免疫性血小板减少症患者骨髓间充质干细胞自身功能缺陷机制的研究进展

Functional deficiency of bone marrow mesenchymal stem cells in patients with immune thrombocytopenia:An update

免疫性血小板减少症(ITP)是一种常见的以持续血小板减少为特征的自身免疫性出血性疾病。因其异质性而涉及不同的发病机制,不同疗法对患者效果有差异。骨髓间充质干细胞(BMMSC)可以调节天然免疫和获得性免疫的过程,从而产生耐受的微环境。BMMSC自身功能缺陷和免疫功能调节障碍是ITP发病的重要原因,ITP患者BMMSC的自身功能缺陷的机制主要为P53通路激活、磷脂酰肌醇3激酶/蛋白激酶B(PI3K/AKT)通路活性减弱、肿瘤坏死因子α诱导蛋白3/核因子κB/SMAD同源物7(TNFAIP3/NF-κB/SMAD7)通路激活,而免疫功能调节障碍与诱导各种免疫细胞功能的受损有关。深入了解ITP的发病机制有助于ITP的治疗,但外源性BMMSC应用于ITP临床治疗的效果仍不清楚,需要更多的临床试验来验证。

Immune thrombocytopenia(ITP) is an acquired autoimmune hemorrhagic disorder characterized by persistent thrombocytopenia. It may be induced by different pathogenesis due to its heterogeneity, and the therapeutic effects vary on different patients. Bone marrow derived mesenchymal stem cells(BMMSCs) can modulate innate and adaptive immunity, thus resulting in a tolerant microenvironment. Functional defects and immunomodulatory disorders of BMMSCs are significant causes of ITP. Functional effects associated with the activation of the P53 pathway include decreased activity of the phosphatidylinositol 3 kinase/AKT pathway and activation of the TNFAIP3/NF-κB/SMAD7 pathway. Immune dysfunction appears to be correlated with an impaired ability of BMMSCs to induce various types of immune cells in ITP. An in-depth investigation into the pathogenesis of ITP facilitates the treatment of ITP, but larger-scale clinical trials are needed to verify the efficacy of exogenous BMMSCs in the clinical treatment of ITP.