Clinical features and prognosis of childhood B-lineage acute lymphoblastic leukemia expressing the PRAME gene / 中国当代儿科杂志

OBJECTIVES@#To study the clinical and prognostic significance of the preferentially expressed antigen of melanoma (PRAME) gene in the absence of specific fusion gene expression in children with B-lineage acute lymphoblastic leukemia (B-ALL).@*METHODS@#A total of 167 children newly diagnosed with B-ALL were enrolled, among whom 70 were positive for the PRAME gene and 97 were negative. None of the children were positive for MLL-r, BCR/ABL, E2A/PBX1, or ETV6/RUNX1. The PRAME positive and negative groups were analyzed in terms of clinical features, prognosis, and related prognostic factors.@*RESULTS@#Compared with the PRAME negative group, the PRAME positive group had a significantly higher proportion of children with the liver extending >6 cm below the costal margin (P<0.05). There was a significant reduction in the PRAME copy number after induction chemotherapy (P<0.05). In the minimal residual disease (MRD) positive group after induction chemotherapy, the PRAME copy number was not correlated with the MRD level (P>0.05). In the MRD negative group, there was also no correlation between them (P>0.05). The PRAME positive group had a significantly higher 4-year event-free survival rate than the PRAME negative group (87.5%±4.6% vs 73.5%±4.6%, P<0.05), while there was no significant difference between the two groups in the 4-year overall survival rate (88.0%±4.4% vs 85.3%±3.8%, P>0.05). The Cox proportional-hazards regression model analysis showed that positive PRAME expression was a protective factor for event-free survival rate in children with B-ALL (P<0.05).@*CONCLUSIONS@#Although the PRAME gene cannot be monitored as MRD, overexpression of PRAME suggests a good prognosis in B-ALL.

The Impact of Induction Treatment Response on the Prognosis of Pediatric Core Binding Factor-Acute Myeloid Leukemia Patients / 中国实验血液学杂志

OBJECTIVE@#To explore the impact of induction treatment response on the prognosis of pediatric core binding factor-acute myeloid leukemia (CBF-AML).@*METHODS@#The result of induce reaction and survival data of 157 pediatric CBF-AML patients in our hospital from September 2008 to December 2018 were retrospectively analyzed．The survival rate of the patients with different degrees of morphological remission after induction chemotherapy was comparative analyzed.@*RESULTS@#Among the 157 children with CBF-AML, 113 (72.4%) patients achieved morphologic leukemia-free state (MLFS) after the first course of induction chemotherapy, 153 (98.1%) patients achieved MLFS after the second course of induction chemotherapy. The 5-year event-free survival (EFS) rate and 5-year overall survival (OS) rate of patients with non-remission (NR) status after the first course of induction of chemotherapy was significantly lower than the patients achieved MLFS and the patients achieved partial remission (PR). The 5-year EFS rate and 5-year OS rate of the patients with PR status after the second course of induction chemotherapy were lower than the patients achieved MLFS, but the difference was not statistically significant. Multivariable analyze showed that NR after the first course of induction chemotherapy and myeloid sarcoma were the independent risk factors affecting EFS of the patients. There were six patients with NR status after the first course of induction chemotherapy, in which all of them harbored t(8;21), three of them with sex chromosome deletion, two of them with myeloid sarcoma.@*CONCLUSION@#NR status after the first course of induction chemotherapy was the independent risk factor affecting EFS and OS of CBF-AML patients, it can be taken as an indicator for higher risk stratification. PR status after the first course of induction chemotherapy may not be used as a diagnostic criterion for primary drug resistance.

