依库珠单抗治疗镰状细胞病的高溶血性和再生障碍性危象
Eculizumab as a Treatment for Hyper-Haemolytic and Aplastic Crisis in Sickle Cell Disease.
作者信息
Patel Ishan, Odak Mihir, Douedi Steven, Alshami Abbas, Upadhyaya Vandan D, Hossain Mohammad, Anne Madhurima, Patel Swapnil V
机构信息
Department of Medicine, University of Iowa Hospitals and Clinics, Iowa City, IA, USA.
Department of Medicine, Hackensack Meridian Jersey Shore University Medical Center, Neptune, NJ, USA.
出版信息
Eur J Case Rep Intern Med. 2021 Aug 7;8(10):002824. doi: 10.12890/2021_002824. eCollection 2021.
BACKGROUND
Patients with sickle cell disease can experience various crises including sequestration crisis, haemolytic crisis and aplastic crisis. Due to alloantibody formation, transfusion alloantibodies can cause a haemolytic crisis. Treatment involves avoiding packed red blood cell transfusions, as well as intravenous immunoglobulin, steroids and eculizumab to decrease the chances of haemolysis.
CASE DESCRIPTION
We report the case of a 42-year-old man who was found to have worsening anaemia after packed red blood cell transfusion with evidence suggestive of haemolytic crisis. Due to reticulocytopenia, aplastic crisis was also suspected and later confirmed via parvovirus IgG and IgM titres. The patient did not improve with steroid and intravenous immunoglobulin therapy and was treated with eculizumab as a salvage therapy.
CONCLUSION
Concurrent hyper-haemolytic crisis and aplastic crisis should be suspected in patients with features of haemolysis and reticulocytopenia. Prompt recognition and treatment with eculizumab are paramount in those who fail steroid and intravenous immunoglobulin treatment.
LEARNING POINTS
Treatment of hyper-haemolytic and aplastic crisis in sickle cell disease with eculizumab offers therapeutic benefit.A high index of suspicion for hyper-haemolytic crisis and aplastic crisis should be maintained in those with haemolytic features as well as reticulocytopenia in the setting of sickle cell disease.
背景
镰状细胞病患者可能会经历各种危机,包括脾隔离危象、溶血危象和再生障碍危象。由于同种抗体的形成,输血同种抗体可引发溶血危象。治疗措施包括避免输注浓缩红细胞,以及使用静脉注射免疫球蛋白、类固醇和依库珠单抗以降低溶血几率。
病例描述
我们报告了一名42岁男性的病例,该患者在输注浓缩红细胞后出现贫血加重,并有溶血危象的迹象。由于网织红细胞减少,也怀疑有再生障碍危象,后来通过细小病毒IgG和IgM滴度得以确诊。该患者接受类固醇和静脉注射免疫球蛋白治疗后并无改善,遂接受依库珠单抗作为挽救治疗。
结论
对于有溶血和网织红细胞减少特征的患者,应怀疑同时存在高溶血性危机和再生障碍危象。对于类固醇和静脉注射免疫球蛋白治疗无效的患者,及时识别并使用依库珠单抗治疗至关重要。
经验教训
使用依库珠单抗治疗镰状细胞病的高溶血性和再生障碍危象具有治疗益处。对于镰状细胞病患者,若有溶血特征以及网织红细胞减少,应高度怀疑高溶血性危机和再生障碍危象。