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Autoimmune Hemolytic Anemia in the Pediatric Setting
Autoimmune hemolytic anemia (AIHA) is a rare disease in children, presenting with variable severity. Most commonly, warm-reactive IgG antibodies bind erythrocytes at 37 °C and induce opsonization and phagocytosis mainly by the splenic macrophages, causing warm AIHA (w-AIHA). Post-infectious cold-rea...
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/PMC7828053/ https://www.ncbi.nlm.nih.gov/pubmed/33435309 http://dx.doi.org/10.3390/jcm10020216 |
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author | Voulgaridou, Aikaterini Kalfa, Theodosia A. |
author_facet | Voulgaridou, Aikaterini Kalfa, Theodosia A. |
author_sort | Voulgaridou, Aikaterini |
collection | PubMed |
description | Autoimmune hemolytic anemia (AIHA) is a rare disease in children, presenting with variable severity. Most commonly, warm-reactive IgG antibodies bind erythrocytes at 37 °C and induce opsonization and phagocytosis mainly by the splenic macrophages, causing warm AIHA (w-AIHA). Post-infectious cold-reactive antibodies can also lead to hemolysis following the patient’s exposure to cold temperatures, causing cold agglutinin syndrome (CAS) due to IgM autoantibodies, or paroxysmal cold hemoglobinuria (PCH) due to atypical IgG autoantibodies which bind their target RBC antigen and fix complement at 4 °C. Cold-reactive antibodies mainly induce intravascular hemolysis after complement activation. Direct antiglobulin test (DAT) is the gold standard for AIHA diagnosis; however, DAT negative results are seen in up to 11% of warm AIHA, highlighting the need to pursue further evaluation in cases with a phenotype compatible with immune-mediated hemolytic anemia despite negative DAT. Prompt supportive care, initiation of treatment with steroids for w-AIHA, and transfusion if necessary for symptomatic or fast-evolving anemia is crucial for a positive outcome. w-AIHA in children is often secondary to underlying immune dysregulation syndromes and thus, screening for such disorders is recommended at presentation, before initiating treatment with immunosuppressants, to determine prognosis and optimize long-term management potentially with novel targeted medications. |
format | Online Article Text |
id | pubmed-7828053 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-78280532021-01-25 Autoimmune Hemolytic Anemia in the Pediatric Setting Voulgaridou, Aikaterini Kalfa, Theodosia A. J Clin Med Review Autoimmune hemolytic anemia (AIHA) is a rare disease in children, presenting with variable severity. Most commonly, warm-reactive IgG antibodies bind erythrocytes at 37 °C and induce opsonization and phagocytosis mainly by the splenic macrophages, causing warm AIHA (w-AIHA). Post-infectious cold-reactive antibodies can also lead to hemolysis following the patient’s exposure to cold temperatures, causing cold agglutinin syndrome (CAS) due to IgM autoantibodies, or paroxysmal cold hemoglobinuria (PCH) due to atypical IgG autoantibodies which bind their target RBC antigen and fix complement at 4 °C. Cold-reactive antibodies mainly induce intravascular hemolysis after complement activation. Direct antiglobulin test (DAT) is the gold standard for AIHA diagnosis; however, DAT negative results are seen in up to 11% of warm AIHA, highlighting the need to pursue further evaluation in cases with a phenotype compatible with immune-mediated hemolytic anemia despite negative DAT. Prompt supportive care, initiation of treatment with steroids for w-AIHA, and transfusion if necessary for symptomatic or fast-evolving anemia is crucial for a positive outcome. w-AIHA in children is often secondary to underlying immune dysregulation syndromes and thus, screening for such disorders is recommended at presentation, before initiating treatment with immunosuppressants, to determine prognosis and optimize long-term management potentially with novel targeted medications. MDPI 2021-01-09 /pmc/articles/PMC7828053/ /pubmed/33435309 http://dx.doi.org/10.3390/jcm10020216 Text en © 2021 by the authors. 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 (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Voulgaridou, Aikaterini Kalfa, Theodosia A. Autoimmune Hemolytic Anemia in the Pediatric Setting |
title | Autoimmune Hemolytic Anemia in the Pediatric Setting |
title_full | Autoimmune Hemolytic Anemia in the Pediatric Setting |
title_fullStr | Autoimmune Hemolytic Anemia in the Pediatric Setting |
title_full_unstemmed | Autoimmune Hemolytic Anemia in the Pediatric Setting |
title_short | Autoimmune Hemolytic Anemia in the Pediatric Setting |
title_sort | autoimmune hemolytic anemia in the pediatric setting |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7828053/ https://www.ncbi.nlm.nih.gov/pubmed/33435309 http://dx.doi.org/10.3390/jcm10020216 |
work_keys_str_mv | AT voulgaridouaikaterini autoimmunehemolyticanemiainthepediatricsetting AT kalfatheodosiaa autoimmunehemolyticanemiainthepediatricsetting |