with primary myelofibrosis have unique molecular changes and increased osteogenic potential, unlike those in other blood disorders.
- The research indicates that targeting both blood cells and these altered MSCs may enhance treatment effectiveness for patients with primary myelofibrosis.
Article Synopsis
- Primary myelofibrosis is a condition involving abnormal blood cell production, characterized by issues such as dysfunctional megakaryocytes and changes in bone marrow and spleen structure.
- The study focuses on the role of CD9, a protein that impacts megakaryocyte formation and interactions with the bone marrow environment, finding that its deregulation is linked to the severity of the disease.
- Results indicate that CD9 influences both the abnormal behaviors of megakaryocytes in primary myelofibrosis and their interactions with stromal cells, supporting a theory that poor cell interaction contributes to the disease's progression.
Myeloproliferative neoplasm-related myelofibrosis is associated with cytopenic or proliferative phases, splenomegaly and constitutional symptoms. Few effective treatments are available and small series suggested that interferon could be an option for myelofibrosis therapy. We performed a retrospective study of pegylated-interferon α-2a (Peg-IFNα-2a) therapy in myelofibrosis.
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- * Key updates include clearer definitions of red cell transfusion dependency and the use of the Myeloproliferative Neoplasm Symptom Assessment Form for tracking symptom changes.
- * Six response categories are outlined, including complete and partial remission, as well as clinical and symptom improvements, with additional guidelines for assessing disease progression and remission status.
Article Synopsis
- The study investigates whether survival rates for patients with primary myelofibrosis (PMF) have improved over time, comparing data from two periods: 1980-1995 and 1996-2007.
- Results show that patients diagnosed between 1996 and 2007 had a median survival of 6.5 years, compared to 4.6 years for those diagnosed earlier, indicating significant improvements in survival rates.
- However, the improvements in survival were not observed in high-risk patients, suggesting that new therapies may need to focus on this subgroup to enhance their outcomes.
Article Synopsis
- * 79 patients were analyzed, with a median follow-up of 56 months, showing a 5-year overall survival rate of 51%, increasing to 62% for those sensitive to salvage treatment.
- * The results indicate that this treatment strategy is promising for refractory PCNSL without causing significant neurocognitive decline in patients under 65 years old.
Article Synopsis
- Primary myelofibrosis (PMF) is marked by abnormal changes in blood cell production and an increase in certain cells (hematopoietic progenitors) linked to inflammation, primarily driven by the FL/Flt3 signaling pathway.
- The study found that FL levels were elevated in PMF patients, which led to increased Flt3 receptor activation on megakaryocytic (MK) cells, contributing to functional issues in blood cell formation (dysmegakaryopoiesis) through the activation of the p38-MAPK pathway.
- Inhibition of the FL/Flt3 pathway or the p38-MAPK signaling restored normal megakaryocyte development and migration, suggesting new potential therapeutic targets to address the
The term RBC-transfusion-dependence is widely-used by hematologists to describe a condition of severe anemia typically arising when erythropoiesis is reduced such that a person continuously requires ≥1 RBC-transfusions over a specified interval. Defining a person as RBC-transfusion-dependent has important implications in diverse hematological disorders especially because it strongly-correlated with decreased survival. Conversely, becoming RBC-transfusion-independent or receiving fewer RBC-transfusions over a specified interval is defined as improvement or response in many disease- and/or therapy-setting.
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- * A study of 172 patients identified significant time-dependent predictors of mortality, including anemia, high leukocyte and low platelet counts, and other clinical factors.
- * Monitoring for these adverse conditions, particularly leukocytosis, during treatment can help assess disease severity and inform treatment decisions.
Article Synopsis
- Therapeutic decision-making for primary myelofibrosis (PMF) is becoming increasingly complex due to new treatments like stem cell transplants and investigational drugs.
- A study involving 1,054 PMF patients identified key survival predictors, including age over 65, low hemoglobin, high leukocyte counts, and constitutional symptoms, leading to the creation of a new risk stratification system with four distinct risk groups.
- This new system offers improved accuracy in predicting survival compared to earlier models and shows that certain cytogenetic abnormalities matter more for patients in intermediate-risk groups; however, the JAK2V617F mutation did not correlate with specific risk outcomes.
Article Synopsis
- - The study investigates the efficacy of intensive chemotherapy followed by autologous stem-cell rescue (IC + HCR) in adults with relapsing primary CNS lymphoma (PCNSL) or intraocular lymphoma after failing high-dose methotrexate treatment.
- - Out of 43 patients treated, 27 underwent IC + HCR, with most achieving complete remission and showing better survival rates compared to the overall cohort.
- - Median overall survival was 18.3 months for all patients, but improved to 58.6 months for those who completed IC + HCR, indicating it as an effective option for treating refractory and recurrent PCNSL.
Article Synopsis
- The International Working Group for Myelofibrosis Research and Treatment (IWG-MRT) is a collaborative group of experts focused on improving research and treatment for myelofibrosis.
- In their second meeting in November 2006, the group decided to standardize terminology for chronic idiopathic myelofibrosis (CIMF), choosing "primary myelofibrosis" (PMF) as the preferred name.
- They also clarified that myelofibrosis related to polycythemia vera and essential thrombocythemia will be termed post-PV MF and post-ET MF, respectively, and recognized "leukemic" transformation as blast phase disease.
Article Synopsis
- Myelofibrosis with myeloid metaplasia (MMM) is a serious condition involving abnormal blood cell production, leading to bone marrow issues and reduced survival quality for patients, often marked by symptoms like anemia and organ enlargement.
- Recent advancements in treatment options, including stem cell transplants and targeted drugs, are improving patient outcomes, particularly those linked to a mutation in the JAK2 gene.
- An international expert panel has established three response categories (complete remission, partial remission, and clinical improvement) to help standardize how treatment responses are measured and to improve comparisons across studies.
Imatinib combined with high-dose chemotherapy is now becoming the gold standard for treatment of Philadelphia chromosome-positive acute leukemias. However, in all studies imatinib dosage was tapered to 400-600 mg per day. We decided to initiate a clinical trial to evaluate an opposite strategy based on high-dose imatinib (800 mg per day) combined with a less intensive chemotherapeutic regimen (vincristine and dexamethasone), which we called the DIV induction regimen.
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- Rearrangements of chromosome 6p are common in various blood cancers, particularly in both myeloid and lymphoid malignancies.
- The research utilized FISH techniques to analyze 16 cases of myeloid disorders, leading to the discovery of two duplications and one triplication involving the HMGA1 gene in myelodysplasia patients, even when obscured by derivative chromosomes.
- HMGA1 proteins are known to be overexpressed in leukemias, making this the first report of HMGA1 duplication, contributing to our understanding of its role in these diseases.
Article Synopsis
- Myelofibrosis with myeloid metaplasia is a rare type of myeloproliferative syndrome, making diagnosis straightforward in its classical form due to specific symptoms like splenomegaly and unique blood cell appearances.
- The disease has a variable progression, with an average survival of 40 to 60 months, and there are several factors, particularly anemia, that can affect prognosis.
- Currently, no treatments have been proven to extend survival either, except for an allogenic bone marrow transplant, leading to challenges in establishing standard approaches for diagnosis, prognosis, and therapy due to the disease's rarity and complexity.