Clinical characteristics and prognostic analysis of pediatric pro-B cell acute lymphoblastic leukemia / 中国当代儿科杂志

OBJECTIVE@#To explore the clinical-biological characteristics and prognosis of pediatric pro-B cell acute lymphoblastic leukemia (pro-B-ALL).@*METHODS@#A total of 64 patients aged less than 18 years old with pro-BALL were enrolled. Clinical characteristics, therapeutic effect and prognostic factors were retrospectively analyzed.@*RESULTS@#Pro-B-ALL occurred in 6.23% (64/1 028) of pediatric ALL. Among the 64 patients, 35 were male and 29 were female. The median age was 7.0 years (range 0.4-16.0 years) at diagnosis, of which 39% and 6% were ≥ 10 years old and < 1 year old respectively. The median WBC count was 25.5×10@*CONCLUSIONS@#Pediatric pro-B ALL is a heterogeneous disease with clinical and biological diversity. Biological characteristics, such as immunological markers, genetic alterations, and MRD at 3 months after chemotherapy may be important factors for the long-term prognosis.

Influence of dasatinib treatment on body height in children with acute myeloid leukemia / 中国当代儿科杂志

OBJECTIVE@#To study the influence of dasatinib treatment on body height in children with acute myeloid leukemia (AML).@*METHODS@#A retrospective analysis was performed for the clinical data of 86 AML children aged <17 years. According to the treatment regimen, these children were divided into a conventional chemotherapy group and a dasatinib chemotherapy group. The 57 children in the conventional chemotherapy group were given conventional chemotherapy drugs without tyrosine kinase inhibitor, and the 29 children in the dasatinib chemotherapy group were given conventional chemotherapy drugs and dasatinib. The two groups were compared in terms of height standard deviation score (HtSDS) at the beginning of treatment and after treatment, as well as the change in HtSDS after 1 and 2 years of treatment.@*RESULTS@#There was no significant difference in HtSDS between the conventional and dasatinib chemotherapy groups before treatment. Within the first two years of treatment, the dasatinib chemotherapy group had a similar change trend of HtSDS as the conventional chemotherapy group. Four children in the dasatinib chemotherapy group reached the final adult height during follow-up, which was significantly lower than the target height (P=0.044). In the conventional chemotherapy group, there was no significant difference between final adult height and target height. In the dasatinib chemotherapy group, the children in adolescence had a significant change in HtSDS after treatment (P=0.032).@*CONCLUSIONS@#Dasatinib treatment may affect the final height of children with AML, and the use of dasatinib after the beginning of adolescence may lead to growth disorder, but dasatinib treatment has little effect on body height in the short-term treatment.

Clinical characteristics and prognostic analysis of children and adolescents over 10 years of age with acute lymphoblastic leukemia / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To explore the clinical characteristics and prognosis of children and adolescents over 10 years of age with acute lymphoblastic leukemia (ALL).</p><p><b>METHODS</b>A total of 86 newly diagnosed ALL children and adolescents over 10 years of age (62 cases of B-ALL and 24 cases of T-ALL) were enrolled. Clinical characteristics, therapeutic effect and prognostic factors were retrospectively analyzed. Event-free survival (EFS) and overall survival (OS) rates were estimated by the Kaplan-Meier method. Prognostic factors were evaluated by COX regression analysis.</p><p><b>RESULTS</b>Of 86 patients, 62 were in medium risk, and 24 in high risk. At diagnosis, 53 patients (62%) had hepatomegaly, 50 patients (58%) had splenomegaly, and 46 patients (54%) had lymphoadenopathy. Twenty-nine patients (34%) showed high leukocyte counts (≥50×10/L) at diagnosis. The karyotype analysis was performed on 78 patients. The percentage of hyperdiploidy was 19% (15 cases), and that of hypodiploidy was 5% (4 cases). Eleven patients (14%) had abnormalities of chromosome structure. Of them, one patient was Philadelphia chromosome-positive, and another patient had the t (1; 19) chromosomal translocation. Three patients (4%) were positive for TEL/AML1, 3 (4%) were positive for E2A/PBX1, 6 were positive for BCR/ABL (7%), and 4 (5%) were positive for SIL/TAL1. During 4 weeks of induction therapy, 85 patients (99%) achieved complete remission (CR). In 86 patients, the 5-year anticipated EFS and OS were (64±6)% and (75±5)% respectively. The 5-year EFS and OS in the medium risk group were significantly higher than those in the high risk group (P<0.05). The 5-year EFS in B-ALL patients was significantly higher than that in T-ALL patients (P<0.05). COX multivariate analysis showed that white blood counts at diagnosis and minimal residual disease (MRD) after induction therapy were independent prognostic factors.</p><p><b>CONCLUSIONS</b>Children and adolescents with ALL over 10 years of age often have clinical characteristics of unfavorable prognosis. White blood counts at diagnosis and MRD after induction therapy may be important factors for the long-term prognosis.</p>

