Department of Pathology, University of Chicago, Chicago, Illinois, USA.
Department of Medicine, Section of Hematology/Oncology, University of Chicago, Chicago, Illinois, USA.
Transfusion. 2022 Jul;62(7):1446-1451. doi: 10.1111/trf.16932. Epub 2022 May 30.
Hyperhemolysis syndrome (HHS) is a severe delayed hemolytic transfusion reaction seen in sickle cell disease (SCD) patients, characterized by destruction of donor and recipient RBCs. It results in a drop in hemoglobin to below pretransfusion levels and frequently reticulocytopenia.
We report a case of a man in his thirties with SCD with a recent hospitalization 2 weeks prior for COVID-19. His red cell antibody history included anti-Fy(a) and warm autoantibody. At that time, he was given 2 units of RBC and discharged with a hemoglobin of 10.2 g/dl. He returned to the hospital approximately 1.5 weeks later with hemoglobin 6.0 g/dl and symptoms concerning for acute chest syndrome. Pretransfusion testing now showed 4+ pan-agglutinin in both gel-based and tube-based testing. Alloadsorption identified an anti-N and a strong cold agglutinin. Three least incompatible units were transfused to this patient over several days, with evidence of hemolysis. Further reference lab work revealed anti-Fy , anti-Fy , anti-Le , anti-Le , and an anti-KN system antibody. The patient's hemoglobin nadired at 4.4 g/dl. The patient was treated with a single dose of tocilizumab, his hemoglobin stabilized, and he was discharged.
We present a case of HHS proximate to recent SARS-CoV-2 infection with multiple allo and autoantibodies identified. Information on the relationship between SARS-CoV-2 infection and HHS is limited; however, it is possible that inflammation related to COVID-19 could predispose to HHS. Tocilizumab is an approved treatment for COVID-19. Additionally, tocilizumab appears to be a promising treatment option for patients with HHS.
高红细胞溶解综合征（HHS）是一种在镰状细胞病（SCD）患者中出现的严重迟发性溶血性输血反应，其特征为供者和受者 RBC 的破坏。它导致血红蛋白降至输血前水平以下，常伴有网织红细胞减少。
我们报告了一例三十多岁的 SCD 男性患者，最近一次住院是在 2 周前因 COVID-19。他的红细胞抗体病史包括抗-Fy(a)和温自身抗体。当时，他接受了 2 单位 RBC 输血，并出院时血红蛋白为 10.2 g/dl。大约 1.5 周后，他因血红蛋白 6.0 g/dl 和急性胸痛综合征的症状返回医院。输血前检测现在显示凝胶和试管基础检测均为 4+全凝集。 alloadsorption 鉴定出抗-N 和强冷自身抗体。在数天内给该患者输注了 3 个最少不相容单位的 RBC，有溶血证据。进一步的参考实验室工作显示抗-Fy(a)、抗-Fy(b)、抗-Le(a)、抗-Le(b)和抗-KN 系统抗体。患者的血红蛋白最低值为 4.4 g/dl。该患者接受了托珠单抗单次治疗，血红蛋白稳定，随后出院。
我们报告了一例 HHS 病例，该病例发生在最近 SARS-CoV-2 感染后，发现了多种同种异体和自身抗体。关于 SARS-CoV-2 感染与 HHS 之间的关系的信息有限；然而， COVID-19 相关炎症可能使 HHS 易于发生。托珠单抗是 COVID-19 的一种批准治疗药物。此外，托珠单抗似乎是 HHS 患者的一种有前途的治疗选择。
