RESUMO
Clinical trials have demonstrated that pediatric acute promyelocytic leukemia (APL) is highly curable. Small-scale studies have reported on the treatment of APL using one or two treatment regimes. Here, we report a multiple center-based study of 119 cases of pediatric APL treated with four regimes based on all-trans-retinoic acid (ATRA). We retrospectively analyzed the clinical characteristics, laboratorial test results and treatment outcome of the pediatric APL patients. Regime 1 used an in-house developed protocol, regime 2 was modified from the PETHEMA LPA99 protocol, regime 3 was modified from the European-APL93 protocol, and regime 4 used a protocol suggested by the British Committee for Standards in Haematology. The overall complete remission rates for the four regimes were 88.9, 87.5, 97.1 and 87.5%, respectively, which exhibited no statistical difference. However, more favorable results were observed for regimes 2 and 3 than regimes 1 and 4, in terms of the estimated 3.5-year disease-free survivals, relapse rates, drug toxicity (including hepatotoxicity, cardiac arrhythmia, and differentiation syndrome) and sepsis. In conclusion, the overall outcomes were more favorable after treatment with regimes 2 and 3 than with regimes 1 and 4, and this may have been due to the specific compositions of regimes 2 and 3.
RESUMO
The aim of study was to investigate the relationship between polymorphisms of FCGR2B232 1/T oligonucleotide and the susceptibility of children with idiopathic thrombocytopenic purpura (ITP). DNA from 76 patients with ITP and 37 controls was extracted. The SNPs of FCGR2B-232 was detected by polymerase chain reaction (PCR) combined with direct sequencing. The genotype distribution and allele frequency among different groups were compared. The results showed that the genotype (I/I, I/T, T/T) of FCGR2B-232 were 55.3%, 42.1%, and 2.6% in 76 patients with ITP, while 81.1%, 18.9%, 0% in 37 controls. The allele frequencies of FCGR2B-232 in patients with ITP were 76.3% (I232) and 23.7% (T232), but 90.5% and 9.5% in controls. There were significant differences in genotype distributions between the ITP patients and controls (chi(2) = 7.45, = 0.024). The enrichment in Thr232 allele carrier was also significant among the ITP patients as compared with the controls (chi(2) = 7.18, p = 0.007, odds ratio 3.47). There were also significant differences in allele frequencies between the ITP patients and controls [chi(2) = 6.54, p = 0.011, odds ratio 2.97, 95% CI (1.25 - 7.05)]. It is concluded that the polymorphisms of FCGR2B-232 significantly correlates with the susceptibility of children suffering from ITP. The minor Thr232 allele may be a risk genetic factor to ITP children.