Night Sleep Duration and Risk of Cognitive Impairment in a Chinese Population: A Cross-sectional Study / 生物医学与环境科学（英文）

<p><b>OBJECTIVE</b>Although sleep is one of the most important health-related behavioral factors, the association between night sleep duration and cognitive impairment has not been fully understood. A cross-sectional study was conducted with a random sample of 2,514 participants (⋝ 40 years of age; 46.6% women) in China to examine the association between night sleep duration and cognitive impairment.</p><p><b>METHODS</b>Night sleep duration was categorized as ⋜ 5, 6, 7, 8, or ⋝ 9 h per night. Cognitive function was measured using the Mini-Mental State Examination. A multivariate regression analysis was used to analyze the association of night sleep duration with cognitive impairment. A total of 122 participants were diagnosed with cognitive impairment.</p><p><b>RESULTS</b>A U-shaped association between night sleep duration and cognitive impairment was found. The odds ratios (95% confidence intervals) of cognitive impairment (with 7 h of daily sleep being considered as the reference) for individuals reporting ⋜ 5, 6, 8, and ⋝ 9 h were 2.14 (1.20-3.83), 1.13 (0.67-1.89), 1.51 (0.82-2.79), and 5.37 (1.62-17.80), respectively (P ⋜ 0.01).</p><p><b>CONCLUSION</b>Short or long night sleep duration was an important sleep-related factor independently associated with cognitive impairment and may be a useful marker for increased risk of cognitive impairment..</p>

Prognostic impact of loss of sex chromosomes in children with acute myeloid leukemia subtype M2 / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the relationship between loss of sex chromosomes and prognosis in children with acute myeloid leukemia (AML) M2 subtype.</p><p><b>METHODS</b>According to cytogenetic characteristics, 106 children with AML were divided into three groups: patients with normal karyotype (Group A, n=26), patients with abnormal karyotype who had no loss of sex chromosomes (Group B, n=52), and patients with abnormal karyotype who had loss of sex chromosomes (Group C, n=28). Prognosis was compared between the three groups.</p><p><b>RESULTS</b>The 5-year event-free survival (EFS) rates of Groups A, B, and C were (38.9±11.2)%, (59.3±7.3)%, and (66.5±10.5)%, respectively; the EFS of Group C was significantly higher than that of Group A (P=0.035). The 5-year overall survival (OS) rates of Groups A, B, and C were (54.3±13.5)%, (68.1±7.7)%, and (77.9±9.8)%, respectively (P>0.05). The 5-year EFS of 58 patients with t(8;21) was (63.3±7.3)%, significantly higher than that of patients with normal karyotype (P=0.015). All the 28 cases in Group C had t(8;21), and their 5-year EFS was not significantly different from that of patients with t(8;21) in Group B (P>0.05).</p><p><b>CONCLUSIONS</b>Loss of sex chromosomes is a favorable karyotype in children with AML M2 subtype and the patients in this group mostly have t(8;21). Why loss of sex chromosomes indicates a favorable prognosis is probably because it is accompanied by t(8;21) in the patients.</p>

