Objective To study the survivin expression in mononuclear cells of both bone marrow and peripheral blood in childhood acute leukemia(AL).Methods Twenty-one children with AL were divided into newly diagnosed group(n=12) and complete remission(CR) group(n=9).The control group was consisted of 15 children without tumor.Mononuclear cells were separated from bone marrow and peripheral blood (PBL) by gradient density centrifugation and RNA was extracted,and RT-PCR was performed to detect survivin mRNA.Results In newly diagnosed AL group,6 out of 12 patients had positive survivin expression in bone marrow(50%) and 4 out of 11 patients in the PBL(36.37%),both of which were higher than the control group(P0.05).In 9 CR patients,all the patients had the negative expression in the PBL,but 1 positive expression in bone marrow(11.11%).The positive expression in CR group was significantly lower than the newly diagnosed AL group.The only one patient with positive survivin expression in CR group had relapse 7 weeks later.In newly diagnosed patients,the average immature leukocytes were(76.43?43.28)?10~(9)/L in the patients with positive survivin expression and(13.85?5.86)?10~(9)/L in those with negative survivin expression(P

Objective To investigate the clinical therapeutic effect of filiform fire needling on tennis elbow.Method Eighty patients with tennis elbow were randomly allocated to a filiform fire needling group of 30 cases, a block group of 20 cases and an electroacupuncture group of 30 cases. The treatment group received. Pain severity was scored using the VAS after one and two weeks and one month of treatment. The therapeutic effects were evaluated and compared after one month of treatment.Result The total efficacy rate was 93.3% in the filiform fire needling group, 60.0% in the block group and 50.0% in the electroacupuncture group; there was a statistically significant difference among the three groups (P<0.05). There was a statistically significant difference between the filiform fire needling and block groups (P<0.05) and between the filiform fire needling and electroacupuncture groups (P<0.05) but no significant difference between the block and electroacupuncture groups (P>0.05). The VAS score decreased significantly in the block group (P<0.05) but did not in the filiform fire needling and electroacupuncture groups (P>0.05) after one week of treatment compared with before. The VAS score decreased significantly in the filiform fire needling and block groups (P<0.05) but did not in the electroacupuncture group (P>0.05) after two weeks of treatment compared with before. There was no statistically significant posttreatment difference in the VAS score between the filiform fire needling and block groups (P>0.05) but there was such a difference between the filiform fire needling or block group and the electroacupuncture group (P<0.05). There was a statistically significant difference in the VAS score in the filiform fire needling and block groups (P<0.01) and also in the electroacupuncture group (P<0.05) between before and after one month of treatment. There was a statistically significant difference in the VAS score after one month of treatment between the filiform fire needling group and the block or electroacupuncture group (P<0.05) and between the block and electroacupuncture groups (P<0.05). The results of the study showed that filiform fire needling, local drug blockage and electroacupuncture all had clinically a better therapeutic effect on tennis elbow and a better improving effect on the symptoms in the patients. Filiform fire needling produced a marked therapeutic effect. Its effect was superior to those of block therapy and electroacupuncture.Conclusion Filiform fire needling is a definitely effective way to treat tennis elbow.

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 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.

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

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.

Acute Disease ; Child ; Down Syndrome ; Humans ; Leukemia

Objective:To understand the clinical manifestations, diagnosis, treatment, and prognosis of children with hereditary thrombocytopenia (HT).Methods:The clinical data of 5 patients with HT in the Hematology and Oncology Department of Children′s Hospital of Chongqing Medical University from August 2015 to October 2017 were retrospectively analyzed. The clinical and laboratory characteristics, treatment, and prognosis of HT were discussed by reviewing relevant literatures.Results:Five patients included 3 boys and 2 girls.The median age at onset of 4 years and 2 months old and the median age at diagnose was 4 years and 4 months old.All patients presented with the thrombocytopenia, among which 4 cases were macrothrombocytopenia and 1 case was normothrombocytopenia.The main clinical presentations of 5 patients were skin petechiae and ecchymoses.Four cases were initially misdiagnosed as immune thrombocytopenia (ITP) and received the glucocorticoid and immunoglobulin, while the therapeutic effect was not satisfactory.The gene sequencing confirmed MYH9 gene mutation(c.3493C>T), MYH9 gene mutation(c.5878G>A), NBEAL2 gene compound heterozygous mutation(c.295C>T; c.4169C>T), GP1BA gene mutation(c.1761A>C), and ANKRD26 gene mutation(c.5123A>G), in 5 patients respectively. Conclusions:HT should be suspected among those with recurrent isolated thrombocytopenia and no response to the ITP regimen, and the early gene screening is of great significance to the patients′ treatment and prognosis.

OBJECTIVETo investigate the incidence, clinical symptoms, signs and laboratory features of childhood hemophagocytic lymphohistiocytosis (HLH) in China.

METHODSA retrospective study was performed on 217 pediatric patients with HLH who were admitted to Children's Hospital of Chongqing Medical University from January 2006 to April 2013. All patients'medical records were reviewed and analyzed.

RESULTSThe Male to female ratio was 1.11:1. The median onset age was 3 years and 5 months old (range of 6 months old to 16 years and 9 months old), and the age of onset peaked between 1-5 years old (61.3%). The most common causes of HLH was infection, especially Epstein-Barr virus-associated HLH (71.0%). Other causes included malignant hemophagocytic syndrome (MAHS), macrophage activation syndrome (MAS) and so on. The outstanding clinical manifestations including persistent fever (100.0%), hepatomegaly (92.6%), splenomegaly (88.4%), and more than half of cases with central nervous system involvement and pulmonary manifestations. Laboratory data indicated that the most prominent abnormality was elevated ferritin (98.0%), and the others were hemophagocytosis in bone marrow (90.7%) and coagulation abnormalities (76.5%). Abnormal lymphocytes classification is very common in HLH.

CONCLUSIONHLH is a heterogeneous disease, with a variety of the etiology and clinical manifestations. HLH-2004 diagnostic protocol had theoretical basis and clinical operability. The hepatitis damages related indicators, lymphocytes classification, central nervous system involvement and pulmonary performance can be used as reference value for HLH diagnosis.

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Lymphohistiocytosis, Hemophagocytic ; diagnosis ; Male ; Retrospective Studies