[Clinical Efficacy of ALL-2005 and ALL-2009 Regimen and Factors Influencing Prognosis of Newly Diagnosed ALL Patients Aged between 10-18 Years Old].

OBJECTIVE: To investigate the clinical efficacy of ALL-2005 and ALL-2009 regimen and factors influencing prognosis of newly diagnosed ALL patients aged between 10-18 years old to provide some reference for clinical diagnosis and treatment. METHODS: The clinical data including baseline clinical characteristics, induction chemotherapy effect, long-term clinical efficacy, recurrence rate and mortality of induction therapy of 119 newly diagnosed ALL patients aged between 10-18 years old from January 2008 to December 2015 were analyzed retrospectively, and the influencing factors of clinical prognosis were evaluated by univariate and multivariate analysis. RESULTS: The complete remission rate at the 5th week after induction therapy was not significantly different between ALL-2005 and ALL-2009 regimen groups (P＞0.05). The cumulative event-free survival rate and overall survival rate of 119 cases after 5-year follow-up were (63.41±3.65)% and (68.95±4.01)% respectively, and after 7-year follow-up were (61.86±3.72)% and (67.22±3.59)% respectively. The cumulative event-free survival rate and overall survival rate were not significantly different between ALL-2005 and ALL-2009 regimen groups (P＞0.05). The total recurrence rate, extramedullary recurrence rate, recurrence time and survival rate were not significantly different between ALL-2005 and ALL-2009 regimen groups (P＞0.05). The survival rate of extramedullary recurrence group was significantly higher than bone marrow recurrence group (P＜0.05). The survival rate in late term recurrence group was significantly higher than in early term recurrence group (P＜0.05). The mortality of ALL-2005 regimens was not significantly different from that of ALL-2009 regimen group (P＞0.05). Univariate analysis showed that age, sex, induction therapy, risk and fusion gene all were the factors influencing clinical prognosis (P＜0.05). Multivariate analysis by Cox regression model showed that male, non-remission after induction therapy and high risk were the independent risk factors for poor prognosis in patients (P＜0.05). The survival rate of patients with BCR-ABL+ treated with ALL-2009 regimen was significantly higher than that of patients treated with ALL-2005 regimen (P＜0.05). The event-free survival rate after 5-year follow-up of middle-risk patients treated with ALL-2005 and ALL-2009 regimens was significantly higher than that of high-risk patients (P＜0.05). The event-free survival rate after 5-year follow-up of patients with B-line ALL treated with ALL-2009 regimen was significantly higher than that of T-line ALL patients (P＜0.05). CONCLUSION: The survival rate of newly diagnosed ALL patients aged between 10-18 years old treated with ALL-2009 regimen was slightly higher than that of ALL-2005 regimen, it is more suitable for the ALL patients with BCR-ABL+, but can not reduce the recurrence rate. And the sex, the effect of induction therapy and risk closely relate with the clinical prognosis of above mentioned patients.

[Analysis of Clinical Characteristics and Prognostic Risk Factors of HLH Children with Central Nervous System Involvement].

