Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy

Objective To investigate the relationship among methotrexate(MTX) plasma concentration,dosage,clinical effecicy and toxicity, and to evaluate it′s clinical significance.Methods MTX was measured by a flurorescence polarization immunoassay in plasma samples obtained from acute lymphoblastic leukemia(ALL) patients treated in different doses of MTX, and these results were analyzed combined with clinical manifestations.Results 1.The average of plasma concentration at 24 hours increased with the increasing doses of MTX. The relapse rate decreased with increased plasma concentration;2.The cerebrospinal fluid(CSF) concentrations prior to the intrathecal MTXinstillation were all below the effective concentration, so the intrathecal MTX instillation was needed;3.No severe toxicity was observed in the study, because the plasma concentration was below the high risk.Conclusion The study of MTX plasma concentration provides us an objective basis for the individualized chemotherapy.

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.

Objective To learn more about the clinical and laboratory features of childhood paroxysmal nocturnal hemoglobinuria(PNH) and to improve the diagnosis.Methods The clinical and laboratory features of 12 cases of PNH were analyzed,who were diagnosed from January 2000 to November 2004,and the positive responses to treatments were observed.Results 1) The youngest age of onset was 2 years;the disease often manifested with anemia(100%),recurrent infections(50%) and hemorrhages(33%),occurring mainly in skin and mucosa.No patient developed a thrombosis.2) 66.7% of the patients showed peripheral blood cytopenia.Dysplasia of bone marrow was observed in 25% of patients.Fifty percent of them had an increased percentage of erythroid lineage.3) Positive hemolytic tests included urine OB 41.6%,Ham′s test 33.3% and Rous′ test 25%.Glycosyl-phosphatidyl inositol(GPI) deficient cells were found in 100% of the patients.4) 57.1% of patients was improved after being treated with adrenocortical hormone,androgens or cysporin.Conclusions Besides hemoglobinuria,peripheral blood cytopenia were also the common manifestation of PNH.Flow cytometry based immunophenotypic methods for the analysis of CD_(55) and CD_(59) may improve the diagnosis of PNH.J Appl Clin Pediatr,2006,21(3):153-154

Objective To explore the immunophenotype of children with acute myeloid leukemia(AML) and its clinical significance.Methods Statistics was used to analyze the relationship between the immunophenotype of AML and their French-American-Britain(FAB) classification,complete remission (CR) in one month and 3-years event-free survival(EFS).Results CR rate was 71.6% and 3-years EFS rate was 50.8%. HLA-DR and CD34 absent mainly in M3, associated with higher CR and EFS rate. So did CD33 negative cases, especially in M2. CD13 positive was significantly predictive factor for achieving CR.Co-expression of lymphoid antigens and NK cell antigens(CD56) with M2 which correlated with lower CR and EFS rate.Conclusions The negative of HLA-DR, CD34, CD33,as well as CD13 positive, have relationship with good prognosis. Lymphoid antigens and CD56 are poor prognostic factors.

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.

OBJECTIVETo explore the clinical characteristics and prognosis of children and adolescents over 10 years of age with acute lymphoblastic leukemia (ALL).

METHODSA 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.

RESULTSOf 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.

CONCLUSIONSChildren 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.

Adolescent ; Child ; Female ; Humans ; Leukocyte Count ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; genetics ; mortality ; Prognosis ; Proportional Hazards Models

OBJECTIVETo study the clinical features and etiological spectrum of pancytopenia in children.

METHODSThe clinical data of 174 children with pancytopenia between September 2003 and January 2010 were retrospectively reviewed.

RESULTSPale 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%).

CONCLUSIONSAnemia, 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.

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Pancytopenia ; blood ; diagnosis ; etiology

OBJECTIVETo study the relationship between loss of sex chromosomes and prognosis in children with acute myeloid leukemia (AML) M2 subtype.

METHODSAccording 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.

RESULTSThe 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).

CONCLUSIONSLoss 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.

Adolescent ; Child ; Child, Preschool ; Chromosomes, Human, Pair 21 ; Chromosomes, Human, Pair 8 ; Female ; Humans ; Karyotype ; Leukemia, Myeloid, Acute ; genetics ; mortality ; Male ; Prognosis ; Sex Chromosome Aberrations ; Translocation, Genetic

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.
Adolescent ; Body Height ; Child ; Dasatinib ; therapeutic use ; Growth Disorders ; Humans ; Leukemia, Myeloid, Acute ; drug therapy ; Retrospective Studies