A comparison of minimal residual disease in children with acute lymphoblastic leukemia of different genetic abnormalities / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the changes of minimal residual disease (MRD) in children with B cell acute lymphoblastic leukemia (B-ALL) of different genetic abnormalities.</p><p><b>METHODS</b>Between February 2004 and April 2013, 271 newly diagnosed B-ALL pediatric patients who had finished the induction chemotherapy were enrolled in the study. The characteristics of changes in MRD in patients with different genetic abnormalities on the 15th day and at the end of the induction therapy were analyzed.</p><p><b>RESULTS</b>On the 15th day of the induction chemotherapy, the MRD positive proportion in patients with hyperdiploid was higher on all the three cut-off levels of MRD≥0.1%, 1% and 10% compared to patients without hyperdiploid (P<0.05), but there was no significant difference in the MRD positive proportion on the three levels of MRD between the TEL-AML1-positive and TEL-AML1-negative groups (P>0.05). On the end of induction chemotherapy, there was no significant difference in the MRD positive proportion on the three levels of MRD between the patients with and without hyperdiploid (P>0.05), neither between the BCR-ABL-positive and negative groups. The MRD positive proportion in TEL-AML1-negative patients was significantly higher than in TEL-AML1-positive patients on all three levels of MRD (P<0.05). The MRD positive proportion on two levels of MRD≥0.01% and 0.1% in E2A-PBX1-negative patients was significantly higher than in E2A-PBX1-positive patients (P<0.05).</p><p><b>CONCLUSIONS</b>Children with B-ALL of different genetic abnormalities have different MRD levels during, and at the end of, induction therapy. The prognostic significance of MRD may be related to the genetic abnormalities.</p>

Therapeutic effect of clofarabine in children with relapsed or refractory acute lymphoblastic leukemia / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To explore the efficacy and adverse effects of clofarabine for relapsed/refractory acute lymphoblastic leukemia in children.</p><p><b>METHODS</b>Twenty-six pediatric patients with relapsed/refractory acute lymphoblastic leukemia were treated with clofarabine. There were 22 males and 4 females, with a mean age of 9.5 years (ranging from 4 to 17 years). They received clofarabine 52 mg/m2 intravenously over 2 hours daily for 5 days. Thirteen patients received two cycles and one patient received three cycles.</p><p><b>RESULTS</b>In the first cycle of clofarabine, complete remission was obtained in 11 children (42%) and partial remission was obtained in 7 children (27%). Eight children (31%) were considered unresponsive. In the second cycle, 11 (85%) of the 13 children obtained complete remission, 1 (8%) partial remission and 1 (8%) was unresponsive. One child received three cycles and obtained complete remission in each cycle. The common adverse events were myelosuppression, infection, liver dysfunction and gastrointestinal adverse reactions. There were no chemotherapy-related deaths.</p><p><b>CONCLUSIONS</b>Clofarabine is effective in the treatment of children with relapsed/refractory acute lymphoblastic leukemia and its adverse effects can be tolerated. Clofarabine could be a promising new treatment for relapsed/refractory acute lymphoblastic leukemia.</p>

Analysis of life-threatening central never system complications of acute leukemia in 26 children / 中华实用儿科临床杂志

Objective To investigate the clinical characteristics and risk factors of childhood acute leukemia (AL) with severe neurologic complications.Methods From Jun.1991 to Mar.2011,26 AL patients with severe neurologic complications in Peking University People's Hospital were enrolled.The incidence,clinical features,and risk factors for severe neurologic complications were retrospectively analyzed.Results There 26 patients included 8 cases of acute lymphoblastic leukemia,17 cases of acute myeloid leukemia(AML) and 1 case of acute mixed lineage leukemia.There were 20 patients taking CT scan and 17 patients were confirmed with intracranial hemorrhage.Six cases of AML without CT scan were dead.The patients suffering from intracranial hemorrhage all had intraparenchymal hemorrhage.The AML-M5 with intracranial hemorrhage had higher white blood cell count and higher level of L-lactate dehydrogenase than those without intracranial hemorrhage.These were 5 cases(31.25％) of AML with platelet count ＜ 20 × 109/L,12 cases(70.58％) of AML with prolonged prothrombin time,7 cases(41.17％) of AML with prolonged activated partial thromboplastin time,and 8 cases(47.06％) of AML with low fibrinogen when the severe neurologic complication occurred.Conclusions The most common type of severe neurologic complications of childhood AL is intracranial hemorrhage.The patients with AML are prone to occur intracranial hemorrhage.Intensive blood production transfusion may be beneficial to reduce the probability of intracranial hemorrhage in these patients.

