Higher plasma bilirubin predicts veno-occlusive disease in early childhood undergoing hematopoietic stem cell transplantation with cyclosporine

AIM: To analyze the association between plasma bilirubin levels and veno-occlusive disease(VOD) in non-adult patients undergoing hematopoietic stem cell transplantation(HSCT) during cyclosporine therapy.METHODS: A total of 123 patients taking cyclosporinewere evaluated using an electronic medical system at the Seoul National University Children's Hospital from the years 2004 through 2011. Patients were grouped by age and analyzed for incidence and type of adverse drug reactions(ADRs) including VOD. RESULTS: The HSCT patients were divided into three age groups: G#1 ≥ 18; 9 ≤ G#2 ≤ 17; and G#3 ≤ 8 years of age). The majority of transplant donor types were cord blood transplantations. Most prevalent ADRs represented acute graft-vs-host disease(a GVHD) and VOD. Although the incidences of a GVHD did not vary among the groups, the higher frequency ratios of VOD in G#3 suggested that an age of 8 or younger is a risk factor for developing VOD in HSCT patients. After cyclosporine therapy, the trough plasma concentrations of cyclosporine were lower in G#3 than in G#1, indicative of its increased clearance. Moreover, in G#3 only, a maximal total bilirubin level(BILmax) of ≥ 1.4 mg/d L correlated with VOD incidence after cyclosporine therapy. CONCLUSION: HSCT patients 8 years of age or younger are more at risk for developing VOD, diagnosed as hyperbilirubinemia, tender hepatomegaly, and ascites/weight gain after cyclosporine therapy, which may be represented by a criterion of plasma BILmax being ≥ 1.4 mg/d L, suggestive of more sensitive VOD indication in this age group.

Treatment of pediatric average-risk medulloblastoma using craniospinal irradiation less than 2500 cGy and chemotherapy: single center experience in Korea

Background:Although craniospinal irradiation (CSI) of 2340 cGy plus tumor booster with chemotherapy have been established as a standard treatment of childhood average-risk (AvR) medulloblastoma (MBL) in Western counties,there are a few recent reports in outcomes ofAvR MBL using this strategy in Korean and other Asian children.We investigated the outcome of the Korean children with AvR MBL who were treated with CSI <2500 cGy and chemotherapy.Methods:Between January 2001 and December 2010,clinical characteristics and outcomes of 42 patients who were diagnosed with AvR MBL postoperatively and treated with radiation including CSI <2500 cGy and chemotherapy in Seoul National University Children's Hospital were analyzed.Results:Their median age was 9 years (range:3-18.8),and 29 were male.Histological subtypes were classic type in 28 patients,nodular/desmoplastic in 7,and large cell/anaplastic (LCA) in 7.All the patients received adjuvant radiotherapy (CSI with median 2340 cGy and booster) and multiagent chemotherapy as the first-fine treatment.With a median follow-up of 54 months,12 patients experienced relapse or progression of the tumor.The 3-and 5-year disease-free survival (DFS) rates were 78.0%±6.5% and 75.0%±6.9%,respectively,and overall survival (OS) rates were 85.3%±5.6% and 76.8%±6.9%,respectively.The LCA subtype was associated with poorer DFS (P=0.023) and OS (P=0.008),compared with non-LCA subtypes.Conclusion:The outcomes of children and adolescents with AvR MBL treated with radiation including CSI <2500 cGy and chemotherapy,are compatible to those in Western countries;however,the LCA subtype has a poor outcome with this strategy.