Department of Hematology, Internal Medicine Division, Dr. Jose E. González University Hospital and School of Medicine, Universidad Autónoma de Nuevo León, Monterrey, Mexico.
Department of Hematology, Internal Medicine Division, Dr. Jose E. González University Hospital and School of Medicine, Universidad Autónoma de Nuevo León, Monterrey, Mexico.
Blood Rev. 2021 Sep;49:100827. doi: 10.1016/j.blre.2021.100827. Epub 2021 Mar 18.
Immune thrombocytopenia (ITP) is a heterogeneous disease with an unpredictable course. Chronicity can develop in up to two-thirds of adults and 20-25% of children, representing a significant burden on patients' quality of life. Despite acceptable responses to treatment, precise etiology and pathophysiology phenomena driving evolution to chronicity remain undefined. We analyzed reported risk factors for chronic ITP and associated them with proposed underlying mechanisms in its pathogenesis, including bone marrow (BM) microenvironment disturbances, clinical features, and immunological markers. Their understanding has diagnostic implications, such as screening for the presence of specific antibodies or BM examination employing molecular tools, which could help predict prognosis and recognize main pathogenic pathways in each patient. Identifying these underlying mechanisms could guide the use of personalized therapies such as all-trans retinoic acid, mTor inhibitors, FcRn inhibitors, oseltamivir, and others. Further research should lead to tailored treatments and chronic course prevention, improving patients' quality of life.
免疫性血小板减少症(ITP)是一种异质性疾病,病程不可预测。高达三分之二的成年人和 20-25%的儿童会发展为慢性疾病,这给患者的生活质量带来了重大负担。尽管治疗有一定效果,但导致疾病向慢性发展的确切病因和病理生理现象仍未确定。我们分析了报告的慢性 ITP 的风险因素,并将其与发病机制中提出的潜在机制相关联,包括骨髓(BM)微环境紊乱、临床特征和免疫标志物。这些机制的理解具有诊断意义,例如筛查特定抗体的存在或使用分子工具进行 BM 检查,这有助于预测预后,并识别每位患者的主要发病途径。确定这些潜在机制可以指导使用个性化治疗方法,如全反式维甲酸、mTor 抑制剂、FcRn 抑制剂、奥司他韦等。进一步的研究应该导致制定个体化的治疗方案和预防慢性病程,从而改善患者的生活质量。