成人免疫性血小板减少性紫癜患者对一线类固醇治疗的反应与病程之间的关系

IF 1.6 Q3 HEMATOLOGY Hematology, Transfusion and Cell Therapy Pub Date : 2024-11-01 Epub Date: 2024-07-23 DOI:10.1016/j.htct.2024.03.008
Esra Seçkin , Rafiye Ciftciler
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引用次数: 0

摘要

背景免疫性血小板减少性紫癜是一种复发性自身免疫性疾病,以血小板减少、紫癜和出血发作为特征,其发病机制主要是针对血小板的自身抗体。自20世纪50年代以来,一线治疗一直是糖皮质激素,它对血小板减少症有间接和直接的影响。虽然在以前的研究中已经调查了诊断时与免疫性血小板减少性紫癜的慢性化相关的特征,但在文献中没有发现对一线类固醇治疗的反应与疾病进程之间的关系,这是本研究的目的。材料和方法本回顾性单中心研究回顾了2012年9月至2022年9月塞尔丘克大学医学院临床血液学系诊断为免疫性血小板减少性紫癜患者的电子档案。血小板计数≤30 × 109/L患者外周血涂片确认血小板计数。根据免疫性血小板减少性紫癜出血评分评估患者诊断时的出血情况。患者对治疗的反应分为三组:血小板计数≤30 × 109/L定义为无反应,血小板计数30 - 100 × 109/L定义为部分缓解,血小板计数>;100 × 109/L定义为完全缓解。随后,部分缓解组或完全缓解组的患者被分为两个亚组:缓解期小于或大于6个月的患者。结果共纳入100例患者;73 %为青年(19-65岁),27 %为老年(65岁)。患者以女性居多(69 %)。41例患者住院,无出血。一线治疗的完全缓解率为61 %。在一线治疗中给予的药物在反应和缓解时间方面没有显着差异。结论免疫血小板减少性紫癜治疗的主要目的是防止大出血,而不是使血小板计数恢复正常。糖皮质激素,治疗的第一步,提供高反应率。在治疗反应和长期缓解方面,糖皮质激素药物之间没有显著差异。在选择糖皮质激素药物时要考虑的要点是副作用概况、给药的容易程度和治疗的个体化。
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Relationship between response to first-line steroid treatment in adult immune thrombocytopenic purpura and the course of the disease

Background

Immune thrombocytopenic purpura, a recurrent autoimmune disease, is characterized by thrombocytopenia, purpura and hemorrhagic episodes with the main factor in the pathogenesis of this disease being autoantibodies against platelets. Since the 1950s, first-line treatment has been glucocorticoids that have indirect and direct effects on thrombocytopenia. Although the characteristics associated with the chronicization of immune thrombocytopenic purpura at the time of diagnosis have been investigated in previous studies, no study was found in the literature investigating the relationship between the response to first-line steroid treatment and the course of the disease, the aim of this study.

Materials and methods

This retrospective, single center study revisited electronic files of patients with a diagnosis of immune thrombocytopenic purpura between September 2012 and September at the Department of Clinical Hematology, Selcuk University Faculty of Medicine 2022. The platelet count had been confirmed by peripheral blood smears of patients with a platelet count ≤30 × 109/L. The bleeding status of patients at the time of diagnosis was evaluated according to the immune thrombocytopenic purpura bleeding score. Patient responses to treatment were categorized in three groups: a platelet count ≤30 × 109/L was defined as no-response, a platelet count of 30–100 × 109/L was defined as partial response, and a platelet count >100 × 109/L was defined as complete response. Subsequently, patients in the partial or complete response groups were divided into two subgroups: patients who remained in remission for less than or more than six months.

Results

A total of 100 patients were included in the study; 73 % were in the young (19–65 years old) and 27 % in the old (>65 years old) age group. Most of the patients were female (69 %). Forty-one patients were hospitalized without bleeding. The complete response rate to first-line treatment was 61 %. There was no significant difference between the agents given in first-line treatment in terms of response and length of remission.

Conclusion

The main purpose of immune thrombocytopenic purpura treatment is to prevent severe bleeding rather than bringing the platelet count to normal values. Glucocorticoids, the first step of treatment, provide high response rates. There is no significant difference between glucocorticoid agents in terms of response to treatment and long-term remission. The points to be considered in the selection of glucocorticoid agents are the side effect profiles, ease of administration and individualization of treatment.
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CiteScore
2.40
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4.80%
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1419
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30 weeks
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