Clinical Manifestations and Factors Associated with Complications and Splenectomy in Hereditary Spherocytosis

Park, Sang Jun; Yang, Woo Shik; Hwang, Hui Seung; Jang, Pil Sang; Kim, Hyun Hee; Kim, Jong Hyun; Koh, Dae Kyun; Lee, Kyung Yil; Cho, Bin; Jeong, Dae Chul; Kim, Hack Ki

Abstract

PURPOSE: Hereditary spherocytosis (HS) is the most common non-immune hemolytic anemia in children. Children with HS often have positive family history as it is inherited by autosomal dominant pattern. Clinical manifestations depends on the severity of hemolysis, and children with HS sometimes suffer from complications, including aplastic crisis or gall stones. We evaluated the clinical manifestations and the risk factors associated with complications or splenectomy in children with HS.
METHODS
We retrospectively investigated the clinical data in children with HS between 1994 and 2004. We analyzed the risk factors for complications and splenectomy according to age at diagnosis and severity from initial laboratory data.
RESULTS
Forty four children (male: 27, female: 17) were enrolled in this study. Median age at diagnosis was 3.3 years old, and 36 children with HS (81.8%) were diagnosed within 9 years of age. Family history in 15 children, and patients diagnosed within 6 months of age had higher than other age group for family history (P<0.05). Symptoms associated with hemolysis were dominant in children diagnosed before 9 years of age. There were no differences in the initial laboratory data and severity according to age at diagnosis. Complications found in 12 patients with 18 events, including aplastic crisis, gall stone, and others. Six out of seven children with aplastic crisis were associated with parvovirus infection. The factors with complications were associated with low MCV, corrected reticulocytes, direct bilirubin, and high RDW (P<0.05). Twenty patients received splenectomy, and had risk factors with high total bilirubin, low direct bilirubin, and presence of complications (P<0.05). Severity correlated with an increase in the incidence of aplastic crisis and not the incidence of complications or splenectomy.
CONCLUSION
We concluded that HS with severe hemolysis might develop complications, and have higher risk of splenectomy. We propose for close follow up in children with severe HS, and need investigation between clinical findings and characteristics of molecular study for children with HS in Korea.