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1.
Zhongguo Dang Dai Er Ke Za Zhi ; 26(4): 365-370, 2024 Apr 15.
Article in Zh | MEDLINE | ID: mdl-38660900

ABSTRACT

OBJECTIVES: To summarize the clinical characteristics, treatment outcomes, and prognostic factors of children with non-metastatic Ewing's sarcoma (ES). METHODS: A retrospective analysis was conducted on the clinical data of 41 children with non-metastatic ES diagnosed and treated at the Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine from January 2010 to December 2018. All patients underwent chemotherapy based on the RMS-2009 protocol of the center, and local treatment such as surgery and/or radiotherapy was performed according to risk grouping. The Kaplan-Meier method was used to calculate the overall survival (OS) and event-free survival (EFS) rates. Univariate prognostic analysis was performed using the log-rank test, and multivariate analysis was conducted with Cox regression. RESULTS: Of the 41 children, 21 were male and 20 were female. The median age at diagnosis was 7.7 years (range: 1.2-14.6 years). The median follow-up time for patients with event-free survival was 68.1 months (range: 8.1-151.7 months). As of the last follow-up, 33 patients were in complete remission, and the overall 5-year EFS and OS rates were (78±6)% and (82±6)%, respectively. Univariate analysis by the log-rank test showed that a tumor diameter ≥8 cm, time from diagnosis to start of local treatment ≥16 weeks, and incomplete surgical resection were associated with poor prognosis (P<0.05). Multivariate Cox regression analysis indicated that incomplete surgical resection (HR=8.381, 95%CI: 1.681-41.801, P=0.010) was an independent risk factor for poor prognosis in children with ES. Secondary tumors occurred in 2 cases. CONCLUSIONS: A comprehensive treatment strategy incorporating chemotherapy, surgery, and radiotherapy can improve the prognosis of children with ES. Poor prognosis is associated with an initial tumor diameter ≥8 cm, while complete surgical resection and early initiation of local treatment can improve outcomes.


Subject(s)
Sarcoma, Ewing , Humans , Sarcoma, Ewing/therapy , Sarcoma, Ewing/mortality , Sarcoma, Ewing/pathology , Female , Male , Child , Adolescent , Child, Preschool , Infant , Retrospective Studies , Bone Neoplasms/therapy , Bone Neoplasms/mortality , Bone Neoplasms/pathology , Prognosis , Treatment Outcome
2.
Mediators Inflamm ; 2022: 4250621, 2022.
Article in English | MEDLINE | ID: mdl-35664920

ABSTRACT

Preeclampsia (PE) is a common pregnancy-related syndrome characterized by chronic immune activation. This study is aimed at exploring the role of miR-155 in the inflammatory pathogenesis of PE. Placental tissues and peripheral blood were collected from all subjects. BSP detection analysis was performed to evaluate miR-155 methylation levels. ELISA was performed to measure the levels of inflammatory cytokines and MMP2 in serum samples and cellular supernatants. HTR-8/SVneo and JEG-3 cells were transfected with miR-155 mimic and the inhibitor to establish the overexpressed miR-155 and silenced miR-155 cell models, respectively. Treatment with 5-Aza was performed to alter the DNA methylation level of miR-155. The PE rat model was established after subcutaneous injection of NG-nitro-L-arginine methyl ester. The CCK-8 assay, TUNEL staining, and Transwell assay were performed. Reverse transcription-quantitative PCR, Western blot analysis, and immunohistochemical assay were used to analyze related gene expression levels. The luciferase reporter assay was used to investigate the direct interaction between FOXO3 and miR-155. Results showed that miR-155 was remarkably upregulated and inversely correlated with the promoter methylation level in the placental tissue from PE patients. The in vitro experiments indicated that miR-155 decreased viability, migration, and invasion, but increased apoptosis in trophoblast cells. FOXO3 was confirmed as the target of miR-155. Transfection of the miR-155 inhibitor suppressed inflammation and oxidative stress, but elevated proliferation, migration, and invasion of trophoblast cells, which were abolished by 5-Aza treatment or cotransfection with si-FOXO3. In summary, our data suggested that methylation-mediated silencing of miR-155 can inhibit the apoptosis, inflammation, and oxidative stress of trophoblast cells by upregulating FOXO3.