Clinical features and prognosis of t (8; 21)/AML1-ETO-positive childhood acute myeloid leukemia / 中国当代儿科杂志

<p><b>UNLABELLED</b>OBJECTIVE To study the clinical and biological characteristics and prognosis of t(8;21)/AML1-ETO-positive childhood acute myeloid leukemia (AML).</p><p><b>METHODS</b>The clinical data of 55 children who were diagnosed as t (8; 21)/AML1-ETO-positive AML were retrospectively studied. Event-free survival (EFS), disease-free survival (DFS), and overall survival (OS) rates were estimated by the Kaplan-Meier method. Prognostic factors were evaluated by COX regression analysis software.</p><p><b>RESULTS</b>Of the 55 patients, 4 patients gave up treatment after the diagnosis was confirmed and 4 patients were lost to follow-up after the first chemotherapy course. The remaining 47 patients received a double-induction therapy. The total complete remission (CR) rate was 71% and 94% after the first and second chemotherapy course, respectively. The disease was relapsed in 10 patients (21%). The 5-year EFS, DFS and OS rates were (56.1 ± 7.9)%, (59.8 ± 8.1)%, and (72.0 ± 8.1)%, respectively. Multivariate analysis showed that age was an independent risk factor for the long-term prognosis. The older children had a greater risk of experiencing an accident or death (P<0.05). The 5-year OS rate in 27 patients with regular consolidation chemotherapy was significantly higher than 13 patients with irregular chemotherapy after CRz [(47.5 ± 17.1)% vs (38.9 ± 17.3)%; P<0.01].</p><p><b>CONCLUSIONS</b>Childhood t(8;21)/AML1-ETO-positive AML is a highly heterogeneous disease, with a high CR rate and a good long-term prognosis. Age is one of the important factors affecting the long-term therapeutic effect. Regular consolidation chemotherapy applied after CR usually is helpful.</p>

Clinical features and etiological spectrum in children with pancytopenia / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the clinical features and etiological spectrum of pancytopenia in children.</p><p><b>METHODS</b>The clinical data of 174 children with pancytopenia between September 2003 and January 2010 were retrospectively reviewed.</p><p><b>RESULTS</b>Pale face was the most common clinical manifestation (147 cases, 84.5%), followed by bleeding (87 cases, 50.0%) and fever (41 cases, 23.6%). Mild to moderate anemia, severe thrombocytopenia and mild leucopenia were common in complete blood count. Of the 174 children, pancytopenia was attributed to hematopoietic system diseases in 155 cases (89.1%) and non-hematopoietic system diseases (virus infections, systemic lupus erythematosus, hypersplenism and neuroblastoma) in 6 cases (3.4%). Aplastic anemia (91 cases, 52.3%) was the most common cause of pancytopenia, followed by myelodysplastic syndrome (37 cases, 21.3%), acute leukemia and other hematological tumours (11 cases, 6.3%) and hemophagocytic syndrome (6 cases, 3.4%). The cause of pancytopenia was not identified in 13 cases (7.5%).</p><p><b>CONCLUSIONS</b>Anemia, bleeding and fever are the main clinical manifestations of pancytopenia in children. Pancytopenia is mostly caused by aplastic anemia in children. Myelodysplastic syndrome, hematological tumours and hemophagocytic syndrome are also the common causes.</p>

