Brigitta I. R. V. Corebima, Charity Monica, Eko Sulistijono, Susanto Nugroho, Sony Wicaksono, Setya Mithra Hartiastuti
Objective: Background: Case Report: Conclusions: Rare disease Hereditary spherocytosis (HS) is an autosomal dominant inherited disorder that causes severe hyperbilirubine-mia in neonates. There is no factual data about the prevalence in Indonesia. It is common that neonates with suspected hereditary spherocytosis are not diagnosed or treated adequately in developing countries such as Indonesia. A 6-day-old baby was referred from a secondary public hospital to our tertiary hospital in Malang, East Java with severe hyperbilirubinemia unresponsive to the 2 days of conventional phototherapy. Initial laboratory ex-amination showed total serum bilirubin level 28.83 mg/dL and indirect bilirubin level 25 mg/dL. Complete blood count showed hemoglobin level of 10.3 g/dL with high MCHC 36.9 g/dL and increased RDW 18.7%. The HS ratio (MCHC per MCV) was 0.41. The blood smear showed spherocytes with positive family history from the mother and grandmother. There were no specific tests such as EMA binding, cryohemolysis, or analysis of erythrocyte membrane protein available in our hospital. The patient was then treated with 2 sessions of intensive photo-therapy with phototherapy unit bilisphere 360 LED. The total serum bilirubin level dropped to 12.19 mg/dL. In this case, we decided to perform intensive phototherapy first, not only because of facility-based constraints to do timely exchange transfusion, but also due to the low socio-economic and educational background of the parents. There are some challenges in diagnosing and treating HS adequately in Indonesia. Limitations of specific tests, inadequacy of conventional phototherapy, lack of awareness of and adherence to guidelines, and facility-based inability to perform timely exchange transfusion all can contribute to severe hyperbilirubinemia and its sequelae. © Am J Case Rep.
Neonatology Division, Department of Pediatrics, Faculty of Medicine, Universitas Brawijaya – Dr. Saiful Anwar General Academic Hospital, Malang, Indonesia; Department of Pediatrics, Faculty of Medicine, Universitas Brawijaya – Dr. Saiful Anwar General Academic Hospital, Malang, Indonesia; Hematology and Oncology Division, Department of Pediatrics, Faculty of Medicine, Universitas Brawijaya – Dr. Saiful Anwar General Academic Hospital, Malang, Indonesia
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