儿童慢性免疫性血小板减少症自发缓解的预测因素

IF 3 3区 医学 Q2 HEMATOLOGY Annals of Hematology Pub Date : 2024-10-27 DOI:10.1007/s00277-024-06056-5
Yuan-Ning Yang, Yun-Hsuan Yeh, Jiann-Shiuh Chen, Li-Wen Chen, Yung-Chieh Lin, Chao-Neng Cheng
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引用次数: 0

摘要

本研究探讨了慢性免疫性血小板减少症(ITP)患儿自发缓解的相关因素。我们回顾性分析了我院1988年1月至2019年12月期间确诊为ITP患者的病历。共发现104名慢性ITP患儿。慢性 ITP 诊断后的中位随访时间为 3.6 年(IQR 1.2-8.3,范围 0.1-31.4)。15名(14.4%)症状严重的患者接受了特定的血小板升高疗法,包括脾切除术、利妥昔单抗和血小板生成素受体激动剂。其中 7 人的病情得到缓解。在诊断为慢性 ITP 时血小板计数为 9/L 的患者中,与未接受特定血小板升高疗法的患者相比,接受特定血小板升高疗法的患者缓解率更高(HR:4.66,95% CI:1.36-16.0)。16名患者(15.4%)发展为系统性红斑狼疮,46名患者(44.2%)在中位随访6.8年后仍患有血小板减少症,42名患者(40.4%)病情得到缓解,中位缓解时间为2.0年(IQR 0.6-4.1,范围0.1-15.7)。儿童慢性 ITP 自发缓解的两个独立预测因素是诊断慢性 ITP 时血小板计数大于 30 × 109/L(HR:3.16,95% CI:1.51-6.62)和随访时 ANA 持续阴性(HR:6.12,95% CI:1.46-25.7)。无危险因素的慢性 ITP 患者在确诊后 10 年自发缓解的累积概率为 72.2%,而有两个危险因素的患者为 0%。
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Predictors for spontaneous remission in childhood chronic immune thrombocytopenia.

This study examined the factors associated with spontaneous remission in children with chronic immune thrombocytopenia (ITP). We retrospectively analyzed the medical records of patients diagnosed with ITP from January 1988 to December 2019 at our institute. A total of 104 children with chronic ITP were identified. The median follow-up time from diagnosis of chronic ITP was 3.6 years (IQR 1.2-8.3, range 0.1-31.4). Fifteen (14.4%) patients with severe symptoms received specific platelet-elevating therapies, including splenectomy, rituximab, and thrombopoietin receptor agonists. Seven of them achieved remission. Among the patients with a platelet count < 30 × 109/L at the time of diagnosis of chronic ITP, those who received specific platelet-elevating therapies had a higher remission rate compared to those who did not (HR: 4.66, 95% CI: 1.36-16.0). Sixteen patients (15.4%) developed systemic lupus erythematosus, 46 (44.2%) still had thrombocytopenia after a median follow-up of 6.8 years, and 42 (40.4%) achieved remission with a median time to remission of 2.0 years (IQR 0.6-4.1, range 0.1-15.7). The two independent predictive factors for spontaneous remission in childhood chronic ITP were platelet counts > 30 × 109/L at the time of diagnosis of chronic ITP (HR: 3.16, 95% CI: 1.51-6.62) and persistently negative ANA at follow-up (HR: 6.12, 95% CI: 1.46-25.7). The cumulative probabilities of spontaneous remission at 10 years post-diagnosis of chronic ITP were 72.2% for patients without risk factor compared to 0% for patients with two risk factors.

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来源期刊
Annals of Hematology
Annals of Hematology 医学-血液学
CiteScore
5.60
自引率
2.90%
发文量
304
审稿时长
2 months
期刊介绍: Annals of Hematology covers the whole spectrum of clinical and experimental hematology, hemostaseology, blood transfusion, and related aspects of medical oncology, including diagnosis and treatment of leukemias, lymphatic neoplasias and solid tumors, and transplantation of hematopoietic stem cells. Coverage includes general aspects of oncology, molecular biology and immunology as pertinent to problems of human blood disease. The journal is associated with the German Society for Hematology and Medical Oncology, and the Austrian Society for Hematology and Oncology.
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