Clinical characteristics of children with B cell type acute lymphoblastic leukemia carrying different fusion gene / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate whether there were differences in the clinical characteristics, cytogenetic characteristics, immunophenotype and prognosis in children with B cell type acute lymphoblastic leukemia (B-ALL) carrying different fusion genes.</p><p><b>METHODS</b>The research included 80 children with B-ALL from Peking University People's Hospital between March 2006 and December 2008. Eighteen children were positive for TEL/AML1, 14 for E2A/PBX1, 11 for BCR/ABL,and 2 cases for MLL/AF4, and 35 cases were negative for all of the 4 fusion genes. Data including clinical characteristics, morphology, immunophenotype and cytogenetic characteristics were collected, and the disease-free survival (DFS) was evaluated. The children were followed up until April 2009.</p><p><b>RESULTS</b>In the 18 children with TEL/AML1+B-ALL, 66.7% were younger than 5 years old. They had low tumor load. FAB-L2 morphology was commonly observed, but t(12;21) was often absence in these children. Up to now,17 children who survived were disease-free. In the 14 children with E2A/PBX1+B-ALL, the majority were female. Thirteen children showed FAB-L1 morphology. Twelve children showed pre-B-ALL immunophenotype. The EFS was close to 80%. In the 11 children with BCR/ABL+B-ALL, 10 children showed common B type immunophenotype. FAB-L1 and FAB-L2 morphology was found in 4 children respectively. The DFS was less than 20%. Two children with MLL/AF4 positive B-ALL had high tumor load. Their morphologic diagnosis was FAB-L1. Both showed the Pro-B-ALL immunophenotype. One child discontinued treatment at the early stage of chemotherapy, and the other child survived disease-free until now.</p><p><b>CONCLUSIONS</b>The B-ALL children with different fusion genes have different clinical characteristics, immunophenotypes and prognosis.</p>

Identification of gene rearrangements in childhood leukemia by using a multiplex polymerase chain reaction-microarray approach / 中国实验血液学杂志

The aim of study was to investigate the application of a novel microarray approach for the eight most common leukemia translocations in children for routine molecular diagnostic hematopathology practice. Bone marrow samples from 84 children with leukemia were analyzed by multiplex nested RT-PCR combined with oligonucleotide microarray. The results showed that out of 84 leukemic samples, 31 (36. 90%) carried 8 types of fusion genes including tel/aml1, e2a/pbx1, bcr/ablp190, bcr/ablp210, mll/af4, aml1/eto, pml/raralpha, cbfbeta/myh11. The sensitivity and specificity of the assay is comparable with the RT-PCR technique. In conclusion, this multiplex nested RT-PCR could quickly screen 8 types of chromosome structural aberrations at the same time. It can provide reliable and helpful information for risk stratification, therapy evaluation and prognosis prediction in childhood leukemia. There are both advantages and disadvantages in applying this new method. The microarray-based assay will be an effective and reliable tool in the clinical screening of leukemia patients for the presence of specific gene rearrangements with important diagnostic and prognostic implications.

Can As2O3 improve the prognosis of childhood acute promyelocytic leukemia?--A single center experience / 中华血液学杂志

<p><b>OBJECTIVE</b>To retrospectively analyze the treatment outcomes and side effects of childhood acute promyelocytic leukemia (APL) treated with all-trans retinoic acid (ATRA) or ATRA + arsenic trioxide (As2O3).</p><p><b>METHODS</b>From 1992 to 2006, 45 patients with newly diagnosed APL were enrolled. All of them were PML-RAR alpha positive. 24 patients were induced with ATRA (group A) and 21 with ATRA + As2O3 (group B). The remission rate and side effects were observed.</p><p><b>RESULTS</b>1) 19 (79.2%) patients in group A achieved CR, while 21(100%) patients in group B achieved CR. The CR rate in group A was lower than that in group B (P=0.027). 2) The recovery time of coagulation parameters and PLT count in group B was shorter than that in group A. 3) The overall survival (OS) and event-free survival(EFS) in group A were 77.8% and 66.9% at 41 months of follow-up, and in group B were 100% and 100% respectively at 34 months of followup. Group A had a significant lower EFS (P=0.0357)than group B. 4) The time of PML-RAR alpha fusion gene converting to negative in group A was longer (P=0.026) than that in group B.</p><p><b>CONCLUSIONS</b>ATRA + As2O3 for patients with newly diagnosed childhood APL is a feasible treatment with higher CR rate, less side effects and longer long-term survival.</p>

Investigation of Leukemia-Associated Immunophenotyping at Relapse and Treatment Failure in Children with Acute Lymphoblastic Leukemia / 实用儿科临床杂志