Subject(s)
MicroRNAs , Pre-Eclampsia , Animals , Cell Line, Tumor , Cell Movement/genetics , Cell Proliferation/genetics , Female , Forkhead Box Protein O3/genetics , Forkhead Box Protein O3/metabolism , Humans , Inflammation/metabolism , Methylation , MicroRNAs/genetics , MicroRNAs/metabolism , Placenta/metabolism , Pre-Eclampsia/metabolism , Pregnancy , Rats , Trophoblasts/metabolism
3.
Pediatr Hematol Oncol ; 38(2): 97-107, 2021 Mar.
Article in English | MEDLINE | ID: mdl-33016804

ABSTRACT

The standard chemotherapy for acute myeloid leukemia (AML) is usually composed of anthracyclines and cytarabine. We previously reported that homoharringtonine (HHT) was incorporated into regimens for pediatric AML with acceptable efficacy and tolerable toxicity. We treated newly diagnosed AML patients aged 0-18 years on the AML-SCMC-2009 protocol. A total of 102 de novo newly diagnosed AML patients aged 0-18 years were enrolled. All patients were treated with ten courses of chemotherapy including double induction, high dose cytarabine consolidation, and maintenance. The cumulative dose of HHT was 165 mg/m2 and the daunorubicin dose was 120 mg/m2. Complete remission (CR), overall survival (OS) rate, event free survival (EFS) rate, adverse effect response and prognosis factors were retrospectively evaluated to investigate the long-term outcome and safety of this protocol. Eighty-two patients (80.4%) achieved complete remission with the first induction. The 5-year overall survival and event-free survival rates were 65.0% (SE, 4.9%) and 53.3% (SE, 5.2%), respectively. A first induction failure, age older than 2 years, and BCR-ABL translocation were associated with a significantly worse outcome (p < 0.05). No significant drug-related cardiac toxicity was observed. AML-SCMC-2009 protocol which use HHT as backbone drug is efficacious and tolerated. HHT could partially replace anthracycline to be an effective option for childhood AML.


Subject(s)
Homoharringtonine/therapeutic use , Leukemia, Myeloid, Acute/drug therapy , Protein Synthesis Inhibitors/therapeutic use , Adolescent , Child , Child, Preschool , China , Female , Homoharringtonine/pharmacology , Humans , Infant , Infant, Newborn , Male , Protein Synthesis Inhibitors/pharmacology , Retrospective Studies
4.
Cancer Immunol Immunother ; 69(11): 2333-2343, 2020 Nov.
Article in English | MEDLINE | ID: mdl-32514618

ABSTRACT

Patients with pediatric cancers such as neuroblastoma (NB) are often unresponsive to checkpoint blockade immunotherapy. One major factor in pediatric tumor resistance to immunotherapy is considered to be the low mutation rate of pediatric tumors. Another factor may be the overexpression of additional inhibitory pathways. While analyzing the RNA-sequencing database TARGET, we found that human NB tumors overexpress immune checkpoint molecule CD200. To determine its significance and impact on tumor immune microenvironment, we analyzed 49 cases of previously untreated, surgically removed NB tumors using immunohistochemistry and multi-color flow cytometry (FACS). We found that CD200 is overexpressed in more than 90% of NB tumors. In the tumor microenvironment of NB, CD200 is mainly overexpressed in CD45- NB tumor cells, while its cognate receptor (CD200R) is mainly expressed in HLA-DR+CD14+ myeloid cells and CD11c+ dendritic cells. Low-level expression of CD200R is also observed in tumor-infiltrating CD4+ and CD8+ T cells. In NB tumors with higher CD200 expression (CD200high), we observed lower numbers of HLA-DR+CD14+ myeloid cells and less tumor-infiltrating CD4+ and CD8+ T cells. Moreover, we found that CD4+ and CD8+ T cells produced less IFN-γ and/or TNF-α in CD200high NB tumors. Thus, CD200-CD200R pathway appears to downregulate anti-tumor immunity in the tumor microenvironment of NB tumors, and blockade of this pathway may be beneficial for NB patients.


