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Autoimmune hemolytic anemia: current knowledge and perspectives

Autoimmune hemolytic anemia (AIHA) is an acquired, heterogeneous group of diseases which includes warm AIHA, cold agglutinin disease (CAD), mixed AIHA, paroxysmal cold hemoglobinuria and atypical AIHA. Currently CAD is defined as a chronic, clonal lymphoproliferative disorder, while the presence of...

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Autores principales: Michalak, Sylwia Sulimiera, Olewicz-Gawlik, Anna, Rupa-Matysek, Joanna, Wolny-Rokicka, Edyta, Nowakowska, Elżbieta, Gil, Lidia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7677104/
https://www.ncbi.nlm.nih.gov/pubmed/33292368
http://dx.doi.org/10.1186/s12979-020-00208-7
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author Michalak, Sylwia Sulimiera
Olewicz-Gawlik, Anna
Rupa-Matysek, Joanna
Wolny-Rokicka, Edyta
Nowakowska, Elżbieta
Gil, Lidia
author_facet Michalak, Sylwia Sulimiera
Olewicz-Gawlik, Anna
Rupa-Matysek, Joanna
Wolny-Rokicka, Edyta
Nowakowska, Elżbieta
Gil, Lidia
author_sort Michalak, Sylwia Sulimiera
collection PubMed
description Autoimmune hemolytic anemia (AIHA) is an acquired, heterogeneous group of diseases which includes warm AIHA, cold agglutinin disease (CAD), mixed AIHA, paroxysmal cold hemoglobinuria and atypical AIHA. Currently CAD is defined as a chronic, clonal lymphoproliferative disorder, while the presence of cold agglutinins underlying other diseases is known as cold agglutinin syndrome. AIHA is mediated by autoantibodies directed against red blood cells (RBCs) causing premature erythrocyte destruction. The pathogenesis of AIHA is complex and still not fully understood. Recent studies indicate the involvement of T and B cell dysregulation, reduced CD4+ and CD25+ Tregs, increased clonal expansions of CD8 + T cells, imbalance of Th17/Tregs and Tfh/Tfr, and impaired lymphocyte apoptosis. Changes in some RBC membrane structures, under the influence of mechanical stimuli or oxidative stress, may promote autohemolysis. The clinical presentation and treatment of AIHA are influenced by many factors, including the type of AIHA, degree of hemolysis, underlying diseases, presence of concomitant comorbidities, bone marrow compensatory abilities and the presence of fibrosis and dyserthropoiesis. The main treatment for AIHA is based on the inhibition of autoantibody production by mono- or combination therapy using GKS and/or rituximab and, rarely, immunosuppressive drugs or immunomodulators. Reduction of erythrocyte destruction via splenectomy is currently the third line of treatment for warm AIHA. Supportive treatment including vitamin supplementation, recombinant erythropoietin, thrombosis prophylaxis and the prevention and treatment of infections is essential. New groups of drugs that inhibit immune responses at various levels are being developed intensively, including inhibition of antibody-mediated RBCs phagocytosis, inhibition of B cell and plasma cell frequency and activity, inhibition of IgG recycling, immunomodulation of T lymphocytes function, and complement cascade inhibition. Recent studies have brought about changes in classification and progress in understanding the pathogenesis and treatment of AIHA, although there are still many issues to be resolved, particularly concerning the impact of age-associated changes to immunity.
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spelling pubmed-76771042020-11-20 Autoimmune hemolytic anemia: current knowledge and perspectives Michalak, Sylwia Sulimiera Olewicz-Gawlik, Anna Rupa-Matysek, Joanna Wolny-Rokicka, Edyta Nowakowska, Elżbieta Gil, Lidia Immun Ageing Review Autoimmune hemolytic anemia (AIHA) is an acquired, heterogeneous group of diseases which includes warm AIHA, cold agglutinin disease (CAD), mixed AIHA, paroxysmal cold hemoglobinuria and atypical AIHA. Currently CAD is defined as a chronic, clonal lymphoproliferative disorder, while the presence of cold agglutinins underlying other diseases is known as cold agglutinin syndrome. AIHA is mediated by autoantibodies directed against red blood cells (RBCs) causing premature erythrocyte destruction. The pathogenesis of AIHA is complex and still not fully understood. Recent studies indicate the involvement of T and B cell dysregulation, reduced CD4+ and CD25+ Tregs, increased clonal expansions of CD8 + T cells, imbalance of Th17/Tregs and Tfh/Tfr, and impaired lymphocyte apoptosis. Changes in some RBC membrane structures, under the influence of mechanical stimuli or oxidative stress, may promote autohemolysis. The clinical presentation and treatment of AIHA are influenced by many factors, including the type of AIHA, degree of hemolysis, underlying diseases, presence of concomitant comorbidities, bone marrow compensatory abilities and the presence of fibrosis and dyserthropoiesis. The main treatment for AIHA is based on the inhibition of autoantibody production by mono- or combination therapy using GKS and/or rituximab and, rarely, immunosuppressive drugs or immunomodulators. Reduction of erythrocyte destruction via splenectomy is currently the third line of treatment for warm AIHA. Supportive treatment including vitamin supplementation, recombinant erythropoietin, thrombosis prophylaxis and the prevention and treatment of infections is essential. New groups of drugs that inhibit immune responses at various levels are being developed intensively, including inhibition of antibody-mediated RBCs phagocytosis, inhibition of B cell and plasma cell frequency and activity, inhibition of IgG recycling, immunomodulation of T lymphocytes function, and complement cascade inhibition. Recent studies have brought about changes in classification and progress in understanding the pathogenesis and treatment of AIHA, although there are still many issues to be resolved, particularly concerning the impact of age-associated changes to immunity. BioMed Central 2020-11-20 /pmc/articles/PMC7677104/ /pubmed/33292368 http://dx.doi.org/10.1186/s12979-020-00208-7 Text en © The Author(s) 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Review
Michalak, Sylwia Sulimiera
Olewicz-Gawlik, Anna
Rupa-Matysek, Joanna
Wolny-Rokicka, Edyta
Nowakowska, Elżbieta
Gil, Lidia
Autoimmune hemolytic anemia: current knowledge and perspectives
title Autoimmune hemolytic anemia: current knowledge and perspectives
title_full Autoimmune hemolytic anemia: current knowledge and perspectives
title_fullStr Autoimmune hemolytic anemia: current knowledge and perspectives
title_full_unstemmed Autoimmune hemolytic anemia: current knowledge and perspectives
title_short Autoimmune hemolytic anemia: current knowledge and perspectives
title_sort autoimmune hemolytic anemia: current knowledge and perspectives
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7677104/
https://www.ncbi.nlm.nih.gov/pubmed/33292368
http://dx.doi.org/10.1186/s12979-020-00208-7
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