Severe Acute Anemia in Pediatric Sickle Cell Disease: Transfusion Indications and Short-term Outcomes by Crisis Etiology—A Systematic Review

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Severe acute anemia is a high-risk complication of pediatric sickle cell disease (SCD) that often prompts emergency evaluation and red blood cell transfusion, yet evidence guiding transfusion decisions for different acute anemia etiologies in pediatric SCD remains limited. We conducted a Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020-guided systematic review of PubMed, Europe PMC, Semantic Scholar, OpenAlex, and Google Scholar (January 2000–January 2026), limiting Google Scholar screening to the top 1000 results. Two reviewers screened studies, extracted data, and assessed risk of bias (Joanna Briggs Institute for case reports/series and Newcastle–Ottawa Scale for observational studies); certainty was evaluated using GRADE. Of 13,355 records, 9 studies were included (4 case series, 3 case reports, and 2 observational), reporting aplastic crisis ( n = 4), hemolytic-related crises ( n = 3), and splenic sequestration ( n = 2). Transfusion decisions were mainly driven by clinical severity rather than standardized thresholds. Outcomes were generally favorable in aplastic crisis but more variable in hemolytic-related crises. Evidence certainty was very low; prospective multicenter studies are needed.