Department of Internal Medicine and Clinical Immunology, Regional Competence Center for Rare and Systemic Auto-Immunes Diseases and Auto-Immune cytopenias, Nancy University Hospital, Lorraine University, Vandoeuvre-lès-Nancy, France; UMR 7365, IMoPA, Lorraine University, CNRS, Nancy, France.
Department of Internal Medicine and Clinical Immunology, Regional Competence Center for Rare and Systemic Auto-Immunes Diseases and Auto-Immune cytopenias, Nancy University Hospital, Lorraine University, Vandoeuvre-lès-Nancy, France.
Blood Rev. 2024 Jan;63:101141. doi: 10.1016/j.blre.2023.101141. Epub 2023 Nov 11.
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.
免疫性血小板减少症（ITP）是一种罕见的自身免疫性疾病，由于抗体依赖性细胞吞噬作用、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。这种情况可能是特发性的，也可能由药物、疫苗、感染、癌症、自身免疫性疾病和系统性疾病引发。我们对 ITP 免疫生物学的理解的最新进展支持这样一种观点，即其他形式的血小板减少症，例如免疫治疗或细胞治疗后发生的血小板减少症，可能与可能具有治疗意义的共同病理生理学有关。如果目前为经典 ITP 部署了大量新旧药物，那么在其他更复杂的免疫介导性血小板减少症疾病中，临床管理的协调程度较低，值得进一步进行前瞻性研究。在这里，我们旨在为受这种疾病影响的成年患者提供对这种疾病的病理生理学和当前治疗方案的新概述，并深入了解编码不良的情况，包括难治性 ITP 和免疫治疗/细胞治疗后免疫介导的血小板减少症。
