Pediatric autoimmune hemolytic anemia: A single-center retrospective study
Background: Autoimmune hemolytic anemia (AIHA) is a rare, life-threatening disease in pediatrics. This article describes the clinical features, diagnostic workup, treatment and outcome in patients with AIHA. Method: Medical charts of under 18-year-old patients with AIHA treated at a tertiary Brazili...
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Elsevier
2024-11-01
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| Series: | Hematology, Transfusion and Cell Therapy |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2531137924000166 |
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| author | Aline Sayuri Sakamoto Fernanda Silva Sequeira Bruna Paccola Blanco Marlene Pereira Garanito |
| author_facet | Aline Sayuri Sakamoto Fernanda Silva Sequeira Bruna Paccola Blanco Marlene Pereira Garanito |
| author_sort | Aline Sayuri Sakamoto |
| collection | DOAJ |
| description | Background: Autoimmune hemolytic anemia (AIHA) is a rare, life-threatening disease in pediatrics. This article describes the clinical features, diagnostic workup, treatment and outcome in patients with AIHA. Method: Medical charts of under 18-year-old patients with AIHA treated at a tertiary Brazilian institution from 2006 to 2021 were retrospectively reviewed. Data analysis was primarily descriptive, using medians, interquartile ranges, and categorical variables presented as absolute frequencies. Main results: Twenty-four patients (14 female, 10 male) were evaluated in this study. The median age at diagnosis was 5.99 years (range: 0.25–17.1 years) and the median hemoglobin level was 4.85 g/dL (range: 4.17–5.57 g/dL). Most had warm antibodies (83.3 %). Twelve patients (50 %) had known underlining diseases, four (16.6 %) presented with AIHA concomitant with acute infectious diseases and three (12.5 %) had an undetermined post-vaccine association. Steroids and intravenous immunoglobulin were first-line therapy in 23 cases. Seven patients (29.1 %) required second and third-line treatments (rituximab, cyclophosphamide and splenectomy). The median follow-up period was 4.4 years (range: 1.0–6.7 years). Thirteen patients (54.1 %) were discharged, five cases (20.8 %) were lost to follow-up and no patient died. The median age for the six remaining patients was 11.53 years (8.5–14.7) with all of them having complete responses with no further therapies. Conclusion: Most cases of AIHA are secondary to an underlying systemic disease or have a possible correlation with infections/vaccines and respond to steroids. The second and third-line therapies for refractory and relapse cases remain a dilemma. A prospective, multicenter study is essential to address the best therapeutic combinations. |
| format | Article |
| id | doaj-art-75b51f79ea344639805c1150cc63f0a3 |
| institution | Kabale University |
| issn | 2531-1379 |
| language | English |
| publishDate | 2024-11-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Hematology, Transfusion and Cell Therapy |
| spelling | doaj-art-75b51f79ea344639805c1150cc63f0a32024-12-01T05:07:43ZengElsevierHematology, Transfusion and Cell Therapy2531-13792024-11-0146S181S188Pediatric autoimmune hemolytic anemia: A single-center retrospective studyAline Sayuri Sakamoto0Fernanda Silva Sequeira1Bruna Paccola Blanco2Marlene Pereira Garanito3Corresponding author: University of Sao Paulo Children Institute, Av. Dr. Enéas Carvalho de Aguiar, 647 - Cerqueira César, São Paulo, SP, CEP: 05403-000, Brazil.; University of São Paulo Children Institute, São Paulo, BrazilUniversity of São Paulo Children Institute, São Paulo, BrazilUniversity of São Paulo Children Institute, São Paulo, BrazilUniversity of São Paulo Children Institute, São Paulo, BrazilBackground: Autoimmune hemolytic anemia (AIHA) is a rare, life-threatening disease in pediatrics. This article describes the clinical features, diagnostic workup, treatment and outcome in patients with AIHA. Method: Medical charts of under 18-year-old patients with AIHA treated at a tertiary Brazilian institution from 2006 to 2021 were retrospectively reviewed. Data analysis was primarily descriptive, using medians, interquartile ranges, and categorical variables presented as absolute frequencies. Main results: Twenty-four patients (14 female, 10 male) were evaluated in this study. The median age at diagnosis was 5.99 years (range: 0.25–17.1 years) and the median hemoglobin level was 4.85 g/dL (range: 4.17–5.57 g/dL). Most had warm antibodies (83.3 %). Twelve patients (50 %) had known underlining diseases, four (16.6 %) presented with AIHA concomitant with acute infectious diseases and three (12.5 %) had an undetermined post-vaccine association. Steroids and intravenous immunoglobulin were first-line therapy in 23 cases. Seven patients (29.1 %) required second and third-line treatments (rituximab, cyclophosphamide and splenectomy). The median follow-up period was 4.4 years (range: 1.0–6.7 years). Thirteen patients (54.1 %) were discharged, five cases (20.8 %) were lost to follow-up and no patient died. The median age for the six remaining patients was 11.53 years (8.5–14.7) with all of them having complete responses with no further therapies. Conclusion: Most cases of AIHA are secondary to an underlying systemic disease or have a possible correlation with infections/vaccines and respond to steroids. The second and third-line therapies for refractory and relapse cases remain a dilemma. A prospective, multicenter study is essential to address the best therapeutic combinations.http://www.sciencedirect.com/science/article/pii/S2531137924000166PediatricsAutoimmuneHemolytic anemia |
| spellingShingle | Aline Sayuri Sakamoto Fernanda Silva Sequeira Bruna Paccola Blanco Marlene Pereira Garanito Pediatric autoimmune hemolytic anemia: A single-center retrospective study Hematology, Transfusion and Cell Therapy Pediatrics Autoimmune Hemolytic anemia |
| title | Pediatric autoimmune hemolytic anemia: A single-center retrospective study |
| title_full | Pediatric autoimmune hemolytic anemia: A single-center retrospective study |
| title_fullStr | Pediatric autoimmune hemolytic anemia: A single-center retrospective study |
| title_full_unstemmed | Pediatric autoimmune hemolytic anemia: A single-center retrospective study |
| title_short | Pediatric autoimmune hemolytic anemia: A single-center retrospective study |
| title_sort | pediatric autoimmune hemolytic anemia a single center retrospective study |
| topic | Pediatrics Autoimmune Hemolytic anemia |
| url | http://www.sciencedirect.com/science/article/pii/S2531137924000166 |
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