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Exchange Transfusion via Peripheral Access for Neonate With Hyperbilirubinemia and Hereditary Spherocytosis in the
Chandler Williams1, Valentyna Tack, Sudeepta Basu
1Author Affiliations: Children's National Health System, NICU, Washington, DC (Drs Williams, Basu, and Rosenstein); Advocare Lovenheim Pediatrics, West Orange, NJ (Dr Tack); and The George Washington University, Washington, DC (Dr Basu).
Background:
Severe neonatal unconjugated hyperbilirubinemia is associated with the risk of neurotoxicity and hence warrants prompt diagnostic and therapeutic interventions, including phototherapy and exchange transfusions. Although relatively rare, Hereditary spherocytosis (HS) is one of the common non-alloimmune hemolytic disorders associated with severe neonatal hyperbilirubinemia and hemolytic anemia during childhood. HS results from a mutation in genes encoding 1 or more proteins in the erythrocyte membrane, which leads to a loss of membrane structure and dysfunction of the cell. The clinical spectrum of HS varies widely, leading to under-diagnosis during the neonatal period, and management differs from alloimmune hemolytic disorders.
Clinical Findings:
In this case report of a neonate with severe unconjugated hyperbilirubinemia due to HS.
Primary Diagnosis:
Severe unconjugated hyperbilirubinemia due to HS.
Interventions:
Phototherapy and exchange transfusion.
Outcomes:
Subsequent decrease in bilirubin levels and safe discharge of neonate with instructions to follow up with pediatrician, hematology, and genetics with outpatient appointments.
Practice Recommendations:
In this case report, we emphasize the need for a high index of suspicion among neonatal caregivers and discuss management options. We also discuss the lack of response to intravenous immunoglobulin as a diagnostic clue and the feasibility of performing exchange transfusion using peripheral intravenous access.
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