Abstract
Autoimmune rheumatic disorders (ARD) represent a wide spectrum of disorders that affect
in priority the joints, bones, muscles, and connective tissues. Examples of ARD include
rheumatoid arthritis, systemic lupus erythematosus, Sjögren syndrome, polymyositis,
systemic sclerosis, antiphospholipid syndrome and mixed connective tissue disease.
Patients with ARD often require transfusion of red cell concentrates (RCC) or other
blood-derived components. The presence of an autoimmune background, often complicated
by the use of immunosuppressive medications, renders these patients quite vulnerable.
Exposing them to RCC, when indicated, can trigger transfusion-related immunomodulation
that can be aggravated by the role played by the donor microbiome, and the complement
activation and the immune dysregulation induced by iron, leading to an amplification
of the immune problems. Furthermore, patients are challenged by the transfused extracellular
vesicles which could have a potentially negative role, particularly in patients with
antiphospholipid syndrome. Despite the very vigorous screening, transfusion transmissible
infections can still represent a risk to these patients, particularly in cytomegalovirus
seronegative patients or when dormant pathogens are activated in the immunosuppressed
transfusion recipient. The ARD population is also more at risk for transfusion-related
reactions. One, therefore, has to consider a restrictive transfusion strategy if possible
and, if needed, resort to the numerous blood bank procedures to reduce the immunogenicity
of blood products or use safer, more targeted, industrial plasma-derived products
subjected to purification and pathogen reduction technologies.
Keywords
Abbreviations:
ARD (Autoimmune rheumatic disease), CMV (Cytomegalovirus), EVs (Extracellular vesicles), FFP (Fresh-frozen plasma), LPS (Lipopolysaccharide), LTA (Lipoteichoic acid), MAPMs (Microbe-associated molecular patterns), MCTD (Mixed connective tissue disease), MSCs (Mesenchymal stromal cells), PRP (Platelet-rich plasma), PRT (Pathogen reduction technologies), RA (Rheumatoid arthritis), RCC (Red blood cell concentrates), SCFA (Short-chain fatty acids), SLE (Systemic lupus erythematosus), TA-GvHD (Transfusion-associated graft versus host disease), TE (Thromboembolic), TLRs (Toll-like receptors), TRALI (Transfusion-related acute lung injury), TRIM (Transfusion-related immunomodulation), TT-CMV (Transmission of CMV by transfusion), TTIs (Transfusion-transmitted infections)To read this article in full you will need to make a payment
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Published online: October 28, 2022
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