莫斯科地区免疫性血小板减少症患者的管理

S. Zakharov, T. Mitina, R. Vardanyan, I. N. Kontievskiy, A. Faenko, Z. Tekeeva
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摘要

背景。特发性血小板减少性紫癜(ITp)是一种以抗体介导的血小板破坏和血小板生成障碍为特征的自身免疫性疾病,表现为:孤立性血小板减少,自发性出血和不同程度出血的风险。ITp是一种血液学孤儿病,发病率为每10万人1-4例。在现代文献中,原发性和继发性免疫性血小板减少症是区分的。原发性免疫性血小板减少症是一种排除性诊断。为了验证它,需要进行某种诊断搜索。评价莫斯科地区原发性免疫性血小板减少症患者的临床特点和治疗效果。材料和方法。本文介绍了对2400多例诊断为血小板减少症的门诊记录(2010年至2022年)的分析结果。其中,约400例各种形式的经证实的临床病例被列入莫斯科州的ITp登记册。所有患者都住在莫斯科地区,在莫斯科地区临床研究所的孤儿病中心接受治疗和观察。在莫斯科地区临床研究所莫斯科地区孤儿病中心登记的确诊ITp患者中有415人(71%(294人)为女性)。69.8%(290例)的患者在发病时出现出血性综合征。作为一线治疗,92.8% (n = 385)的患者接受了皮质类固醇(强的松龙、甲基强的松龙、地塞米松),在二线治疗中,82% (n = 340)的患者推荐使用血小板生成素受体激动剂(romiplostim、eltrombopag)。ITp患者的三线治疗选择是利妥昔单抗单药治疗、脾切除术和静脉注射免疫球蛋白。3.37% (n = 14)的患者行脾切除术。当评估这一记录时,观察到血小板生成素受体激动剂(romiplostim, eltrombopag)的最高效率- 84.1%的客观反应。
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Management of patients with immune thrombocytopenia in the Moscow region
Background. Idiopathic thrombocytopenic purpura (ITp) is an autoimmune disease characterized by antibody-mediated platelets destruction and impairment of their production, which manifests itself as: isolated thrombocytopenia, risk of spontaneous hemorrhage and bleeding of varying severity. ITp is a hematological, orphan disease with an incidence of 1–4 cases per 100,000 population. In modern literature, primary and secondary immune thrombocytopenias are distinguished. primary immune thrombocytopenia is a diagnosis of exclusion. To verify it, a certain diagnostic search is required.Aim. To evaluate clinical characteristics and treatment efficacy in patients with a confirmed primary immune thrombocytopenia in the Moscow region.Materials and methods. This article presents the results of an analysis of more than 2,400 outpatient records of patients diagnosed with thrombocytopenia (for the period from 2010 to 2022). Of these, about 400 confirmed clinical cases of various ITp forms were included in the ITp registry of the Moscow Region. All patients live in the Moscow region, receive treatment and are observed at the Center for Orphan diseases of the M.f. vladimirskiy Moscow Regional Research Clinical Institute.Results. There are 415 patients with a verified diagnosis of ITp in the register of the Moscow Region Center for Orphan diseases of the M.f. vladimirskiy Moscow Regional Research Clinical Institute (71 % (n = 294) are female). In 69.8 % (n = 290) of patients at the time of disease manifestation, hemorrhagic syndrome was recorded. As a first-line therapy, 92.8 % (n = 385) of patients received corticosteroids (prednisolone, methylprednisolone, dexamethasone), in the second-line therapy, 82 % (n = 340) of patients were recommended therapy with thrombopoietin receptor agonists (romiplostim, eltrombopag). The options for third-line therapy in patients with ITp are rituximab monotherapy, splenectomy, and intravenous immunoglobulin. Splenectomy was performed in 3.37 % (n = 14) of patients.Conclusion. when evaluating this register, the highest efficiency of thrombopoietin receptor agonists (romiplostim, eltrombopag) is observed – 84.1 % of the objective response.
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