Objective To investigate the stability of immunophenotyping in the course of relapse or at treatment failure of patients with acute lymphoblastic leukemia(ALL) and that of immunophenotyping of positive minimal residual disease(MRD).Methods From Aug.2000 to Dec.2007,33 children with ALL who relapsed or treated failure were enrolled. These children were detected MRD by flow cytometry. The immunophenotyping of children who relapsed or treated failure were compared with that of initial therapy;the immunophenotyping of MRD relapsed was compared with that of initial therapy.Results 1.In 23 out of 27 cases (85.18%) with B-ALL,changed at least 1 antigen between diagnosis and relapse.Six children with CD45 down-modulation and 2 children with CD45 up-modulation.Two children with CD19 down-modulation and 1 child with CD19 up-modulation.Six children with CD34 down-modulation and 4 children with CD34 up-modulation. Five children with CD10 down-modulation and 7 children with CD10 up-modulation.2.Six children with T-ALL had the same expression in CD45 between relapse and treatment failure. 3.These were 15 children had the least 1 case MRD,25 cases MRD were detected,these was 1 case up-modulation in CD45,1 case down-modulation in CD19,2 cases up-modulation and 8 cases down-modulation in CD34,3 cases up-modulation and 6 cases down-modulation in CD10.Conclusions Immunophenotyping of children with ALL may change at relapse and treatment failure. The frequency of change in B-ALL is higher than that of in T-ALL,but the change can not impact the detection of MRD.

Clinical Value of Acute Myeloblastic Leukemia 1-ETO Fusion Transcripts in Children with Acute Myeloblastic Leukemia by Using Real-Time Quantitative Reverse Transcriptase Polymerase Chain Reaction / 实用儿科临床杂志

Objective To analyze the clinical value of minimal residual disease(MRD) of acute myeloblastic leukemia(AML) with AML 1-ETO by using the real-time quantitative reverse transcriptase polymerase chain reaction(RQ-RT-PCR).Methods From Jan.2001 to Jan.2007,the MRD of 32 AML1-ETO-positive AML patients were analyzed by using PQ-RT-PCR.The detection of the AML1-ETO was taken after the induced chemotherapy every 1.5-2.0 months during the consolidation therapy.The survival of different stages in children with AML was analyzed by SPSS 10.0 software and calculated by using Kaplan-Meier analysis.Results Thirty-two patients received the induced chemotherapy and 29 patients with complete remission morphologically,3 patients had no complete remission morphologically and then gave up.Patients with molecular remission were associated with a high probability of survival(P=0.001 8).Patients with high transcript levels at diagnosis had no difference in event free survival with patients with low transcript levels.The quality of molecular response after induction,6 months in the chemotherapy as well as consolidation period,has significant impact on the event free survival(P=0.023,0.000 1,0.004 9).Conclusion The current study demonstrate that quantitative evaluation of AML1-ETO transcript levels is important and may be helpful for therapeutic decisions in future.

Clinical Analysis of Nosocomial Infection in 71 Children with Acute Leukemia / 实用儿科临床杂志

Objective To explore the clinical characteristics of nosocomial infection in children with acute leukemia and the strategy of prevention and treatment.Methods One hundred and thirty-three cases of nosocomial infection in children with acute leukemia were analyzed by retrospective study.The relationship between nosocomial infection and stage of leukemia,hospitalization duration,and the rate of infection were investigated.Results Nosocomial infection rate was 53.4%(71/133 cases),significant difference of infection rate between acute lymphoblastic leukemia and nonlymphoblastic leukemia group was found(P0.05).The main pathogens of septicaemia were gram negative bacilli,and they were generally sensitive to Amicacin and Pi-peracillin/tazobactam.Conclusions Children with acute leukemia have high nosocomial infection rate.The occurrence of nosocomial infection was related to the type and stage of leukemia and hospitalization duration but not to the prognosis.The main pathogens of septicaemia were gram negative bacilli.

