Invasive angiomyxoma（AAM） is characterized by unclear boundaries, non enveloped glial growth, high recurrence rate, and belongs to a benign tumor, but it is invasive and grows slowly. A patient with recurrent left vestibular invasive angiomyxoma was admitted to the Otorhinolaryngology ward of Beijing Traditional Chinese Medicine Hospital Affiliated with Capital Medical University. The patient underwent two repeated surgeries and underwent a combined internal and external nasal approach for the removal of the nasal vestibular angiomyxoma. The patient recovered well after the surgery and has not recurred since follow-up.
Humans ; Myxoma/pathology* ; Neoplasm Recurrence, Local ; Nose Neoplasms/pathology*

Objective:To analyze the clinical characteristics and risk factors of asparaginase-associated pancreatitis(AAP)in children with acute lymphoblastic leukemia(ALL),and to investigate the impact of AAP on their prognosis following re-exposure to asparaginase(ASP).Methods:Clinical children data with ALL at Tongji Hospital,Tongji Medical College of Huazhong University of Science and Technology between January 2015 and June 2020 were collected to analyze the clinical features of AAP.Logistic regression was used to identify risk factors for AAP.Prognostic analysis was performed using the Log-rank test and Kaplan-Meier survival curves.Results:Overall,252 children with ALL were included,among whom 23(9.1%)developed AAP.Most AAP cases(82.6%)occurred during remission induction,with a medi-an time from the last ASP to AAP of 12 d.Elevated total cholesterol(≥3.5 mmol/L)at initial diagnosis was identified as an independent risk factor.Six children(26.1%)were re-exposed to ASP,leading to recurrent pancreatitis in 3 cases.The 5-year overall survival(OS)was signific-antly lower in the AAP group(78.3%±8.6%)compared to the non-AAP group(90.3%±2.2%)(P<0.05).Similarly,children who discontinued ASP due to AAP had a 5-year OS of 77.8%±9.8%,significantly lower than the control group(90.1%±2.1%).Conclusions:AAP typically oc-curred within 12 d of the last ASP administration and was associated with poorer 5-year OS.Re-exposure to ASP posed a risk of recurrent AAP;however,completing the ASP chemotherapy regimen may be crucial for improving prognosis.

Objective:To investigate the expression of WT1 gene in children with acute lymphoblastic leukemia (ALL), and explore its clinical characteristics and correlation with the prognosis of ALL.Methods:The clinical data of 183 children with newly diagnosed ALL in Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology from January 2015 to May 2019 were retrospectively analyzed. The expression level of WT1 gene in bone marrow samples was detected by real-time fluorescence quantitative polymerase chain reaction. The children were followed up to June 2021 with a median follow-up time of 46 months (0 to 63 months).Results:Among 183 children with ALL, the WT1 gene positive was in 130 cases (71.04%), and the expression level was 1.41% (0.26%, 6.73%); WT1 gene negative was in 53 cases (28.96%). The expression levels of WT1 gene in children with T-cell lymphoblastic leukemia (T-ALL), non-hyperdiploid and middle/high-risk were significantly increased, and there were statistical differences ( P<0.05 or <0.01); however, there were no statistical differences in the expression levels of WT1 gene between children with different gender, chromosome karyotype, hepatosplenomegaly and the first diagnosis white blood cell count ( P>0.05). There were no statistical differences in complete remission rate and recurrence rate after induction chemotherapy between WT1 gene positive children and WT1 gene negative children: 87.69% (114/130) vs. 86.79% (46/53) and 16.15% (21/130) vs. 18.87% (10/53), P>0.05. By the end of follow-up, 179 children were followed up, and there was no statistical difference in survival rate between WT1 gene positive children and WT1 gene negative children: 89.68% (113/126) vs. 86.79% (46/53), P>0.05. Among the children with WT1 gene positive, relapse was in 21 cases, and there was no statistical difference in the expression level of WT1 gene after complete remission or after relapse, compared with that while the first diagnosis ( P>0.05); among non-relapse children, 96 completed the detection, the expression level of WT1 gene after complete remission was significantly lower than the first diagnosis: 0.17% (0.04%, 0.49%) vs. 2.01% (0.41%, 8.82%), and there was statistical difference ( P<0.01). Kaplan-Meier survival curve analysis result showed there was no statistical difference in survival time between WT1 gene positive children and WT1 gene negative children ( P>0.05). According to the median expression level of WT1 gene (1.41%), the children with WT1 gene positive were divided into high expression (66 cases) and low expression (64 cases), there was no statistical difference in survival time between high expression children and low expression children ( P>0.05). Conclusions:WT1 gene is commonly expressed in children with ALL and is associated with some clinical features and prognosis of the children. Decreased WT1 gene expression may result in better prognosis.

