Hereditary Spherocytosis in Children: Experiences in a Single Center

Park, Eun Ah; Kim, Mi Kyoung; Park, Ji Kyoung; Lee, Soon Yong

Abstract

PURPOSE
The aim of this study was to evaluate the clinical and laboratory findings of hereditary spherocytosis (HS) by comparing patients of different age groups and profiling the outcomes of patients undergoing splenectomy.
METHODS
The clinical and laboratory findings of 31 patients with HS cared for at Busan Paik Hospital between November 1991 and August 2007 were analyzed retrospectively. The patients were categorized as follows: group I, underwent splenectomy (n=24) group Ia, diagnosed at <10 years of age (n=18) group Ib, diagnosed at > 10 years of age (n=6) and group II, a splenectomy was not performed (n=7).
RESULTS
The mean age at the time of diagnosis was as follows: all patients (n=31), 5 years 8 months group I, 6 years 2 months and group II, 1 year 11 months. The family history was positive for HS in 37.5% and 71.4% of group I and II patients. Neonatal hyperbilirubinemia was present in 62.5% and 71.4% of group I and II patients. Splenectomies were performed in 77.4% of all HS patients. Gallstones were found in 33.3% and 0% of group I and II patients and aplastic crisis developed in 8.3% and 26.7% of group I and II patients. The median age of patients with gallstone was 8 years 5 months. The laboratory findings after splenectomy improved as follows: hemoglobin concentration, 8.52+/-2.19 g/dL (pre-splenectomy) to 13.42+/-1.01 g/dL (post-splenectomy); corrected reticulocyte count, 6.86+/-3.54% (pre-splenectomy) to 1.29+/-0.40% (post-splenectomy); total bilirubin, 5.63+/-5.26 mg/dL (pre-splenectomy) to 0.97+/-0.27 mg/dL (post-splenectomy).
CONCLUSION
There were no differences between groups I and II with respect to family history, incidence of neonatal hyperbilirubinemia, severity, and complications according to age or splenectomy (P>0.05). The median age at the time of diagnosis was lower in group II than group I (P<0.05). Gallstone was found at median age younger than 10 years in HS patients. The laboratory parameters improved more in patients who underwent splenectomy than patients who did not.