Affiliation:
1. Faculty of Medicine University of Ottawa Ottawa Ontario Canada
2. Children's Hospital of Eastern Ontario Research Institute Ottawa Ontario Canada
3. Department of Pediatrics, Section of Pediatric Hematology Alberta Children's Hospital, University of Calgary Calgary Alberta Canada
4. Division of Hematology Oncology Children's Hospital of Eastern Ontario Ottawa Ontario Canada
Abstract
AbstractBackground and objectiveAutoimmune hemolytic anemia (AIHA) is a rare but important cause of morbidity in pediatric hematology patients. Given its rarity, there is little high‐quality evidence on which to base the investigation and management of pediatric AIHA. This scoping review aims to summarize the current evidence and highlight key gaps to inform future studies.MethodsThis review searched MEDLINE and the Cochrane CENTRAL Trials Register from 2000 to November 03, 2023. Experimental and observational studies reporting AIHA diagnostic criteria, laboratory workup, or treatment/management in populations with at least 20% of patients ≤18 years were included.ResultsForty‐three studies were included, with no randomized controlled trials identified. AIHA diagnostic criteria, diagnostic tests, and treatments were highly variable. First‐line treatment approaches include corticosteroids, intravenous immunoglobulin, or both. Approaches to AIHA resistance to first‐line therapy were widely variable between studies, but most commonly included rituximab and/or cyclosporine.ConclusionsWe identify a heterogenous group of observational studies into this complex, immune‐mediated disorder. Standardized definitions and classifications are needed to guide collaborative efforts needed to study this rare disease. The work done by the CEREVANCE group provides an important paradigm for future studies.