Objective:To investigate the influencing factors of delayed methotrexate (MTX) elimination after high-dose methotrexate (HD-MTX) treatment in children with acute lymphoblastic leukemia (ALL) and the effects of delayed MTX elimination and HD-MTX reduction on the prognosis of children with ALL.Methods:The clinical data of 242 children with ALL diagnosed and treated in Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology from January 2015 to June 2020 in accordance with the Chinese Children's Cancer Group study ALL 2015 (CCCG-ALL 2015) were retrospectively analyzed. Low risk and intermediate/high risk children respectively received 3 g/m 2 and 5 g/m 2 HD-MTX for 4 times, and the serum MTX concentration was monitored. The serum MTX concentration > 1 μmol/L at 44 h of administration was considered as the delayed elimination, which was divided into mild (> 1 μmol/L and ≤ 5 μmol/L), moderate (> 5 μmol/L and ≤ 10 μmol/L) and severe (> 10 μmol/L) delayed elimination. Univariate and multivariate logistic regression analysis were used to analyze the influencing factors of delayed MTX elimination, and univariate Cox proportional hazards model was used to analyze the related factors of ALL relapse. Results:The 242 children with ALL completed 962 times of HD-MTX chemotherapy. The median serum MTX concentration [ M ( Q1, Q3)] at 44 h of administration was 0.45 μmol/L (0.33 μmol/L, 0.72 μmol/L). The total incidence of delayed MTX elimination was 17.7% (170/962). The incidence of mild, moderate and severe delayed elimination was 13.8% (133/962), 2.6% (25/962) and 1.2% (12/962), respectively. Logistic regression analysis showed that age ≥ 7 years old ( OR = 1.68, 95% CI 1.17-2.41, P = 0.005), MTX dose >3 g/m 2 at each course ( OR = 2.14, 95% CI 1.52-3.03, P < 0.001) and the first course of HD-MTX chemotherapy ( OR = 2.05, 95% CI 1.43-2.93, P < 0.001) were independent risk factors for delayed MTX elimination. The median follow-up time was 50 months (34 months, 68 months), 12.8% (31/242) of the children relapsed, and the median relapse time was 30 months (30 months, 39 months). Univariate Cox regression analysis showed that there were no significant differences in the relapse rates among children with different gender, immunophenotype, risk, the number of delayed MTX elimination, and the completion of HD-MTX chemotherapy (the ratio of MTX average dose to initial planned dose) (all P > 0.05). Conclusions:The independent risk factors of delayed elimination of MTX in children with ALL are age ≥ 7 years old, MTX dose > 3 g/m 2 at each course and the first course of HD-MTX chemotherapy. Delayed elimination of MTX and reduction of HD-MTX have no significant effect on ALL relapse.

Objective:To investigate the clinical features of acute megakaryocytic leukemia (AMKL) in children.Methods:The clinical data of 14 children with AMKL in Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology from January 2012 to July 2021 were retrospectively analyzed, and the related literature was reviewed.Results:Among 14 children with AMKL, there were 5 males and 9 females, and the median age of onset was 19 months (0.1-109 months); 1 case was Down syndrome-related AMKL, and 13 cases were non-Down syndrome-related AMKL. Most of the children presented with fever, anemia or bleeding symptoms, and a few patients presented with joint pain as the primary symptom. Some children were accompanied by extramedullary infiltration such as hepatomegaly, splenomegaly or lymphadenovarix. Initial investigations of 14 children showed that the median white blood count, hemoglobin concentration and platelet count were 10.67×10 9/L [(6.56-83.62)×10 9/L], 84 g/L (55-121 g/L), 37×10 9/L [(8-1443) ×10 9/L], respectively, and the median proportion of naive cells in peripheral blood was 0.09 (0.00-0.79). Bone marrow smear showed that the primitive megakaryocytes were characterized by various size and irregular form, a few of which had cytoplasmic vacuoles, and the median proportion of bone marrow primitive megakaryocytes was 0.636 (0.332-0.976); the nuclei were round or irregular, with multiple nucleoli or hidden nucleoli. RAS staining was partially positive, and immunohistochemical assay showed that POX, AS-DNCE and α-NBE were negative. Detection of megakaryocyte-associated antigens by flow cytometry showed 12 children expressed CD41a or CD61, and 10 children expressed CD42b. Among 3 children who completed chemotherapy, 1 case of Down syndrome-related AMKL and 1 case of non-Down syndrome-related AMKL were event-free survival, and 1 case of non-Down syndrome-related AMKL died after bone marrow relapse. Conclusions:The clinical manifestations and biological characteristics of children with AMKL are complicated and the prognosis is poor. Some children can achieve disease-free survival through chemotherapy alone.

