A case of hyperhaemolysis syndrome in a pregnant Chinese woman with β-thalassemia during perinatal transfusion.

Objectives: 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.

Background: 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.

Methods: 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.

Results: 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.

Discussion: 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.