OBJECTIVE: To investigate the clinical characteristics and prognostic risk factors of HLH children with central nervous system (CNS) involvement so as to provide more reference for further improving the prognosis of HLH children. METHODS: The clinical data of 45 HLH children with CNS involvement treated in our hospital from January 2006 to October 2016 were collected and analyzed retrospectively. The clinical characteristics of HLH children with CNS involvement were recorded, moreover the possible factors influencing the prognosis of HLH children with CNS involvement were analyzed using univariate and multivariate analysis through the establishment of Cox risk ratio model. RESULTS: Among 45 HLH children with CNS involvement, male was 19 cases and female was 26 cases. The median age of 4.0 years old (1.0-15.1). The detection showed that EBV found in 38 cases (84.44%), CMV infection in 1 case (2.22%), bacterial infection in 3 cases (6.67%), connection tissue disease in 1 case (2.22%) and indefinite etiology infection in 2 cases (4.44%). After lumbar puncture of 27 HLH children with CNS involvement, 10 cases (37.04%) showed cerebrospinal fluid abnormality. In addition, 22 cases showed the craniography abnormality. The follow-up results showed that the OS rate of 1 year was 46.67% (21/45), the OS rate of 3 years was 44.44% (20/45); the median survival time was 5.0 months. The OS analysis indicated that 1 years OS rate of diseased children with cerebrospinal fluid abnormality was significantly lower than that of diseased children with cerebrospinal fluid normality (10/45 vs 17/45) (P＜0.05), and 1 years OS rate of diseased children who not received intrathecal injection was significantly lower that of diseased children who received intrathecal (10/45 vs 17/45) (P＜0.05). The univariate analysis showed that the symptoms of nervous system, abnormal cerebrospinal fluid, absence of intrathecal injection and treatment schedule all were the risk factors affecting the prognosis of HLH children with CNS involvement (P＜0.05). The multivariate analysis by Cox risk model showed that abnormal cerebrospinal fluid and absence of intrathecal injection were independent risk factors for of HLH children with CNS involvement (P＜0.05). CONCLUSION: The clinical prognosis of HLH children with CNS involvement is relatively poor, moreover some of HLH children with CNS involvement have neural sequelae. The cerebrospinal fluid abnormality and absence of intrathecal injection are independent risk factors leading to poor prognosis for HLH clildren with CNS involvement.

[Related Factors Affecting Long-term Prognosis of AML Children with Positive RUNX1-RUNX1T1].

OBJECTIVE: To analyze the related factors affecting the long-term prognosis of acute myeloid leukemia (AML) children with positive RUNX1-RUNX1T1. METHODS: The clinical data of 63 chlidren with positive RUNX1-RUNX1T1 AML treated by BCH-AML 05 regimen in our hospital from January 2010 to December 2015 were collected and analyzed retrospectively. The level of RUNX1-RUNX1T1 was detected at the time of initial diagnosis (T1), after the first induction treatment (T2), after the second induction treatment (T3), after the first consolidation treatment (T4), after the second consolidation treatment (T5) and after the third consolidation treatment (T6). According to the fusion transcript levels of RUNX1-RUNX1T1 the AML children were divided into low-expression group and high-expression group; the threshold values for grouping were 104 copies/104 ß-glucuronidase (GUS), 103 copies/104 GUS, 102 copies/104 GUS, 10 copies/104 GUS, 1 copies/104 GUS and 0 copies respectively. The gained data were enrolled in the statistical analysis. RESULTS: 23 cases of 63 children died during the follow-up period, and the median follow-up time of the remaining 40 children were 30.04 (11-60) months. There were statistically significant differences in CD15 positive rate between low-expression group and high-expression group (P<0.05), however, there were no statistically significant differences in sex, age, FAB typing, platelet (Plt) count, hemoglobin (Hb) and white blood cell (WBC) count and the ratio of bone marrow immature cells at T2 between the two groups (P>0.05). Univariate analysis showed that sex, Plt counts at T1 and fusion transcript levels at T1, T2 and T6 correlated with the 5-year overall survival rate (P<0.05). Multivariate analysis showed that fusion transcript level >103 copies/104 GUS at T2 was an independent risk factor for 5-year overall survival rate (HR=2.13, 95%CI: 1.04-7.78)(P<0.05). CONCLUSION: The fusion transcript level after the first induction therapy in RUNX1-RUNX1T1-positive AML children is an independent factor influencing the long-term prognosis.

[Muscles reposition in correction of nasal deformity after unilateral cleft lip].

OBJECTIVE: To investigate the effective method for correction of nasal deformity after unilateral cleft lip. METHODS: 50 cases with secondary nasal deformity after unilateral cleft lip were retrospectively analyzed. All the patients underwent nasal and lip muscle reposition operation to restore the symmetry of nasal alar. RESULTS: The nasal deformity was greatly improved in all the 50 cases. The malposition of nasal column and nasal alar was corrected. The symmetry was markedly improved. The measurement before and after operation showed significant difference ( P<0.05). CONCLUSIONS: The reasons of secondary nasal deformity after unilateral cleft lip are complicated. The muscle reposition operation can effectively improve the deformity.