Department of Blood Transfusion, Dongguan Maternal and Child Health Hospital, Dongguan, China.
Institute of Clinical Blood Transfusion, Guangzhou Blood Center, Guangzhou, China.
Transfus Med. 2021 Feb;31(1):24-29. doi: 10.1111/tme.12748. Epub 2020 Dec 16.
To report a case of hyperhaemolysis syndrome (HHS) that occurred during perinatal blood transfusion in a pregnant Chinese woman with β-thalassemia to deepen the understanding of HHS and the risk of transfusion therapy for patients with thalassemia.
Most HHS cases occur in people with sickle cell disease. So far, no cases of HHS have been reported in the Chinese population. Here, we report a pregnant Chinese women with β-thalassemia experiencing HHS.
The patient received ABO- and RhD-matched red blood cell transfusion from six blood donors in four perinatal transfusions. Haemoglobinuria and lower haemoglobin levels compared to those before transfusion were observed after each transfusion, and the lactate dehydrogenase was consistently elevated. The blood samples were collected at different time points during the hospitalisation for direct antiglobulin test (DAT), antibody screening test and acid elution test. The antigens of six blood donors were identified, and the cross-matching tests were repeated using the blood sample of the patient with specific irregular antibodies after the last transfusion.
The DAT of the patient was negative for anti-IgG and positive (1+) for anti-C3d, and no red blood cell antibodies were detected in the eluent before, between and after transfusions. Before and between transfusions, blood samples were negative for red blood cell irregular antibodies, whereas IgM anti-P and IgG anti-Jk were detected in blood samples the next day after the last transfusion. In the six donors, two were negative for P and Jk , one was positive for P and negative for Jk , and three were negative for P and positive for Jk . The tentative cross-matching tests using the indirect antiglobulin method in saline showed that only agglutination occurred in the blood samples of the patient collected after last transfusion and the three Jk -positive blood donors.
The clinical manifestations and laboratory test results suggested that HHS occurred in this patient with β-thalassemia after each transfusion. Clinicians should be aware that HHS can occur with compatible blood transfusion.
报道 1 例围产期输血期间发生于中国妊娠β地中海贫血妇女的高红细胞溶解综合征（HHS）病例，以加深对 HHS 及地中海贫血患者输血治疗风险的认识。
大多数 HHS 病例发生于镰状细胞病患者。迄今为止，尚未有中国人群中发生 HHS 的报道。现报道 1 例妊娠β地中海贫血妇女发生 HHS。
该患者在 4 次围产期输血中共接受了来自 6 名献血者的 ABO 和 RhD 均匹配的红细胞输注。每次输血后均观察到血红蛋白尿和血红蛋白水平较输血前下降，且乳酸脱氢酶持续升高。在住院期间于不同时间点采集血样进行直接抗球蛋白试验（DAT）、抗体筛查试验和酸洗脱试验。鉴定 6 名献血者的抗原，并在最后一次输血后使用患者具有特异性不规则抗体的血样重复进行交叉配血试验。
患者的 DAT 对 IgG 阴性，对 C3d 阳性（1+），且在输血前后洗脱液中均未检测到红细胞抗体。在输血前和输血期间，血样均为红细胞不规则抗体阴性，而在最后一次输血后第 2 天的血样中检测到 IgM 抗-P 和 IgG 抗-Jk。在 6 名供者中，2 名为 P 和 Jk 阴性，1 名为 P 阳性、Jk 阴性，3 名为 P 和 Jk 均阴性。使用盐水间接抗球蛋白法进行的初步交叉配血试验显示，仅在最后一次输血后采集的患者血样和 3 名 Jk 阳性供者血样中发生凝集。
临床表现和实验室检验结果提示，该β地中海贫血患者在每次输血后均发生 HHS。临床医生应意识到 HHS 可能在相容输血中发生。
