The common problems for non-special pediatric medical practitioners in the depart-ment of hematology include short-time of rotation, weak foundation of pediatric hematology and low enthusiasm of learning. The entrance education was highlighted in order to make refreshers familiar-ize with the severity of illness and eliminate the medical error. Basic theoretical study was strength-ened aiming at improving clinical skill and enhancing the learning interesting. Method of doctor-patient communication was reformed to reduce medical disputes.

Objective To evaluate the treatment of current status and prognosis in childhood APL with APL2008 ,which was administrated since 2008 in our center .Methods A total of 43 children with newly diagnosed APL between 2008 to 2014 were studied retrospectively .Treatment options and current status were summarized from 28 patients who received APL2008 therapy . Results Studied 43 patients were at median age of 8 years and 4 months ,with 28 boys and 15 girls .The main clinical manifestations were infection ,anemia ,bleeding ,fever ,hepatomegaly ,splenomegaly and lymphadenopathy .The proportions of low ,intermediate and high risk groups were 27 .9% ,48 .8% and 23 .3% ,respectively .Eleven cases could be diagnosed as DIC .Bone marrow morphology showed abnormal elevation of promyelocyte .37 patients had distinctive immunophenotype such as frequent expression of CD33 , CD117 and MPO .PML/RARαfusion gene positive rate was 100% in 43 children and cytogenetic analysis were positive in 37 cases , of which specific genetic lesion in APL cells with t (15 ;17)(q22 ;q12) was found in 28 cases ,and karyotypes was found in 9 cases as infrequent chromosomal abnormalities .In 43 patients ,4 cases were early dead from intracranial hemorrhage at early stage ,and 11 cases were given up early .There were only 2 cases dead ,2 cases relapsed and 1 case lost among 28 APL children ,which enabled ef‐ficacy analysis possible .96 .4% of these 28 cases achieved HCR .The 2 year Kaplan Meier estimates of OS and EFS were 85 .9% ± 7 .6% and 80 .4% ± 8 .8% .But OS and EFS would be 94 .7% ± 5 .1% and 88 .9% ± 7 .4% if 3 patients who had non standard treat‐ment were excluded .Conclusion Childhood APL were characterized by anemia ,bleeding ,fever and infiltration .APL′s coincidence rate between PML/RARa fusion gene and morphology ,immunology and cytogenetics were 95 .3% ,90 .2% and 86 .5% ,respective‐ly .APL2008 significantly improved the prognosis of APL .

Objective To investigate the relationship between EVI1 gene expression and clinical features and prognosis of children with acute myeloid leukemia (AML). Methods EVI1 gene was detected in AML children, correlation of clinical and lab features, prognosis of AML children with EVI1 gene were analyzed. Results EVI1 expression is positive in 38 of 145 children with AML. There were no significant differences in age, gender, hemoglobin concentration, leukocytes and platelet count, subtype of morphology, ratio of chromosomal anomaly and complex karyotypes between EVI1 positive and EVI1 negative group (P>0.05); coexist genes were detected in 9 cases (23.68%) of EVI1 positive group. Rate of complete remission (CR) was 91.67% in 24 cases of EVI1 positive patients received chemotherapy. Relapse rate was 64.29% and 14.29% in EVI1 positive patients who received chemotherapy and allo-hematopoietic stem cell transplantation (allo-HSCT), retrospectively and significant differences were found (P<0.05). There was no significant difference in CR but significant difference was found in event free survival (P<0.05) for EVI1 positive and EVI1 negative patients who received chemotherapy. EVI1 gene kept negative when bone marrow relapse occurred in two patients with EVI1 positive at diagnosis. Conclusion EVI1 gene may play adverse role in pediatric AML; prognosis of EVI1 positive AML patients can be improved by allo-HSCT; follow-up of EVI1 transcript levels is insufficient to monitoring of minimal residual disease.

