Thomas Moulinet , Anthony Moussu , Ludovic Pierson , Simona Pagliuca
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引用次数: 0
摘要
免疫性血小板减少症(ITP)是一种罕见的自身免疫性疾病,是由于抗体依赖性细胞吞噬、补体依赖性细胞毒性、细胞毒性 T 淋巴细胞介导的细胞毒性和巨核细胞生成改变导致外周血小板破坏所致。这种疾病可能是特发性的,也可能由药物、疫苗、感染、癌症、自身免疫性疾病和全身性疾病引发。最近,我们对 ITP 免疫生物学的认识取得了进展,这支持了一种观点,即其他形式的血小板减少症,例如免疫疗法或细胞疗法后出现的血小板减少症,可能具有共同的病理生理学,并可能具有治疗意义。如果说传统的 ITP 目前采用了一系列新旧药物,那么对于其他更为复杂的免疫介导血小板减少症,临床管理则不太协调,值得进一步进行前瞻性研究。在此,我们试图对病理生理学和当前治疗算法进行新的概述,以帮助受这种疾病影响的成年患者了解包括难治性 ITP 和免疫疗法/细胞疗法后免疫介导血小板减少症在内的编码不清的情况。
The many facets of immune-mediated thrombocytopenia: Principles of immunobiology and immunotherapy
Immune thrombocytopenia (ITP) is a rare autoimmune condition, due to peripheral platelet destruction through antibody-dependent cellular phagocytosis, complement-dependent cytotoxicity, cytotoxic T lymphocyte-mediated cytotoxicity, and megakaryopoiesis alteration. This condition may be idiopathic or triggered by drugs, vaccines, infections, cancers, autoimmune disorders and systemic diseases. Recent advances in our understanding of ITP immunobiology support the idea that other forms of thrombocytopenia, for instance, occurring after immunotherapy or cellular therapies, may share a common pathophysiology with possible therapeutic implications. If a decent pipeline of old and new agents is currently deployed for classical ITP, in other more complex immune-mediated thrombocytopenic disorders, clinical management is less harmonized and would deserve further prospective investigations.
Here, we seek to provide a fresh overview of pathophysiology and current therapeutical algorithms for adult patients affected by this disorder with specific insights into poorly codified scenarios, including refractory ITP and post-immunotherapy/cellular therapy immune-mediated thrombocytopenia.
期刊介绍:
Blood Reviews, a highly regarded international journal, serves as a vital information hub, offering comprehensive evaluations of clinical practices and research insights from esteemed experts. Specially commissioned, peer-reviewed articles authored by leading researchers and practitioners ensure extensive global coverage across all sub-specialties of hematology.