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Autoimmune Hemolytic Anemia as a Complication of Congenital Anemias. A Case Series and Review of the Literature
Congenital anemias may be complicated by immune-mediated hemolytic crisis. Alloantibodies are usually seen in chronically transfused patients, and autoantibodies have also been described, although they are rarely associated with overt autoimmune hemolytic anemia (AIHA), a serious and potentially lif...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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MDPI
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8347040/ https://www.ncbi.nlm.nih.gov/pubmed/34362222 http://dx.doi.org/10.3390/jcm10153439 |
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author | Motta, Irene Giannotta, Juri Ferraresi, Marta Barbullushi, Kordelia Revelli, Nicoletta Graziadei, Giovanna Barcellini, Wilma Fattizzo, Bruno |
author_facet | Motta, Irene Giannotta, Juri Ferraresi, Marta Barbullushi, Kordelia Revelli, Nicoletta Graziadei, Giovanna Barcellini, Wilma Fattizzo, Bruno |
author_sort | Motta, Irene |
collection | PubMed |
description | Congenital anemias may be complicated by immune-mediated hemolytic crisis. Alloantibodies are usually seen in chronically transfused patients, and autoantibodies have also been described, although they are rarely associated with overt autoimmune hemolytic anemia (AIHA), a serious and potentially life-threatening complication. Given the lack of data on the AIHA diagnosis and management in congenital anemias, we retrospectively evaluated all clinically relevant AIHA cases occurring at a referral center for AIHA, hemoglobinopathies, and chronic hemolytic anemias, focusing on clinical management and outcome. In our cohort, AIHA had a prevalence of 1% (14/1410 patients). The majority were warm AIHA. Possible triggers were recent transfusion, infection, pregnancy, and surgery. All the patients received steroid therapy as the first line, and about 25% required further treatment, including rituximab, azathioprine, intravenous immunoglobulins, and cyclophosphamide. Transfusion support was required in 57% of the patients with non-transfusion-dependent anemia, and recombinant human erythropoietin was safely administered in one third of the patients. AIHA in congenital anemias may be challenging both from a diagnostic and a therapeutic point of view. A proper evaluation of hemolytic markers, bone marrow compensation, and assessment of the direct antiglobulin test is mandatory. |
format | Online Article Text |
id | pubmed-8347040 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-83470402021-08-08 Autoimmune Hemolytic Anemia as a Complication of Congenital Anemias. A Case Series and Review of the Literature Motta, Irene Giannotta, Juri Ferraresi, Marta Barbullushi, Kordelia Revelli, Nicoletta Graziadei, Giovanna Barcellini, Wilma Fattizzo, Bruno J Clin Med Article Congenital anemias may be complicated by immune-mediated hemolytic crisis. Alloantibodies are usually seen in chronically transfused patients, and autoantibodies have also been described, although they are rarely associated with overt autoimmune hemolytic anemia (AIHA), a serious and potentially life-threatening complication. Given the lack of data on the AIHA diagnosis and management in congenital anemias, we retrospectively evaluated all clinically relevant AIHA cases occurring at a referral center for AIHA, hemoglobinopathies, and chronic hemolytic anemias, focusing on clinical management and outcome. In our cohort, AIHA had a prevalence of 1% (14/1410 patients). The majority were warm AIHA. Possible triggers were recent transfusion, infection, pregnancy, and surgery. All the patients received steroid therapy as the first line, and about 25% required further treatment, including rituximab, azathioprine, intravenous immunoglobulins, and cyclophosphamide. Transfusion support was required in 57% of the patients with non-transfusion-dependent anemia, and recombinant human erythropoietin was safely administered in one third of the patients. AIHA in congenital anemias may be challenging both from a diagnostic and a therapeutic point of view. A proper evaluation of hemolytic markers, bone marrow compensation, and assessment of the direct antiglobulin test is mandatory. MDPI 2021-08-02 /pmc/articles/PMC8347040/ /pubmed/34362222 http://dx.doi.org/10.3390/jcm10153439 Text en © 2021 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 | Article Motta, Irene Giannotta, Juri Ferraresi, Marta Barbullushi, Kordelia Revelli, Nicoletta Graziadei, Giovanna Barcellini, Wilma Fattizzo, Bruno Autoimmune Hemolytic Anemia as a Complication of Congenital Anemias. A Case Series and Review of the Literature |
title | Autoimmune Hemolytic Anemia as a Complication of Congenital Anemias. A Case Series and Review of the Literature |
title_full | Autoimmune Hemolytic Anemia as a Complication of Congenital Anemias. A Case Series and Review of the Literature |
title_fullStr | Autoimmune Hemolytic Anemia as a Complication of Congenital Anemias. A Case Series and Review of the Literature |
title_full_unstemmed | Autoimmune Hemolytic Anemia as a Complication of Congenital Anemias. A Case Series and Review of the Literature |
title_short | Autoimmune Hemolytic Anemia as a Complication of Congenital Anemias. A Case Series and Review of the Literature |
title_sort | autoimmune hemolytic anemia as a complication of congenital anemias. a case series and review of the literature |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8347040/ https://www.ncbi.nlm.nih.gov/pubmed/34362222 http://dx.doi.org/10.3390/jcm10153439 |
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