Svahn Johanna, Fioredda Francesca, Calvillo Michaela, Molinari Angelo C, Micalizzi Concetta, Banov Laura, Schmidt Madalina, Caprino Daniela, Marinelli Doretta, Gallisai Domenico, Dufour Carlo
Haematology Unit, G Gaslini Children's Institute, Genova, Italy.
Br J Haematol. 2009 Apr;145(1):96-100. doi: 10.1111/j.1365-2141.2009.07594.x. Epub 2009 Feb 4.
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.
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。
