Background: The diagnosis of autoimmune hemolytic anemia (AIHA) can be challenging since the direct antiglobulin test (DAT) has been reported to be falsely negative in 3%-11% of cases. In children with anemia, laboratory and/or clinical evidence of hemolysis and a negative DAT, clinicians should consider further specialized testing to confirm AIHA to accurately diagnose and treat this uncommon pediatric entity.
Study design and methods: A retrospective chart review was undertaken at a large tertiary care academic pediatric hematology practice to describe our experience with DAT-negative AIHA.
Results: From January 1, 2010 through August 1, 2016, 10 children were described who had clinical and laboratory evidence of AIHA, a negative DAT, and further specialized serologic testing confirming this diagnosis.
Conclusion: This case series highlights the need for further serologic workup when a child's clinical presentation is highly consistent with AIHA despite a negative DAT.
© 2019 AABB.