Objective:To explore the clinical features and prognosis of childhood acute lymphoblastic leukemia(ALL) complicated with EB virus (EBV) infection.Methods:The results of detection of EBV antibody and EBV-DNA in peripheral blood mononuclear cells of 196 children with ALL diagnosed in Tongji Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology from January 2015 to January 2019 were collected. According to the results, 196 children with ALL were divided into EBV infection group and non-EBV infection group. The hepatomegaly and splenomegaly, chromosome, peripheral blood routine, immunophenotyping, clinical risk, secondary infection during chemotherapy, minimal residual disease (MRD) of day 46 after chemotherapy, karyotype, and prognosis were compared between the two groups. The children were followed up until April 30, 2019.Results:Among 196 children with ALL, EBV infection rate was 72.96% (143/196). The EBV-DNA level [median ( P25, P75)] of peripheral blood mononuclear cells was 3.7×10 3 copies/L(1.6×10 3 copies/L, 8.8×10 3 copies/L). The incidence of hepatosplenomegaly (subcostal ≥ 5 cm) in EBV infection group was higher than that in non-EBV infected group [14.69% (21/143) vs. 3.77% (2/53), χ 2= 4.45, P= 0.035]. There was no significant difference in the number of white blood cells and the incidence of abnormal karyotype between EBV infection group and non-EBV infection group (both P > 0.05). The secondary infection rate in EBV infection group was higher than that in the non-EBV infection group [41.96% (60/143) vs.24.53% (13/53), χ2= 5.03, P= 0.025], and the remission rate of day 46 in EBV-infection group was lower than that in non-EBV infection group [80.42% (115/143) vs. 98.11% (52/53), χ2= 9.60, P= 0.020]. The recurrence rate in EBV-infection group was higher than that in non-EBV infectious group [11.89% (17/143) vs. 1.89% (1/53), χ2= 4.64, P= 0.031], and there was a significant difference in the component ratio of immunophenotyping and clinical risk between the two groups (both P < 0.05). Conclusions:The hepatosplenomegaly in children with ALL complicated with EBV infection is obvious, the secondary infection rate is high, the remission rate is low, the recurrence rate is high, and the prognosis is poor. EBV infection may be related to immunophenotyping and clinical risk in children with ALL, and has nothing to do with the abnormal karyotypes.

Objective To study the clinical features and prognostic analysis of 36 children with relapsed acute lymphoblastic leukemia(ALL)treated with the ALL 2006 protocol.Methods The data of 308 children who were new-ly diagnosed as ALL at the Department of Pediatric Hematology,Tongji Hospital,Tongji Medical College,Huazhong Uni-versity of Science Technology treated with the ALL 2006 protocol between January 2007 and December 2012 were col-lected,and the clinical features and prognosis of 36 children with relapsed ALL were retrospectively analyzed.The date included initial treatment time,age,gender,immunophenotyping,the white blood cell count,the risk classification,the chromosome,the fusion gene(29 kinds of fusion genes including MLL rearrangement,BCR/ABL,E2A/PBX1,TEL/AML1)in relapsed patients with ALL on the initial diagnosis and recurrence time,recurrence site,as well as whether to get second complete remission(CR2),follow-up time,follow-up deadline condition.Results After treatment with ALL 2006 protocol,the recurrence rate was 11.7%(36/308 cases);the 3 years overall survival rate was(38.0 ± 9.0)%.The recurrence happened almost in the very early stage,about 75.0%(27/36 cases),and the recurrence rate of the early stage and the late stage patients was 16.7%(16/36 cases)and 8.3%(3/36 cases),respectively.The re-lapsed sites were mainly in the bone marrow alone(66.7%,24/36 cases),and the extramedullary recurrence and the combination of bone marrow with extramedullary recurrence was 16.7%(6/36 cases).The 3-year OS was(16.4 ± 8.0)% and(80.0 ± 18.0)% for those relapsed in the very early stage and early stage,respectively(P=0.002).The 3-year OS of the high-risk,medium-risk relapsed patients were(21.0 ± 11.0)%,(51.9 ± 16.0)% and(64.3 ± 21.0)%(P=0.022).Conclusions After the treatment with ALL 2006 protocol,the patients with recurrence were almost at the very early stage.The relapsed sites were mainly in bone marrow alone.The factor associated with survival time of children with relapsed ALL is the relapse at the very early stage.