Subject(s)
Antigens, CD/immunology , Lymphocytes, Tumor-Infiltrating/immunology , Neuroblastoma/immunology , Tumor Microenvironment/immunology , CD4-Positive T-Lymphocytes/immunology , CD8-Positive T-Lymphocytes/immunology , Child, Preschool , Female , Humans , Infant , Male , Orexin Receptors/immunology , Tumor Escape/immunology
5.
Int J Cancer ; 144(1): 117-124, 2019 01 01.
Article in English | MEDLINE | ID: mdl-30098202

ABSTRACT

Langerhans cell histiocytosis (LCH) is the most common histiocytosis with constitutive activation of the RAS-RAF-MEK-ERK (MAPKinase) cell signaling pathway. We analyzed 89 cases of BRAF and MAP2K1 mutations by Sanger sequencing, of which 18 cases showed that these two gene mutations are negative. Whole genome sequencing of suitable specimens in these negative cases revealed a translocation from the 3 intron of PLEKHA6 to the 13 intron of NTRK3 in one case. We identified that this translocation could cause a novel fusion mutation, PLEKHA6-NTRK3. Overexpression of the PLEKHA6-NTRK3 mutant in NIH 3T3 cells enhanced MAPKinase pathway activation, promote cell growth. Our result suggested that a new mutation need be included in LCH molecular screening panel to better define its prevalence in LCH.


Subject(s)
Histiocytosis, Langerhans-Cell/genetics , Intracellular Signaling Peptides and Proteins/genetics , Receptor, trkC/genetics , Recombinant Fusion Proteins/genetics , Adolescent , Animals , Cell Proliferation/genetics , Child , Child, Preschool , Female , Histiocytosis, Langerhans-Cell/pathology , Humans , Infant , Male , Mice , Mutation , NIH 3T3 Cells
6.
Opt Express ; 27(7): 9933-9940, 2019 Apr 01.
Article in English | MEDLINE | ID: mdl-31045140

ABSTRACT

Development of the wideband and tunable quasi-optic terahertz (THz) components is in high demand. In this work, we demonstrate a tunable achromatic quarter-wave plate (AQWP) for the THz frequency range. The phase retardation of this device can be set at 90° ± 9° from 0.20 to 0.50 THz. The operation range from 0.20 to 0.50 THz can be tuned to from 0.30 to 0.70 THz by introducing three nematic liquid crystals phase retarders, of which the birefringence can be magnetically tuned. The frequency-dependent phase retardation is in good agreement with theoretical predictions.

