新诊断的免疫性血小板减少症患者的治疗结果:遵循临床建议是否合理?

I. S. Dolgopolov, A. M. Mnatsakanian, A. V. Ivanova, A. D. Volianskaya, E. A. Nakhodnova, M. Rykov, A. V. Zaitseva
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摘要

免疫性血小板减少症(ITP)是一种获得性免疫介导的疾病,其特征是孤立的一过性或持续性血小板减少<100-109/L。免疫性血小板减少症的发病率为每105名儿童/年4-6.4例。根据俄罗斯卫生部网站发布的临床指南(ID699),分析新诊断的免疫性血小板减少症的治疗结果。分析对象包括 2023 年入住特维尔地区儿童医院的 13 名免疫性血小板减少症患者(女 46%,男 54%,中位年龄 9.5(4-17)岁)。69%的病例在免疫性血小板减少之前有过感染史,8%的病例接种过麻疹疫苗。感染发病的中位时间为 11(5-15)天。出血程度为 1 克-4(31%),2 克-3(23%),3 克-6(46%)。3例(23%)出现血尿,1例(8%)出现月经过多。入院时的平均血小板计数为 9.0 (1.0-86) - 109/升。发病率为每105名儿童/年5.7例。54%的病例使用地塞米松 20 mg/m2,第 1-3 天;15%的病例使用 IVIG 1000 mg/kg,第 1 天;8%的病例使用泼尼松龙 2 mg/kg,第 21 天;23%的病例进行了动态随访。有 2 例患者(17%)因出血性综合征恶化或/和类固醇治疗并发症而需要停止类固醇治疗并转用 IVIG。分别有 8 例(62%)和 4 例(31%)患者获得了部分和完全应答。1例(8%)患者的疗效无法评估。一线治疗的总有效率为 92%。临床指南ID699在获得初治应答和预防儿童免疫性血小板减少症复发方面非常有效。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Results of treatment of patients with newly diagnosed immune thrombocytopenia: is it justified to follow clinical recommendations?
Immune thrombocytopenia (ITP) is an acquired immune-mediated disease characterized by isolated transient or persistent thrombocytopenia < 100·109/L. The incidence of immune thrombocytopenia is 4–6,4 per 105 children/year.Purpose. To analyze the results of treatment newly diagnosed immune thrombocytopenia according to the clinical guidelines (ID699) published on the website of the Ministry of Health of Russia.Material and methods. The analysis included 13 patients (F–46%, M–54%, median age — 9.5 (4–17) years) with immune thrombocytopenia admitted to the Tver Regional children hospital in 2023. A history of infection preceding the immune thrombocytopenia was in 69% of cases and measles vaccination in 8%. The median period from the onset of infection was 11 (5–15) days. Degree of bleeding was — 1 gr. — 4 (31%), 2 gr. — 3 (23%), 3 gr. — 6 (46%). Hematuria was observed in 3 (23%), menorrhagia in 1 (8%) case. The mean platelet count at the time of admission was 9,0 (1.0–86) · 109/l.Results. The incidence was 5.7 per 105 children/year. Dexamethasone 20 mg/m2, days 1–3, was used in 54% of cases; IVIG 1000 mg/kg, day 1 in 15%, prednisolone 2 mg/kg, day 21 in 8% and in 23% of cases dynamic follow-up was performed. In 2 cases (17%), discontinuation of steroid therapy and switching to IVIG was required due to a hemorrhagic syndrome deterioration or/and complication of steroid therapy. Partial and complete responses were achieved in 8 (62%) and 4 (31%) cases respectively. In 1 (8%) case, the response could not be evaluated. The summary efficacy of first-line therapy was 92%.Conclusion. The clinical guidelines ID699 was highly effective in achieving a primary response and preventing recurrence of immune thrombocytopenia in children.
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