Objective: To analyze the mutation type of FⅧ gene in children with hemophilia A and to explore the relationship among hemophilia gene mutation spectrum, gene mutation and clinical phenotype. Method: Sixty-two children with hemophilia A from Department of Pediatric Hematology, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology between January 2015 and March 2017 were enrolled. All patients were male, aged from 4 months to 7 years and F Ⅷ activity ranged 0.2%-11.0%. Fifty cases had severe, 10 cases had moderate and 2 cases had mild hemophilia A. DNA was isolated from peripheral blood in hemophilia A children and the target gene fragment was amplified by PCR, in combination with the second generation sequencing, 22 and 1 introns were detected. Negative cases were detected by the second generation sequencing and results were compared with those of the international FⅧ gene mutation database. Result: There were 20 cases (32%) of intron 22 inversion, 2 cases (3%) of intron 1 inversion, 18 cases (29%) of missense mutation, 5 cases (8%) of nonsense mutation, 7 cases (11%) of deletion mutation, 1 case(2%)of splice site mutation, 2 cases (3%) of large fragment deletion and 1 case of insertion mutation (2%). No mutation was detected in 2 cases (3%), and 4 cases (7%) failed to amplify. The correlation between phenotype and genotype showed that the most common gene mutation in severe hemophilia A was intron 22 inversion (20 cases), accounting for 40% of severe patients, followed by 11 cases of missense mutation (22%). The most common mutation in moderate hemophilia A was missense mutation (6 cases), accounting for 60% of moderate patients. Conclusion The most frequent mutation type in hemophilia A was intron 22 inversion, followed by missense mutation, again for missing mutation. The relationship between phenotype and genotype: the most frequent gene mutation in severe hemophilia A is intron 22 inversion, followed by missense mutation; the most frequent gene mutation in medium hemophilia A is missense mutation.

OBJECTIVETo compare the clinical efficacy difference in dysantonomia between transcutaneous electrical stimulation at Renying(ST 9) combined with stellate ganglion block(SGB) and simple SGB.

METHODSSixty patients in accord with the diagnostic criteria of dysantonomia were randomly divided into two groups,30 cases in each group. In the observation group,transcutaneous electrical stimulation at Renying(ST 9) combined with SGB were adopted; in the control group,simple SGB was applied. In the two groups, treatment was used three times a week,and nine treatments were considered as one course. There was an interval of one week between courses,and two courses were treated. Total seven weeks were required. Scores were evaluated according to subjective symptoms before treatment,one month and three months after treatment in the two groups.

RESULTSThe scores of subjective symptoms were not statistically different before treatment in the two groups(P>0. 05). The scores of subjective symptoms one month and three months after treatment were all lower than those before treatment(all P< 0. 01), and subjective symptoms scores in the observation group were lower than those in the control group(both P<0. 01).

CONCLUSIONTranscutaneous electrical stimulation at Renying(ST 9) combined with SGB could obviously enhance the clinical effects for dysantonomia, and the control and improvement for clinical symptoms are apparently superior to simple SGB.

Acupuncture Points ; Adult ; Anesthetics ; administration & dosage ; Autonomic Nerve Block ; Autonomic Nervous System Diseases ; drug therapy ; therapy ; Combined Modality Therapy ; Female ; Humans ; Male ; Middle Aged ; Stellate Ganglion ; drug effects ; physiopathology ; Transcutaneous Electric Nerve Stimulation ; Young Adult

Objective To analyze the clinical features of pediatric patients with acute lymphoblastic leukemia(ALL) with bcr-abl fusion gene transcript,and discuss the treatment,prognosis factors of this kind of ALL.Methods Clinical features,treatment and prognosis were studied retrospectively in 7 bcr-abl fusion gene positive ALL patients.bcr-abl fusion gene was detected by reverse transcription polymerase chain reaction (RT-PCR).Results The average age of the 7 patients was 8 years and 1 month old.All of them were common B-immunology ALL.The rate of complete response was 50 ％ after 33 days' treatment.Conclusions The incidence rate of bcr-abl fusion gene positive ALL in pediatric is low.This type of ALL has poor remission,high relapse rate and poor prognosis.