Revisited Diagnostic Guidelines for Familial Hemophagocytic Lymphohistiocytosis (FHL) Based on HLH-2004: Updates to Diagnostic Criteria and Development of Multipathway Diagnostic Strategies Introduction Familial hemophagocytic lymphohistiocytosis (FHL) is a severe hyperinflammatory disease characterized by abnormal accumulation of macrophages and l...

Review Report on the Paper: Structural Adaptations of Von Willebrand Factor, Factor VIII, and Factor IX to Coordinate Complex Functions Background and Research Motivation Coagulation factors are crucial elements of maintaining the dynamic balance of the hemostatic system. Among them, von Willebrand Factor (VWF) plays a central role in hemostasis, p...

Inhibition of JAK2/mTOR Fails to Effectively Prevent Acute Graft-versus-Host Disease (GVHD): Limitations of Targeting Th1/Th17 Cells In recent years, the role of systemic immunosuppressive therapies in preventing acute graft-versus-host disease (aGVHD) following allogeneic hematopoietic cell transplantation (alloHCT) has come under scrutiny. Th1 (T...

Whole-Genome Analysis of Plasma Fibrinogen Reveals Population-Differentiated Genetic Regulators with Potential Liver Roles Academic Background Fibrinogen is a crucial coagulation factor and acute-phase reactive protein. Under normal physiological conditions, fibrinogen is abundant in circulation, but during the acute inflammatory response, transcri...

Report on the Compatibility of mRNA-Lipid Nanoparticle-Based Gene-Engineered Platelets with Blood Banking Practices Academic Background Platelets play an essential role in hemostasis, inflammation, sepsis, and cancer. However, clinical applications of platelet transfusions are primarily limited to managing thrombocytopenia and bleeding. To broaden ...

Genetic Risk Stratification and Outcomes Among Treatment-Naive AML Patients Treated with Venetoclax and Azacitidine Academic Background Acute myeloid leukemia (AML) is a highly heterogeneous hematologic malignancy, with prognosis closely related to the genetic characteristics of patients. The European LeukemiaNet (ELN) 2017 and 2022 risk stratifica...

Daratumumab in Pediatric Relapsed/Refractory Acute Lymphoblastic Leukemia or Lymphoblastic Lymphoma: The DELPHINUS Study Academic Background Acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LL) are among the most common malignancies in children. Although the cure rate for newly diagnosed ALL and LL patients is relatively high, 10% to ...

The Effect of Oral Nintedanib vs Placebo on Epistaxis in Hereditary Hemorrhagic Telangiectasia: The EPICURE Multicenter Randomized Double-Blind Trial Academic Background Hereditary Hemorrhagic Telangiectasia (HHT) is a rare genetic disorder characterized by vascular malformations, particularly capillary dilatations. Epistaxis (nosebleeds) is the mo...

Neuropilin-1 (Nrp1) is a multifunctional transmembrane protein that is abundantly expressed on the surface of various cell types, where it binds to class 3 semaphorins (Sema3), heparan sulfate, and vascular endothelial growth factors (VEGFs). Nrp1 plays a crucial role in angiogenesis and vascular permeability regulation, particularly in the VEGF si...

Genetic and Pharmacological Targeting of mTORC1 in Mouse Models of Arteriovenous Malformation Expose Non-Cell Autonomous Signalling in HHT Hereditary Hemorrhagic Telangiectasia (HHT) is a genetic disorder caused by mutations leading to the loss of function in either Activin Receptor-Like Kinase 1 (ACVRL1) or Endoglin (ENG), which act as receptors a...