Objective To evaluate the efficacy and safety in treatment of patients with mild to moderate Alzheimer’s disease (AD). Methods A total of 233 patients with mild to moderate potential AD were enrolled in a 16-week multi-center double blind clinical trial. All patients were randomized into two groups. 110 patients in galantamine group and 108 patients in donepezil group were enrolled in efficacy analysis. The scales of Alzheimer’s Disease Assessment Scale-Cognitive Subscale (ADAS-cog), Alzheimer’s Disease Cooperative Study Activities of Daily Living Scale (ADCS-ADL) and The Neuropsychiatric Inventory (NPI) were used to assess the effect at both baseline and the end of 16 weeks. Safety issues, including vital signs, lab assays and ECG examinations were measured. Results Patients in both groups were obviously improved in the total score of ADAS-cog (-5.4?6.4) in the galantamine group and (-4.0?7.3) in the donepezil group, P=0.098). 76% patients of the galantamine group had a score of ADAS-cog less than 20 at the end of 16 weeks treatment, which was higher than that of the donepezil group (58%, P=0.015). The sub-score of speech ability in ADAS-cog were improved in the galantamine group (baseline 2.8?2.9,16 weeks 1.8?2.5) compared with the donepezil group (baseline 2.8?3.0, 16 weeks 2.3?2.9, P=0.035). No significant difference of ADSC-ADL and NPI scale was found between the two groups (P=0.447 and 0.936 respectively). The sleep/night behavior was improved in the donepezil group (baseline 14%, 16 weeks 10%) compared with the galantamine group (baseline 23%, 16 weeks 22%, P=0.012). Two drug-related severe adverse events occurred during the trial, which were platelet reduction in the galantamine group and acute drug-induced hepatic injury in the donepezil group. The incidence of adverse events was 44% in the galantamine group and 47% in the donepezil group respectively. Galantamine had little influence on vital signs and lab assays. Conclusion Safe and well tolerated, galantamine improves the cognition, activities of daily living and neuropsychiatric symptoms of patients with mild to moderate AD.

Objective: To investigate the characteristics and prognostic factor of central nervous system (CNS) involvement in patients with hemophagocytic lymphohistiocytosis (HLH) . Methods: From January 2006 to October 2015, 152 patients with HLH, 88 patients had CNS involvement, their clinical data were collected, and survival was analyzed using the Kaplan-Meier life table method, univariate and multivariate Cox regression model analyses were applied to identify the risk factors of prognosis. Results: ①57.9% patients complicated with neurological symptoms, cerebrospinal fluid abnormalities were observed in 37.0% patients, 57.5% patients had abnormal neuroradiology. ②36 patients survived well, 3 patients lost to follow-up, 49 dead, 1 survival patient had epilepsy. ③The 3-year overall survival rate of 88 patients was 44%. ④abnormal CSF and unreceived IT bore a significant, independent adverse prognostic value (P<0.05) . Conclusion CNS involvement in HLH has a high frequency and poor prognosis, few patients remained neurologic sequelae; abnormal CSF related to poor prognosis, positive intrathecal injections could improve the prognosis.

Objective To explore the risk factors affecting the prognosis of patients with small bowel adenocarcinoma(SBA),construct the SBA survival risk model,and evaluate the clinical predictive value.Methods Clinical information and prognosis data of 2 639 patients included in the surveillance,epidemiology,and end results(SEER)database were retrospectively analyzed.Overall survival(OS)and disease specific survival(DSS)were used as prognostic indicators.The training group and validation group were randomized at a 7 ∶ 3 ratio using univariate and multivariate Cox regression analysis.Prognostic factors affecting SBA sur-vival were screened,and a prognostic prediction model was constructed.The receiver operation characteristic curve,model valida-tion by validation group,and clinical decision curve.Results Age(P<0.01),tumor site(P = 0.018),size(P = 0.042),T stage(P<0.01),detection rate of positive lymph nodes(P<0.01),single tumor focus(P<0.01),and secondary liver metastasis(P<0.01)were independent risk factors affecting prognosis of OS in patients with SBA;age(P<0.01),tumor size(P= 0.022),T stage(P<0.01),detec-tion rate of positive lymph nodes(P<0.01),single tumor focus(P<0.01),and secondary liver metastasis(P<0.01)were independent risk factors affecting the prognosis of DSS in patients with SBA.The nomogram,survival risk assessment model,and calibration prediction curve were consistent with the actual curve.Conclusion Age,tumor size,T stage,detection rate of positive lymph nodes,single tumor focus,and secondary liver metastasis were independent risk factors for OS and DSS in patients with SBA.Tumor site was also an inde-pendent risk factor for OS in SBA patients.The established prognostic prediction model has good predictive value,can effectively evaluate the prognosis of SBA patients,and can provide reasonable treatment advice for patients.

Objective To investigate the clinical features and prognosis of central nervous system(CNS)in-volvement in Epstein-Barr virus(EBV)associated hemophagocytic lymphohistiocytosis(HLH).Methods A total of 89 patients with EBV-HLH diagnosed in Children's Hospital of Chongqing Medical University from June 2006 to Octo-ber 2015 were divided into involved CNS group and non-involved group according to whether there was CNS involve-ment. The clinical manifestations,laboratory examinations and outcomes of these two groups were analyzed. Results Among these 89 patients with EBV-HLH,39 patients developed CNS disease,19 cases of them had neuro-logical symptoms or signs,including convulsions,unconsciousness,facial palsy,dysarthria,dysphagia,irritability,neck stiffness,Babinski sign positive,opisthotonus;9 cases of them had abnormal cerebrospinal fluid(CSF),with elevated white blood cell count and protein level;26 patients had abnormal brain images,including deepen or widening cortical sulci,atrophy,hemorrhage,high T2 signal in magnetic resonance imaging(MRI).The 3-year survival rate in involved CNS group was lower than those of non-involved group(66.7% vs.86.0%),and there was statistical significance (χ2=4.267,P=0.039).The involved CNS group had higher ferritin(χ2=5.092,3.921;P=0.024,0.048)and lower platelets(Z= -2.643,P=0.008)than those of non-involved group,and there were statistical significances.COX mul-tivariate analysis showed that neurological symptom and abnormal CSF were independent prognostic factors(RR=3.134, 3.339,all P<0.05).Conclusion CNS involvement is frequent in EBV-HLH.The prognosis of children with involved CNS group is worse than those of non-involved group.Neurological symptoms and abnormal CSF are related to poor prognosis.

