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Scopus
ISSN 2572-9241 · Wiley · 6.934 artikel di indeks
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Artikel Jurnal
Highly effective combination of BRG1/BRM inhibitor with BET inhibitor or decitabine for high‐risk MECOM‐rearranged AML
Abstract In AML with 3q26.2 rearrangements (r) the distal GATA2 hematopoietic enhancer becomes aberrantly relocated leading to activation of EVI1 expression. EVI1 is a transcriptional regulator that plays a role in proliferation and maintenance of a stem cell‐like phenotype in AML. BRG1 (SMARCA4)…
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Artikel Jurnal
Blinatumomab in de novo AYA ALL—Results of the Australasian Leukaemia and Lymphoma Group ALL09 “SUBLIME” study
Abstract Pediatric regimens improve outcomes in adolescent and young adult (AYA) acute lymphoblastic leukemia (ALL) patients. End‐consolidation (time point 2 [TP2]) minimal residual disease negativity (MRDneg) is associated with improved survival. In this study, standard consolidation chemotherapy…
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Artikel Jurnal
Additive effect of multiple genetic variants in SEC23B and PIEZO1 on iron metabolism dyshomeostasis in hereditary anemias
Abstract Hereditary anemias encompass a genetically heterogeneous spectrum of disorders, often involving multi‐locus inheritance, which can complicate clinical management and worsen disease severity. This study investigates the impact of the co‐inheritance of SEC23B loss‐of‐function pathogenic…
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Artikel Jurnal
Tet2 deficiency promotes IgG1+ B‐cell expansion and differentiation blockade through deregulation of the Nfkbia–c‐Rel axis
Abstract The germinal center (GC) reaction is essential for orchestrating humoral immunity by producing plasma cells (PCs) and memory B cells (MBCs). TET2, an α‐ketoglutarate‐dependent dioxygenase, plays a critical role in B‐cell exit from the GC and in plasma cell differentiation. Moreover, TET2…
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Artikel Jurnal
Application of machine learning in the diagnostic work‐up of telomere biology disorders
ABSTRACT We applied supervised and unsupervised machine learning (ML) analyses to a cohort of 140 patients referred to the Hematology Unit of the G. Gaslini Institute from 1989 to 2023 for persistent cytopenia and/or features suggestive of telomere biology disorders (TBDs). Patients were labeled as…
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Artikel Jurnal
Unveiling relative lymphopenia and elevated monocyte‐to‐lymphocyte count as novel independent adverse prognostic factors in chronic myelomonocytic leukemia (CMML)—Proposal of the objective prognostic index for CMML (OPIC)
Abstract Chronic myelomonocytic leukemia (CMML) shows marked prognostic heterogeneity. Although leukocytosis is a recognized adverse prognostic factor, the contribution of its individual components remains insufficiently defined. In a cohort of 240 patients classified according to International…
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Artikel Jurnal
MeMAGEN: A Phase IIa/IIb open‐label trial of memantine testing safety and tolerability in sickle cell patients
ABSTRACT Administration of memantine, an antagonist of the N‐methyl‐ d‐aspartate receptor, prevents Ca2+ overload and dehydration of red blood cells (RBCs) in patients with sickle cell disease (SCD). The objectives of the 1‐year dose‐escalation Phase IIa/IIb Memantine trial (MeMAGEN – NCT 03247218)…
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Artikel Jurnal
Daratumumab‐based quadruplet for patients with extramedullary multiple myeloma: Results from the Phase II prospective EMN19 study
ABSTRACT Novel therapies are needed for patients with multiple myeloma (MM) and extramedullary plasmacytomas. The prospective, Phase II EMN19 study assessed the efficacy and safety of daratumumab plus bortezomib, cyclophosphamide, and dexamethasone (DaraVCD) in 40 patients with newly diagnosed MM…
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Artikel Jurnal
Erythropoiesis in health and disease: Distinguishing defective and ineffective erythropoiesis
Abstract Erythropoiesis is a finely regulated process ensuring continuous red blood cell production to maintain oxygen delivery. Disruptions in this process give rise to defective erythropoiesis, characterized by impaired lineage commitment and progenitor development, and ineffective erythropoiesis…
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Artikel Jurnal
Drivers of clinical resistance to venetoclax and hypomethylating agents in acute myeloid leukemia and strategies for improving efficacy
ABSTRACT The B‐cell lymphoma 2 (BCL‐2) inhibitor venetoclax (VEN) in combination with hypomethylating agents (HMAs) has improved treatment outcomes for acute myeloid leukemia (AML) patients unfit for intensive chemotherapy and is increasingly used in the relapsed/refractory setting. However…
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Artikel Jurnal
Navigating the evolving management of smoldering multiple myeloma
Abstract Smoldering multiple myeloma (SMM) represents an intermediate clinical stage between monoclonal gammopathy of undetermined significance (MGUS) and symptomatic multiple myeloma (MM). SMM carries a highly variable risk of progression to MM, requiring individualized risk stratification to…
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Artikel Jurnal
Hypomorphic ATP11c is a novel regulator of decreased efficacy of transfused red blood cells in humans and mice
Abstract Chronic red blood cell (RBC) transfusion sustains patients with diverse hematologic disorders, but repeated transfusion leads to iron overload and alloimmunization. Reducing transfusion burden requires identifying donor units that circulate more effectively after storage, yet determinants…
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Artikel Jurnal
Triplet regimens for frontline treatment of CLL—Great company or just a crowd?
Abstract Standard frontline treatment of chronic lymphocytic leukemia (CLL) is with fixed‐duration venetoclax‐based doublets or indefinite covalent Bruton tyrosine kinase inhibitor (BTKI). Although these approaches achieve excellent results, venetoclax doublets have diminished efficacy in high‐risk…
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Artikel Jurnal
Chronic graft‐versus‐host disease in long‐term survivors of childhood leukemia
Abstract Hematopoietic stem cell transplantation (HSCT) is a curative treatment for high‐risk childhood leukemia but may lead to chronic graft‐versus‐host disease (cGvHD), a severe long‐term complication. This study analyzed data from the LEA cohort, including 446 childhood leukemia survivors…
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Artikel Jurnal
Rps19R67∆ mutation creates a model of Diamond–Blackfan anemia and reveals downstream mediators of p53 pathway
Abstract Diamond–Blackfan anemia (DBA) is a rare bone marrow failure syndrome accompanied by cardiovascular, skeletal, and urogenital abnormalities. Most of the affected individuals carry mutations in ribosomal proteins, including RPS19, a component of the 40S ribosomal subunit. We developed a…
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Artikel Jurnal
IL21—STAT3 controls the pentose phosphate pathway to support metabolic reprogramming and tumor progression in chronic lymphocytic leukemia
Abstract Studying how microenvironmental cues influence metabolic reprogramming can uncover mechanisms driving tumor progression. Using an in vitro model with proliferative stimuli of the in vivo lymph node niche (LN)—including interleukin‐21 (IL‐21)—we examined metabolic rewiring in chronic…
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Artikel Jurnal
Biological heterogeneity in adult IRF4‐rearranged large B‐cell lymphoma: Stratification by age and anatomical site
Abstract Large B‐cell lymphoma (LBCL) with IRF4 rearrangement (LBCL‐IRF4‐R) is a rare subtype predominantly diagnosed in children and young adults. Whether adult LBCL cases with IRF4 rearrangement (IRF4‐R) should be classified as LBCL‐IRF4‐R remains unclear. Clinicopathological and molecular…
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