Publications by authors named "Demory J"
Essential thrombocythemia (ET) and polycythemia vera (PV) are rare in adolescent and young adult (AYA). These conditions, similar to those in older patients, are linked with thrombotic complications and the potential progression to secondary myelofibrosis (sMF). This retrospective study of ET and PV patients diagnosed before age 25 evaluated complication rates and impact of cytoreductive drugs on outcomes.
View Article and Find Full Text PDFArticle Synopsis
- Myeloproliferative neoplasms (MPNs) are rare in individuals under 25, and a study examined 444 such patients over a median follow-up of 9.7 years across 38 centers globally.
- The study found that 11.1% had a history of thrombosis, with higher risks associated with the JAK2V617F mutation and hyperviscosity symptoms, while new thrombotic and hemorrhagic events occurred at significant rates.
- It highlighted that disease transformation, particularly to myelofibrosis, was common, with splenomegaly identified as a new risk factor, indicating a need for updated management guidelines for young MPN patients.
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.
In a quantum network based on atoms and photons, a single atom should control the photon state and, reciprocally, a single photon should allow the coherent manipulation of the atom. Both operations require controlling the atom environment and developing efficient atom-photon interfaces, for instance by coupling the natural or artificial atom to cavities. So far, much attention has been drown on manipulating the light field with atomic transitions, recently at the few-photon limit.
View Article and Find Full Text PDFBright single photon sources have recently been obtained by inserting solid-state emitters in microcavities. Accelerating the spontaneous emission via the Purcell effect allows both high brightness and increased operation frequency. However, achieving Purcell enhancement is technologically demanding because the emitter resonance must match the cavity resonance.
View Article and Find Full Text PDFArticle 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.
Entangling a single spin to the polarization of a single incoming photon, generated by an external source, would open new paradigms in quantum optics such as delayed-photon entanglement, deterministic logic gates or fault-tolerant quantum computing. These perspectives rely on the possibility that a single spin induces a macroscopic rotation of a photon polarization. Such polarization rotations induced by single spins were recently observed, yet limited to a few 10(-3) degrees due to poor spin-photon coupling.
View Article and Find Full Text PDFMyeloproliferative 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.
View Article and Find Full Text PDFArticle 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
- * 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.
In a case with secondary myelofibrosis occurring after essential thrombocythemia, cytogenetic analysis revealed an isolated translocation t(X;17)(q27;q22) in all cells. We found that a bacterial artificial chromosome (BAC) encompassing the breakpoint on chromosome 17 long arm contained only one gene, NOG. We therefore investigated the occurrence of this rare breakpoint in myeloproliferative disorders (MPDs).
View Article and Find Full Text PDFArticle Synopsis
- 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.
Article Synopsis
- - The European Myelofibrosis Network (EUMNET) established a set of criteria to define response in patients with myelofibrosis, utilizing clinicohematologic, histologic, and cytogenetic factors.
- - A group of 16 experts evaluated the responses of 104 patients, defining major, moderate, minor, or no response based on core changes in hemoglobin, spleen size, and other blood-related metrics.
- - These standardized criteria aim to enhance the consistency and reporting in future clinical studies focused on myelofibrosis with myeloid metaplasia (MMM).
A minority of patients with newly diagnosed polycythemia vera (PV) have an abnormal karyotype in their myeloid cells but no invariant chromosomal aberration has been found. The most frequent visible alteration is a 20q deletion, also characterized in other myeloproliferative diseases (MPD) and myeloid malignancies; among other chromosomal changes, trisomy 9 appears more common in PV than in other MPDs. When a myelofibrosis complicates the course of the disease, cytogenetic anomalies become quite common with a striking frequency of partial duplication 1q; an evolution towards myelodysplasia or acute leukemia is almost always associated with nonspecific chromosomal aberrations.
View Article and Find Full Text PDFArticle Synopsis
- - The study examined the safety and effectiveness of low-dose thalidomide in patients with advanced myelofibrosis, starting treatment at 50 mg and potentially increasing to 400 mg based on tolerance.
- - Out of 63 patients, about half managed to take over 100 mg daily, but the dropout rate at 6 months was 51%, with lower dropouts in those reporting higher fatigue levels.
- - Results showed benefits like improved anemia (22%), reduced need for transfusions (39%), increased platelet counts in 22% of certain patients, and significant reductions in disease severity and fatigue in 31% of participants.
Dysregulation and overexpression of HMGA2 in myelofibrosis with myeloid metaplasia.
Genes Chromosomes Cancer
January 2004
Among cytogenetic studies of patients affected with myelofibrosis with myeloid metaplasia (MMM), a rare chronic myeloproliferative disorder, we found several reports of structural abnormalities of the long arm of chromosome 12. Two MMM patients had a balanced translocation involving 12q: t(4;12)(q32;q15) and t(5;12)(p14;q15), respectively. FISH (fluorescence in situ hybridization) analysis showed that BAC (bacterial artificial chromosome) RP11-366L20 overlaps the breakpoint in both cases.
View Article and Find Full Text PDFKaryotypic abnormalities in myelofibrosis following polycythemia vera.
Cancer Genet Cytogenet
January 2003
Polycythemia vera (PV) is a chronic myeloproliferative disease characterized by an increase of total red cell volume; in 10% to 15% of cases, bone marrow fibrosis complicates the course of the disease after several years, resulting in a hematologic picture mimicking myelofibrosis with myelocytic metaplasia (MMM). This condition is known as post polycythemic myelofibrosis (PPMF). Among 30 patients with PPMF followed in Northern France, 27 (90%) expressed one or two abnormal clones in myelocytic cell cultures.
View Article and Find Full Text PDFAmong cytogenetic studies of 205 patients diagnosed as myelofibrosis with myeloid metaplasia, we found seven cases with structural abnormalities of the long arm of chromosome 12. The karyotype showed six balanced translocations, that is, t(4;12)(q33;q21), t(5;12)(p14;q21), t(1;12)(q22;q24), t(12;17)(q24;q11), t(7;12) (p11;q24), and t(1;12)(p12;q24), as well as other cytogenetic abnormalities such as del(12)(q21;q24) and inv(12) (p12q24). Some isolated cases involving the 12q21 region have also been described in the literature.
View Article and Find Full Text PDF