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The Role of the Spleen and the Place of Splenectomy in Autoimmune Hemolytic Anemia—A Review of Current Knowledge

Autoimmune hemolytic anemia (AIHA) is a rare, very heterogeneous, and sometimes life-threatening acquired hematologic disease characterized by increased red blood cell (RBC) destruction by autoantibodies (autoAbs), either with or without complement involvement. Recent studies have shown that the inv...

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Autores principales: Cvetković, Zorica, Pantić, Nikola, Cvetković, Mirjana, Virijević, Marijana, Sabljić, Nikica, Marinković, Gligorije, Milosavljević, Vladimir, Pravdić, Zlatko, Suvajdžić-Vuković, Nada, Mitrović, Mirjana
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10527817/
https://www.ncbi.nlm.nih.gov/pubmed/37761258
http://dx.doi.org/10.3390/diagnostics13182891
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author Cvetković, Zorica
Pantić, Nikola
Cvetković, Mirjana
Virijević, Marijana
Sabljić, Nikica
Marinković, Gligorije
Milosavljević, Vladimir
Pravdić, Zlatko
Suvajdžić-Vuković, Nada
Mitrović, Mirjana
author_facet Cvetković, Zorica
Pantić, Nikola
Cvetković, Mirjana
Virijević, Marijana
Sabljić, Nikica
Marinković, Gligorije
Milosavljević, Vladimir
Pravdić, Zlatko
Suvajdžić-Vuković, Nada
Mitrović, Mirjana
author_sort Cvetković, Zorica
collection PubMed
description Autoimmune hemolytic anemia (AIHA) is a rare, very heterogeneous, and sometimes life-threatening acquired hematologic disease characterized by increased red blood cell (RBC) destruction by autoantibodies (autoAbs), either with or without complement involvement. Recent studies have shown that the involvement of T- and B-cell dysregulation and an imbalance of T-helper 2 (Th2) and Th17 phenotypes play major roles in the pathogenesis of AIHA. AIHA can be primary (idiopathic) but is more often secondary, triggered by infections or drug use or as a part of other diseases. As the location of origin of autoAbs and the location of autoAb-mediated RBC clearance, as well as the location of extramedullary hematopoiesis, the spleen is crucially involved in all the steps of AIHA pathobiology. Splenectomy, which was the established second-line therapeutic option in corticosteroid-resistant AIHA patients for decades, has become less common due to increasing knowledge of immunopathogenesis and the introduction of targeted therapy. This article provides a comprehensive overview of current knowledge regarding the place of the spleen in the immunological background of AIHA and the rapidly growing spectrum of novel therapeutic approaches. Furthermore, this review emphasizes the still-existing expediency of laparoscopic splenectomy with appropriate perioperative thromboprophylaxis and the prevention of infection as a safe and reliable therapeutic option in the context of the limited availability of rituximab and other novel therapies.
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spelling pubmed-105278172023-09-28 The Role of the Spleen and the Place of Splenectomy in Autoimmune Hemolytic Anemia—A Review of Current Knowledge Cvetković, Zorica Pantić, Nikola Cvetković, Mirjana Virijević, Marijana Sabljić, Nikica Marinković, Gligorije Milosavljević, Vladimir Pravdić, Zlatko Suvajdžić-Vuković, Nada Mitrović, Mirjana Diagnostics (Basel) Review Autoimmune hemolytic anemia (AIHA) is a rare, very heterogeneous, and sometimes life-threatening acquired hematologic disease characterized by increased red blood cell (RBC) destruction by autoantibodies (autoAbs), either with or without complement involvement. Recent studies have shown that the involvement of T- and B-cell dysregulation and an imbalance of T-helper 2 (Th2) and Th17 phenotypes play major roles in the pathogenesis of AIHA. AIHA can be primary (idiopathic) but is more often secondary, triggered by infections or drug use or as a part of other diseases. As the location of origin of autoAbs and the location of autoAb-mediated RBC clearance, as well as the location of extramedullary hematopoiesis, the spleen is crucially involved in all the steps of AIHA pathobiology. Splenectomy, which was the established second-line therapeutic option in corticosteroid-resistant AIHA patients for decades, has become less common due to increasing knowledge of immunopathogenesis and the introduction of targeted therapy. This article provides a comprehensive overview of current knowledge regarding the place of the spleen in the immunological background of AIHA and the rapidly growing spectrum of novel therapeutic approaches. Furthermore, this review emphasizes the still-existing expediency of laparoscopic splenectomy with appropriate perioperative thromboprophylaxis and the prevention of infection as a safe and reliable therapeutic option in the context of the limited availability of rituximab and other novel therapies. MDPI 2023-09-09 /pmc/articles/PMC10527817/ /pubmed/37761258 http://dx.doi.org/10.3390/diagnostics13182891 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Cvetković, Zorica
Pantić, Nikola
Cvetković, Mirjana
Virijević, Marijana
Sabljić, Nikica
Marinković, Gligorije
Milosavljević, Vladimir
Pravdić, Zlatko
Suvajdžić-Vuković, Nada
Mitrović, Mirjana
The Role of the Spleen and the Place of Splenectomy in Autoimmune Hemolytic Anemia—A Review of Current Knowledge
title The Role of the Spleen and the Place of Splenectomy in Autoimmune Hemolytic Anemia—A Review of Current Knowledge
title_full The Role of the Spleen and the Place of Splenectomy in Autoimmune Hemolytic Anemia—A Review of Current Knowledge
title_fullStr The Role of the Spleen and the Place of Splenectomy in Autoimmune Hemolytic Anemia—A Review of Current Knowledge
title_full_unstemmed The Role of the Spleen and the Place of Splenectomy in Autoimmune Hemolytic Anemia—A Review of Current Knowledge
title_short The Role of the Spleen and the Place of Splenectomy in Autoimmune Hemolytic Anemia—A Review of Current Knowledge
title_sort role of the spleen and the place of splenectomy in autoimmune hemolytic anemia—a review of current knowledge
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10527817/
https://www.ncbi.nlm.nih.gov/pubmed/37761258
http://dx.doi.org/10.3390/diagnostics13182891
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