Key Findings
- Purpose
To present a clinical case illustrating the diagnostic and therapeutic challenges when multiple severe hemolytic complications of SCD converge in a single pediatric patient. - Population
A 5-year-old female with SCD (Hb SS), inconsistent hydroxyurea use, and baseline splenomegaly who experienced severe anemia and overlapping immune and sequestration phenomena following transfusion. - Headline Result
- Following an antigen-unmatched red blood cell transfusion for orbital bone infarct, the patient developed alloimmunization (anti-D, anti-C, anti-E, and nonspecific IgG) and evidence of warm autoimmune hemolytic anemia and hyperhemolysis syndrome.
- Laboratory evaluation showed profound anemia and elevated markers of hemolysis; multidisciplinary therapy with intravenous immunoglobulin (IVIG), high-dose methylprednisolone, rituximab, and subsequent phenotypically matched transfusions stabilized the patient.
- Splenectomy was performed after stabilization, and the patient was evaluated for stem cell transplant as definitive therapy.
- Why It Matters
Immune-mediated hemolytic processes (alloimmunization and wAIHA) can complicate standard transfusion strategies in SCD, especially in patients with prior mismatched antigens, poor disease control, or splenic involvement. Early recognition and coordinated immunomodulatory care, phenotype-matched blood, and surgical planning can be critical in managing life-threatening anemia and preventing further hemolysis. - Evidence Gaps & Limitations
As a single case report, the observations cannot quantify prevalence or predict outcomes broadly. The case highlights mechanisms and management insights but does not compare alternative strategies or long-term outcomes beyond discharge and transplant planning.
Source: Journal of Sickle Cell Disease — “Challenging case of hemolytic anemia and splenic sequestration in sickle cell disease.”
Regulatory & Guideline Watch
Current SCD guidelines underscore the importance of antigen matching and strategies to minimize alloimmunization, particularly in children with recurrent transfusions. They also address management of severe hemolytic complications, including hyperhemolysis and autoimmune hemolytic anemia, yet consensus recommendations on stepwise immunomodulatory therapy (IVIG, steroids, rituximab) remain evolving and often rely on expert guidance due to limited robust evidence.