Quinti Isabella, Pulvirenti Federica, Milito Cinzia, Granata Guido, Giovannetti Gianluca, La Marra Fabiola, Pesce Anna M, Farrugia Albert, Coluzzi Serelina, Girelli Gabriella
Department of Molecular Medicine, Sapienza University of Rome, Rome, Italy.
Unit of Immunohematology and Transfusion Medicine, Sapienza University of Rome, Rome, Italy.
Transfusion. 2015 May;55(5):1067-74. doi: 10.1111/trf.12939. Epub 2014 Dec 22.
Immunoglobulin (Ig)G replacement with intravenous or subcutaneous immunoglobulins is a lifelong substitutive therapy in patients with primary antibody deficiencies (PADs). Hemolysis after immunoglobulin therapy was described in patients receiving high immunoglobulin dosages. The issue of hemolysis after immunoglobulin administration at replacement doses has been considered of little clinical significance.
This was a single-center observational study over a 2-year period on immunoglobulin-induced hemolysis in a cohort of 162 patients with PADs treated with immunoglobulin administered at replacement dosages.
Six patients had signs and symptoms of immunoglobulin-induced hemolysis. Two additional asymptomatic patients were identified by a short-term study run on 16 randomly selected asymptomatic patients. Alloantibodies eluted from patients' red blood cells (RBCs) had anti-A and Rh specificities (anti-D and anti-C). The immunoglobulins contained alloantibodies with the same specificities of the antibodies eluted from patients' RBCs.
Hemolysis occurred in patients receiving immunoglobulin at replacement dosages. Polyvalent immunoglobulin preparations contained multiple clinically significant antibodies that could have unexpected hemolytic consequences, as anti-C whose research and titration are not required by the European Pharmacopoeia. The issue of hemolysis in long-term recipients of immunoglobulin treatment administered at replacement dosages should be more widely recognized.
对于原发性抗体缺陷(PAD)患者,静脉注射或皮下注射免疫球蛋白进行免疫球蛋白(Ig)G替代治疗是一种终身替代疗法。接受高剂量免疫球蛋白治疗的患者曾有免疫球蛋白治疗后溶血的报道。而关于以替代剂量给予免疫球蛋白后发生溶血的问题,一直被认为临床意义不大。
这是一项为期2年的单中心观察性研究,观察对象为162例接受替代剂量免疫球蛋白治疗的PAD患者队列中免疫球蛋白诱导的溶血情况。
6例患者出现免疫球蛋白诱导溶血的体征和症状。通过对16例随机选择的无症状患者进行的短期研究,又发现了2例无症状患者。从患者红细胞(RBC)上洗脱的同种抗体具有抗A和Rh特异性(抗D和抗C)。免疫球蛋白中含有的同种抗体与从患者RBC上洗脱的抗体具有相同的特异性。
接受替代剂量免疫球蛋白治疗的患者发生了溶血。多价免疫球蛋白制剂含有多种具有临床意义的抗体,可能会产生意想不到的溶血后果,如抗C,而欧洲药典并不要求对其进行研究和滴定。对于接受替代剂量免疫球蛋白长期治疗的患者发生溶血的问题,应得到更广泛的认识。