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基于利妥昔单抗的免疫抑制疗法治疗婴儿自身免疫性溶血性贫血

Rituximab-based immunosuppression for autoimmune haemolytic anaemia in infants.

作者信息

Svahn Johanna, Fioredda Francesca, Calvillo Michaela, Molinari Angelo C, Micalizzi Concetta, Banov Laura, Schmidt Madalina, Caprino Daniela, Marinelli Doretta, Gallisai Domenico, Dufour Carlo

机构信息

Haematology Unit, G Gaslini Children's Institute, Genova, Italy.

出版信息

Br J Haematol. 2009 Apr;145(1):96-100. doi: 10.1111/j.1365-2141.2009.07594.x. Epub 2009 Feb 4.

Abstract

We report a case series of four infants with severe autoimmune haemolytic anaemia (AIHA) who responded to treatment with rituximab and cyclosporine after having failed first line therapy with high-dose steroid (prednisolone 4-8 mg/kg/d). Rituximab was started at 11-90 d from onset due to continued haemolysis; three infants also received cyclosporine A. Three of four infants reached complete response, defined as normal haemoglobin, reticulocytes and negative indices of haemolysis, at 7-21 months from diagnosis. In long-term follow-up two infants remained disease-free with normal immunology, one had undefined immunodeficiency and one had autoimmune lymphoproliferative syndrome.

摘要

我们报告了一组4例患有严重自身免疫性溶血性贫血(AIHA)的婴儿病例,这些婴儿在接受高剂量类固醇(泼尼松龙4-8mg/kg/d)一线治疗失败后,对利妥昔单抗和环孢素治疗有反应。由于持续溶血,利妥昔单抗在发病后11-90天开始使用;4例婴儿中有3例还接受了环孢素A治疗。4例婴儿中有3例在诊断后7-21个月达到完全缓解,定义为血红蛋白、网织红细胞正常且溶血指标为阴性。在长期随访中,2例婴儿病情缓解且免疫功能正常,1例有未明确的免疫缺陷,1例有自身免疫性淋巴增殖综合征。

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