Chinchilla Langeber Sara, Osuna Marco Marta Pilar, Benedit María, Cervera Bravo Áurea
Department of Paediatrics, Hospital Universitario de Mostoles, Mostoles, Spain.
Department of Paediatrics, Hospital Puerta del Sur, Mostoles, Spain.
BMJ Case Rep. 2018 Mar 27;2018:bcr-2017-223209. doi: 10.1136/bcr-2017-223209.
A 13-month-old boy with sickle cell disease (SCD) from Equatorial Guinea, who had recently arrived in Spain, presented with fever. He had suffered from malaria and had received a blood transfusion. Following physical examination and complementary tests, intravenous antibiotics and a red blood cell (RBC) transfusion were administered. Soon after a second transfusion 5 days later, the haemoglobin level fell below pretransfusion levels, together with reticulocytopenia, and haematuria-the so-called hyperhaemolysis syndrome-requiring intensive care and treatment with intravenous immunoglobulins and corticosteroids, with resolution of the complication. We want to emphasise the importance of suspecting this rare, though severe complication that can appear after any RBC transfusion especially in patients with SCD, as the clinical syndrome can simulate other more common complications of these patients and a further transfusion is contraindicated. There is no standardised treatment, but intravenous immunoglobulin and corticosteroids are usually effective.
一名来自赤道几内亚的13个月大镰状细胞病(SCD)男孩,近期抵达西班牙后出现发热症状。他曾患疟疾并接受过输血治疗。经过体格检查和辅助检查后,给予了静脉抗生素治疗及红细胞(RBC)输血。5天后第二次输血后不久,血红蛋白水平降至输血前水平以下,同时出现网织红细胞减少和血尿,即所谓的高溶血综合征,需要重症监护并使用静脉注射免疫球蛋白和皮质类固醇进行治疗,并发症得以缓解。我们想强调的是,要怀疑这种虽罕见但严重的并发症,它可能出现在任何红细胞输血后,尤其是在镰状细胞病患者中,因为该临床综合征可能会模拟这些患者其他更常见的并发症,且再次输血是禁忌的。目前尚无标准化治疗方法,但静脉注射免疫球蛋白和皮质类固醇通常有效。