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Autoimmune Hemolytic Anemia in Children: Clinical Profile and Outcome
Shrutiprajna Kar1, C G Delhi Kumar2, Rakhee Kar3
1Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, 605006, India.
Insights
In children, autoimmune hemolytic anemia (AIHA) is often secondary to conditions like lupus, with poorer outcomes than primary AIHA. Treatment involves glucocorticoids and mycophenolate mofetil.
Area of Science:
- Pediatric Hematology
- Immunology
- Autoimmune Diseases
Background:
- Autoimmune hemolytic anemia (AIHA) is a condition where the immune system attacks red blood cells.
- Identifying triggers and understanding outcomes in pediatric AIHA is crucial for effective management.
Purpose of the Study:
- To determine common triggers of AIHA in children.
- To describe the clinical presentation, treatment response, and outcomes of pediatric AIHA.
- To compare prognosis between primary and secondary AIHA in children.
Main Methods:
- An ambispective descriptive study was conducted from 2013 to 2020.
- Included children aged 1 month to 14 years with hemolytic anemia and a positive direct antiglobulin test (DAT).
- Collected data on clinical profile, treatment, and disease outcome from a structured pro forma.
Main Results:
- 46 children were enrolled; 63% had secondary AIHA, with Systemic Lupus Erythematosus (SLE) as the most frequent trigger (45%).
- Pallor, hepatomegaly, and splenomegaly were common clinical signs. Mixed immunophenotype was most frequent (59%).
- Complete remission was achieved in 71% of primary AIHA cases versus 14% of secondary AIHA. Overall mortality was 15%, exclusively in secondary AIHA cases.
Conclusions:
- Secondary AIHA is more prevalent than primary AIHA in children, with SLE being a significant trigger.
- Primary AIHA demonstrates a better prognosis compared to secondary AIHA.
- Effective treatment strategies are essential, particularly for secondary AIHA with poorer outcomes.
Objective:
To discover the common triggers for AIHA in children, their clinical profile, treatment response, and outcome.
Methods:
This was an ambispective descriptive study conducted between 2013 and 2020. Children aged 1 mo to 14 y with hemolytic anemia and a positive direct antiglobulin test (DAT) were included. Children with a positive DAT but without any clinicolaboratory evidence of hemolysis were excluded. Data were collected from a structured pro forma with particulars comprising clinicolaboratory profile, treatment administered, and disease outcome.
Results:
A total of 46 children (aged between 1 mo and 14 y) were enrolled in the study. The mean age of onset was 8.7 (± 4.34) y, and 24 (52.8%) were males. Secondary causes were observed in 29 (63%) cases, while the primary cause was found in 17 (37%). Systemic lupus erythematosus (SLE) was the common trigger in 13 (45%) cases, followed by malignancy in 4 (14%) cases. Pallor (98%), hepatomegaly (72%), and splenomegaly (48%) were the most commonly observed clinical signs. The mixed immunophenotype was observed in 27 (59%) cases, followed by warm type in 12 (26%) and cold agglutinin type in 7 (15%) cases. All children received glucocorticoid therapy, and mycophenolate mofetil was commonly used as second-line therapy in 15 (33%) cases. 13 cases (71%) of primary AIHA and only 4 (14%) cases of secondary anemia achieved complete remission. Overall, 7 children (15%) died, all belonging to secondary AIHA.
Conclusion:
Secondary AIHA was more common than primary in the present study, and SLE was the standard trigger. Primary AIHA carries a better prognosis than secondary.
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