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The North American Pediatric Aplastic Anemia Consortium (NAPAAC) is a group of pediatric hematologist‐oncologists, hematopathologists, and bone marrow transplant physicians from 46 institutions in North America with interest and expertise in aplastic anemia, inherited bone marrow failure syndromes, and myelodysplastic syndromes. The NAPAAC Bone Marrow Failure Diagnosis and Care Guidelines Working Group was established with the charge of harmonizing the approach to the diagnostic workup of aplastic anemia in an effort to standardize best practices in the field. This document outlines the rationale for initial evaluations in pediatric patients presenting with signs and symptoms concerning for severe aplastic anemia.

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.

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