OBJECTIVETo explore the clinical features, laboratory findings and treatment of infant leukemia.

METHODSA retrospective analysis of the clinical data was performed of the cases with the diagnosis of infant acute leukemia from August 1993 to October 2014 in our hospital.

RESULTSA total of 144 cases of infant leukemia were diagnosed in the defined period, including 83 cases of acute lymphoblastic leukemia, 55 myeloid leukemia, 1 hybrid acute leukaemia and 5 with incompatible cytological and immunophenotyping findings. The patients at the age of 9 to 12 months accounted for the largest proportion (38.2%), and 87.5% of the patients had hepatosplenomegaly; Six patients below 6 months old had skin infiltration. In about 1/3 of the patients, the white blood cells count was no greater than 100 × 10⁹ /L. Ninety-five patients had chromosome examinations, which identified chromosome abnormalities in 67 patients, including 18 positive for t(4;11)or t(9;11)or t(11;19), and younger patients were more likely to have chromosome abnormalities. Thirty-seven patients underwent MLL gene detection and 11 of them had positive results; the positive patients had higher rate of chromosome 11 abnormalities than the negative patients. Most of the patients gave up treatments after diagnosis and only 6 patients older than 6 months completed regular chemotherapeutic treatments and were now in complete remission.

CONCLUSIONInfant leukemia is a rare type of leukemia with different clinical features from other types of leukemia. The patients often present with hepatosplenomegaly, high white blood cell counts, MLL gene fusion, and chromosome 11 abnormalities. The prognosis of infant leukemia is not favorable, and the current treatment still relies on chemotherapy.

Acute Disease ; Chromosome Aberrations ; Chromosome Disorders ; Chromosomes, Human, Pair 11 ; Humans ; Immunophenotyping ; Infant ; Leukemia, Myeloid ; pathology ; Leukocyte Count ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; pathology ; Prognosis ; Retrospective Studies

Acute Disease ; Child ; Down Syndrome ; Humans ; Leukemia

OBJECTIVETo explore the risk factors, and control measures of cytomegalovirus (CMV) infection after hematopoietic stem cell transplantion (HSCT) in children with primary immunodeficiency diseases(PID).

METHODSWe retrospectively analyzed results of 26 patients with PID-Wiskott-Aldrich syndrome (WAS, n=20), severe combined immunodeficiency (SCID, n=1) , X-linked chronic granulomatous disease (XCGD, n=2) and X-linked hyper-immunoglobulin M (IgM) syndrome (XHIM, n=3)-who underwent HSCT from June 2007 to December 2012 in our center. Serologic studies (ELISA) and weekly CMV infection surveillance (quantitative PCR, qPCR) were routinely performed before and after HSCT. Ganciclovir or forcarnet was used for pre-emptive and curative therapy.

RESULTSAll 26 patients were male with the median age at HSCT of 27 months (range 7-77 months). At a median follow up of 24 months (range 5-66 months), the 5-year overall survival rate was (75.0 ± 9.0) %. CMV infection occurred in 42.3% (11 of 26) of the patients, two of them developed CMV interstitial pneumonia (CMVIP). Univariate analysis revealed that the incidence of pre-transplant CMV infection between with and without CMV activation groups after HSCT was significantly different (62.5% vs 10.0%, P=0.010). Additional variables not associated with CMV infection were stem-cell sources, donor type, HLA disparity and acute GVHD (all P values>0.05).

CONCLUSIONCMV infection was a major complication of HSCT. Sensitive monitoring, early diagnosis, timely treatment may improve the survival rate for these PID undergoing HSCT.

Child ; Child, Preschool ; Cytomegalovirus Infections ; virology ; Graft vs Host Disease ; Granulomatous Disease, Chronic ; therapy ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Infant ; Male ; Retrospective Studies ; Risk Factors ; Severe Combined Immunodeficiency ; therapy ; Tissue Donors ; Wiskott-Aldrich Syndrome ; therapy