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

Abstract

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‐P1 and IgG anti‐Jka were detected in blood samples the next day after the last transfusion. In the six donors, two were negative for P1 and Jka, one was positive for P1 and negative for Jka, and three were negative for P1 and positive for Jka. 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 Jka‐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.

Case Report: Drug-Induced Immune Haemolytic Anaemia Caused by Cefoperazone-Tazobactam/ Sulbactam Combination Therapy

“…This may be attributed to the potential destruction of reticulocytes during haemolysis. Macrophages in the endothelial system may play a key role in lysis of reticulocytes (27)(28)(29). In this patient, the percentage of reticulocytes was significantly below the reference level during the severe haemolysis that followed the fourth cycle of cefoperazone treatment.…”

Epidemiological and clinical features, therapeutic strategies and outcomes in patients with hyperhaemolysis: A systematic review

“…Additionally, many studies did not meet our strict inclusion criteria, particularly those lacking either qualitative or quantitative reticulocyte data or those defining HHS by reticulocytosis. Since reticulocytopenia is a well‐documented finding in HHS, 4,34 measurement of reticulocytes and comparison to the patient's baseline is recommended to aid in the diagnosis of HHS at its onset, with recovery manifesting as reticulocytosis 35 . However, these limitations also represent a strength, as only definitive HHS cases were analysed, allowing us to characterize the features of this rare reaction.…”

“…Since reticulocytopenia is a well-documented finding in HHS, 4,34 measurement of reticulocytes and comparison to the patient's baseline is recommended to aid in the diagnosis of HHS at its onset, with recovery manifesting as reticulocytosis. 35 However, these limitations also represent a strength, as only definitive HHS cases were analysed, allowing us to characterize the features of this rare reaction. Prior studies that have been more inclusive may be biased by cases that were not HHS.…”

Hyperhaemolysis Syndrome: A Review of Cases

“…The diagnostic criteria for HHS in SCD patients should consist of three criteria [3]. Firstly, there should be an unexplained reduction in haemoglobin level after blood transfusion to a level lower than the pre-transfusion level.…”