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[成人继发性免疫性血小板减少症]

[Secondary ITP in adults].

作者信息

Michel M, Lega J-C, Terriou L

机构信息

Service de médecine interne, centre de référence pour les cytopénies auto-immunes de l'adulte, CHU Henri-Mondor, université Paris Est Créteil, Assistance publique-Hôpitaux de Paris, Créteil, France.

Service de médecine interne et vasculaire, centre de compétences cytopénies auto-immunes, hôpital Lyon Sud, Lyon, France.

出版信息

Rev Med Interne. 2021 Jan;42(1):50-57. doi: 10.1016/j.revmed.2020.08.004. Epub 2020 Nov 1.

DOI:10.1016/j.revmed.2020.08.004
PMID:33139079
Abstract

Secondary forms of immune thrombocytopenia (ITP) represent approximately 20% of all ITP cases in adulthood and this rate increases with age. Since some causes may influence both the prognosis and outcome but also the management of ITP, a minimal workup must be performed at ITP diagnosis to look for an associated or underlying cause. Among adults, B-cell lymphomas and mainly chronic lymphocytic leukemia, systemic auto-immune diseases such as systemic lupus or primary immunodeficiencies mainly represented by common variable immunodeficiency are the most frequent causes of secondary ITP. Whereas first-line therapy used for secondary ITP is usually similar to the one commonly used in primary ITP and relies mostly on corticosteroids±intravenous immunoglobulin according to the severity of bleeding, second and third-line treatments must take into account the type and degree of activity of the underlying disease.

摘要

继发性免疫性血小板减少症(ITP)约占成年ITP病例的20%,且这一比例随年龄增长而升高。由于某些病因可能影响ITP的预后、结局及治疗管理,因此在ITP诊断时必须进行最低限度的检查,以寻找相关或潜在病因。在成人中,B细胞淋巴瘤,主要是慢性淋巴细胞白血病,系统性自身免疫性疾病如系统性红斑狼疮,以及主要以常见变异型免疫缺陷为代表的原发性免疫缺陷,是继发性ITP最常见的病因。虽然用于继发性ITP的一线治疗通常与原发性ITP常用的治疗相似,主要根据出血严重程度使用糖皮质激素±静脉注射免疫球蛋白,但二线和三线治疗必须考虑基础疾病的类型和活动程度。

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