Epidemiological and clinical features, therapeutic strategies and outcomes in patients with hyperhaemolysis: A systematic review
Corresponding Author
Jeremy W. Jacobs
Department of Laboratory Medicine, Yale School of Medicine, New Haven, Connecticut, USA
Correspondence
Jeremy W. Jacobs, 55 Park St, New Haven, CT 06511, USA.
Email: [email protected]
Search for more papers by this authorLaura D. Stephens
Department of Pathology, University of California San Diego, La Jolla, California, USA
Search for more papers by this authorElizabeth S. Allen
Department of Pathology, University of California San Diego, La Jolla, California, USA
Search for more papers by this authorThomas C. Binns
Department of Laboratory Medicine, Yale School of Medicine, New Haven, Connecticut, USA
Search for more papers by this authorGarrett S. Booth
Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, Tennessee, USA
Search for more papers by this authorJeanne E. Hendrickson
Department of Laboratory Medicine, Yale School of Medicine, New Haven, Connecticut, USA
Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA
Search for more papers by this authorMatthew S. Karafin
Department of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill, North Carolina, USA
Search for more papers by this authorChristopher A. Tormey
Department of Laboratory Medicine, Yale School of Medicine, New Haven, Connecticut, USA
Search for more papers by this authorJennifer S. Woo
Department of Pathology, City of Hope National Medical Center, Irvine, California, USA
Search for more papers by this authorBrian D. Adkins
Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
Search for more papers by this authorCorresponding Author
Jeremy W. Jacobs
Department of Laboratory Medicine, Yale School of Medicine, New Haven, Connecticut, USA
Correspondence
Jeremy W. Jacobs, 55 Park St, New Haven, CT 06511, USA.
Email: [email protected]
Search for more papers by this authorLaura D. Stephens
Department of Pathology, University of California San Diego, La Jolla, California, USA
Search for more papers by this authorElizabeth S. Allen
Department of Pathology, University of California San Diego, La Jolla, California, USA
Search for more papers by this authorThomas C. Binns
Department of Laboratory Medicine, Yale School of Medicine, New Haven, Connecticut, USA
Search for more papers by this authorGarrett S. Booth
Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, Tennessee, USA
Search for more papers by this authorJeanne E. Hendrickson
Department of Laboratory Medicine, Yale School of Medicine, New Haven, Connecticut, USA
Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA
Search for more papers by this authorMatthew S. Karafin
Department of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill, North Carolina, USA
Search for more papers by this authorChristopher A. Tormey
Department of Laboratory Medicine, Yale School of Medicine, New Haven, Connecticut, USA
Search for more papers by this authorJennifer S. Woo
Department of Pathology, City of Hope National Medical Center, Irvine, California, USA
Search for more papers by this authorBrian D. Adkins
Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
Search for more papers by this authorJeremy W. Jacobs and Laura D. Stephens contributed equally to this study.
Summary
Hyperhaemolysis syndrome (HHS), a severe form of delayed haemolytic transfusion reaction most commonly described in patients with sickle cell disease (SCD), involves destruction of both donor and recipient red blood cells (RBCs). As the epidemiology and underlying pathophysiology have yet to be definitively elucidated, recognition can be challenging. We systematically reviewed PubMed and EMBASE to identify all cases of post-transfusion hyperhaemolysis and characterized the epidemiological, clinical and immunohaematological characteristics and treatments of HHS. We identified 51 patients (33 females and 18 males), including 31 patients with SCD (HbSS, HbSC and HbS/β-thalassaemia). The median haemoglobin nadir (3.9 g/dL) occurred a median of 10 days post-transfusion. 32.6% and 45.7% of patients had a negative indirect anti-globulin test and a negative direct anti-globulin test, respectively. The most common therapies included corticosteroids and intravenous immune globulin. 66.0% of patients received ≥1 supportive transfusion, which was associated with a longer median hospital stay/time to recovery (23 days vs. 15 days; p = 0.015) compared to no supportive transfusion. These findings illustrate that HHS that often results in marked anaemia 10 days post-transfusion is not restricted to patients with haemoglobinopathies, and additional transfused RBCs may be associated with a longer time-to-recovery.
Graphical Abstract
CONFLICT OF INTEREST STATEMENT
All authors declare no conflicts of interest related to this research. All authors have no relevant disclosures.
Open Research
DATA AVAILABILITY STATEMENT
For original data, please contact [email protected].
Supporting Information
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Table S1. |
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