Abstract Number: PB0954
Meeting: ISTH 2021 Congress
Background: Primary immune thrombocytopenia (ITP) is highly complex, heterogeneous and life threating autoimmune disease. Although, the pathogenesis of ITP is multifactorial, it remains incompletely understood. The main characteristics of ITP etiology is attributed to T and B cell dysfunction, which further lead to auto-immune intolerance and release of auto-immune antibodies. Maternally expressed gene 3 (MEG3), a maternally expressed lncRNA, had closed relationship with autoimmune-related diseases, including ITP. Recently, it was discovered that MEG3 induces immune imbalance of Treg/Th17 in ITP. On the other hand, evidence demonstrated that Notch1 signaling pathway play an important role in the immune system, it regulates the development and differentiation of many other hematopoietic and immune cells. Deregulation of Notch signaling has been linked to multiple human diseases especially T-acute lymphocytic leukemia and recently, it has been related to the development of many autoimmune disorders as ITP.
Aims: The goal of this study is to investigate the expression pattern of lnc-MEG3 and lnc-NOTCH1 genes in patients with chronic ITP, in addition to correlate the expression level with disease phenotype, in order to evaluate its prognostic value.
Methods: A total of 85 cases with chronic ITP and 35 heathy controls were included. The lnc-MEG3 and lnc-NOTCH1 expression level were analyzed in peripheral blood mononuclear cells (PBMCs).
We demonstrated higher expression level of Notch1 in chronic ITP patients than controls with high statistical significant difference. In addition, higher expression levels of lnc-NOTCH1 is significantly associated with high risk patients. In contrast, lnc-MEG3 was downregulated in chronic ITP patients compared to healthy controls, and lower expression levels were significantly associated with poor prognosis and refractory disease phenotype.
Conclusions: Lnc-MEG3 and lnc-NOTCH1 are independent non-invasive prognostic biomarker in chronic ITP, hence they could be therapeutically targeted in future.
To cite this abstract in AMA style:El-Khazragy N, Matbouly S, Abdelsamee HF. Aberrant Expression of Lnc-MEG3 and Lnc-NOTCH1 Predicts Refractory Phenotype in Chronic Idiopathic Thrombocytopenic Purpura [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/aberrant-expression-of-lnc-meg3-and-lnc-notch1-predicts-refractory-phenotype-in-chronic-idiopathic-thrombocytopenic-purpura/. Accessed February 28, 2024.
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