Immune thrombocytopenia (ITP) is an acquired immune disorder characterized by increased platelet destruction and reduced platelet (Plt) production. Hypoxia-inducible factor-1α (HIF-1α) have regulatory effects on Treg/Th17 axis balance and may represent relevant factors in the pathogenesis of ITP. Treg/Th17 ratio, serum levels and gene expression were investigated in new diagnosed ITP (NITP) and chronic ITP (CITP).
View Article and Find Full Text PDFBackground And Aims: The pathophysiology of acquired aplastic anemia (aAA) is most associated with T cell mediated immune dysfunction, but the role of CD4 CD8 double negative T cells (DNTs) in pediatric patients with aAA is unclear. In this study, we aimed to investigate the proportion of TCR-αβ DNTs in pediatric patients with aAA and correlation with the response to immunosuppressive therapy (IST).
Materials And Methods: Assessment of DNTs from peripheral blood was done by sensitive multi-color flow cytometry.
Background And Objective: Immune thrombocytopenia (ITP) is an autoimmune-mediated hemorrhagic disease. Anti-glycoprotein autoantibodies play a key role in the pathophysiology of ITP, but the relationship between platelet-specific antibodies and bleeding severity is unclear. This study aimed to analyze the relationship between anti-glycoprotein autoantibodies and bleeding severity in children with newly diagnosed ITP and platelet count less than 10 × 10 /L.
View Article and Find Full Text PDFBackground: No studies evaluated the role of mutations in outcomes for low-dose immune tolerance induction (ITI) in people with severe hemophilia A (SHA) with high-titer inhibitors.
Objectives: To explore the association between mutation types and low-dose ITI outcomes in children with SHA with high-titer inhibitors.
Methods: Children SHA with high-titer inhibitors who received low-dose ITI therapy at least for 1 year were included in this study.
Introduction: Type of gene mutation is the most important risk factor for inhibitor development in people with severe hemophilia A. However, there are few large cohort studies on the mutation spectrum of people with severe hemophilia A with inhibitors.
Objective: This was the first large cohort study in children with severe hemophilia A with inhibitors from China that aimed to analyze the association between variant types and inhibitor status.
Background: Hypoxia-inducible factor-1α (HIF-1α) plays a crucial role in both innate and adaptive immunity. Emerging evidence indicates that HIF-1α is associated with the inflammation and pathologic activities of autoimmune diseases, suggesting that HIF1α may be involved in immune dysregulation in patients with immune thrombocytopenia (ITP). The purpose of this study was to evaluate whether single nucleotide polymorphisms (SNPs) of the HIF1A gene are associated with susceptibility to ITP and its clinical prognosis including incidence of chronic ITP and glucocorticoid sensitivity.
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