The clinical significance of detecting minimal residual disease in acute childhood B-cell lymphoblastic leukemia with flow cytometry / 中华儿科杂志

<p><b>OBJECTIVE</b>Flow cytometry may be used to detect minimal residual disease (MRD) in acute lymphoblastic leukemia because leukemic cells often display aberrant phenotypes when compared to normal cells. The present study was designed to establish a flow cytometric method for detecting MRD in children with B-ALL and evaluate its clinical prognostic value. The investigators also aimed to study the value of the detection of MRD by flow cytometry in childhood B-ALL without effective antibody combinations.</p><p><b>METHODS</b>Thirty-six cases of childhood B-ALL with effective antibody combinations were performed MRD analysis after induction therapy. The authors detected MRD in 6 cases without effective antibody combinations by the four-color antibody combinations consisting of CD(45)/CD(19)/CD(10)/CD(34) and CD(45)/CD(19)/CD(20)/CD(22) and detected the aberrance of the minor subsets of CD(19)(+) cells.</p><p><b>RESULTS</b>(1) Forty-two cases of childhood B-ALL were screened for antibody combinations of interest and were identified in 86% (36/42) of the cases. The sensitivity of this method was 0.01%. (2) Patients with MRD levels > or = 0.01% at 9 and 12 months of therapy had significantly low disease-free survival compared with patients with MRD levels < 0.01%. (3) Six out of seven patients with recurrence in the BM had MRD levels > or = 0.1% prior to recurrence. Patients with MRD levels > or = 0.1% during chemotherapy had significantly low disease-free survival as compared with patients with MRD values < 0.1%. (4) Two out of seven patients with recurrence had positive results of the qualitative PCR prior to recurrence. (5) Five patients with recurrence had no shift of antigen expression at relapse except that a patient missed CD(13). (6) Detectable MRD was not found in six patients without effective antibody combinations.</p><p><b>CONCLUSION</b>(1) Flow cytometry is a sensitive and specific method for detecting MRD of childhood ALL, and could predict the coming relapse. (2) Patients with MRD levels > 10(-3) had poor prognosis. (3) The levels of MRD at month 9 and 12 had prognostic value. (4) The value of antibody combinations consisting of CD(45)/CD(19)/CD(10)/CD(34) and CD(45)/CD(19)/CD(20)/CD(22) should be further investigated in patients without effective antibody combinations.</p>

Clinical significance of detection of AML1/ETO fusion transcripts in childhood AML using real-time quantitative reverse transcription polymerase chain reaction / 中国实验血液学杂志

This study was aimed to investigate the clinical value of quantification of AML1/ETO fusion transcripts using real-time reverse transcription PCR. Fourteen AML1/ETO positive children out of 52 AML children were selected. A serial dilution of AML1/ETO plasmid was used as a template for the AML1/ETO real-time PCR. AML1/ETO was quantified according to the expression of the GAPDH housekeeping gene at new diagnosis and during/after chemotherapy and transplantation. SPSS statistics was used to analyze the data. The results showed that the ratio of AML1/ETO: GAPDH expression level at new diagnosis varied in the range 0.219-2.080 (median 0.648) among the patients, without relevance with percentage of blasts. The detection sensitivity was up to the dilution of 1:10(5). Six patients showed a slight decline of AML1/ETO (higher than 5 x 10(-2)) at 1 month, three of whom relapsed in the early stage and one later. Five patients had a higher level than 5 x 10(-3) at 3 months, three of whom relapsed. Four patients with always a higher level than 5 x 10(-3) all relapsed in early stage. After six months, four out of them with constant low-level expression (10(-4) - 10(-6)) were in continuous complete hematological remission (CCR). In another patient, a rapid rise of AML1/ETO transcripts could be detected at CR stage and he relapsed 5 months later. The AML1/ETO gene expression leveling off by 10(-5) - 10(-6) could be detected in 3 patients at their complete remission after 9 months. It is concluded that real-time RT-PCR is a suitable approach for quantifying AML1/ETO transcripts in monitoring of AML patients with t(8;21) during/after chemotherapy and provides data of diagnostic relevance.