7.
Pediatr Hematol Oncol ; 36(2): 113-121, 2019 Mar.
Article in English | MEDLINE | ID: mdl-31002269

ABSTRACT

OBJECTIVES: To assess the impact of second-look biopsy of residual mass during or after chemotherapy in pediatric mature B-cell NHL. METHODS: Patients with mature B-cell non-Hodgkin lymphoma (NHL) who were suspicious of radiological residual mass at mid or end of treatment and subjected to second biopsy were treated at our center between January 2001 and December 2015. Their clinical characteristics, imaging findings, pathological changes, management, and prognosis were reviewed retrospectively. RESULTS: A total of 31 children were included (13 boys and 18 girls, median age at diagnosis 6.1 years). The median time from diagnosis to second biopsy was 3.15 months (range 2.3-18 months). Biopsy confirmed the presence of viable tumor in eight patients. The specificity and positive predictive value of conventional imaging in detecting residual detectable by biopsy were at 9 and 28.6%, while sensitivity and negative predictive value of this approach were both 100%. Three of the histologic positive patients experienced progressive disease or relapse while the others achieved complete remission (CR) and 21 patients achieved long-time CR at median follow-up of 3.2 years. The median progression-free survival (PFS) time of all 31 was 28 months and 5-year PFS rate was 90.0%. Five-year PFS rate of negative-biopsy and positive-biopsy group were 100 and 62.5%, respectively (p = 0.002). CONCLUSION: Conventional imaging has very high sensitivity but very low specificity for the accurate determination of residual disease in pediatric NHL. Second-look biopsy is necessary to differentiate viable tumor from necrosis or fibrosis and is solid evidence-based foundation of subsequent treatment.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biopsy , Lymphoma, B-Cell/pathology , Adolescent , Child , Child, Preschool , Female , Humans , Kaplan-Meier Estimate , Lymphoma, B-Cell/diagnostic imaging , Lymphoma, B-Cell/drug therapy , Magnetic Resonance Imaging , Male , Neoplasm, Residual , Predictive Value of Tests , Prognosis , Progression-Free Survival , Remission Induction , Retrospective Studies , Sensitivity and Specificity , Tomography, X-Ray Computed
8.
Zhongguo Dang Dai Er Ke Za Zhi ; 21(11): 1073-1078, 2019 Nov.
Article in Zh | MEDLINE | ID: mdl-31753087

ABSTRACT

OBJECTIVE: To study the clinical effect of the SCMC APL-2010 regimen in the treatment of acute promyelocytic leukemia (APL) in children. METHODS: A retrospective analysis was performed for the clinical data of 44 children with APL who received treatment with the SCMC APL-2010 regimen between April 2010 and July 2016. The Kaplan-Meier survival analysis was used to evaluate event-free survival (EFS) rate and overall survival (OS) rate. RESULTS: Of the 44 children with APL, 42 (95%) achieved a complete remission (CR) after one course of treatment and 1 achieved CR after two courses of treatment, with an overall CR rate of 98%. The 9-year EFS and OS rates were 96%±3% and 97.7%±2.2% respectively. As for adverse events, 41 (93%) had infection, 29 (66%) had granulocyte reduction, 12 (27%, 1 died) had differentiation syndrome, 16 (36%) had liver dysfunction, 12 (27%) had adverse gastrointestinal reactions, and 7 (16%) had QT prolongation, 1 (2%) had orchitis, and no secondary neoplasm was observed. CONCLUSIONS: Children with APL receiving the SCMC APL-2010 regimen have a good prognosis and can achieve a long-term survival, while treatment-related infection is commonly seen.


Subject(s)
Leukemia, Promyelocytic, Acute , Antineoplastic Combined Chemotherapy Protocols , Child , Disease-Free Survival , Humans , Male , Remission Induction , Retrospective Studies , Treatment Outcome , Tretinoin
9.
Hematol Oncol ; 36(4): 679-688, 2018 Oct.
Article in English | MEDLINE | ID: mdl-30133806

ABSTRACT

Long-term follow-up data for childhood acute lymphoblastic leukemia (ALL) are scarce in China because of lacking population-based and hospitalized registry system. This retrospective study, conducted at Shanghai's Children's Medical Center in China (SCMC), aimed to investigate the long-term results of childhood ALL and to identify prognostic factors. The Pediatric Oncology Network Database, designed by St. Jude Children's Research Hospital, USA, were used to collect data for the enrolled patients starting in 2005. From 2005 to 2014, 1085 evaluable patients with ALL aged 1 to 18 years old were enrolled and treated using SCMC-ALL-2005 risk-stratified protocol. Complete remission was achieved in 95.6% of patients. At 5 and 10 years, the event-free survival rate was 68.3 ± 1.4% and 64.6 ± 1.6%, and the overall survival rate was 80.0 ± 1.2% and 76.3 ± 1.6%, respectively. The 5-year event-free survival rates were 81.8 ± 2.0%, 67.0 ± 1.9%, and 14.3 ± 4.0% for patients in low-risk, intermediate-risk, and high-risk groups, respectively. The cumulative risk of relapse was 24.5% at 10 years. Induction failure conferred worse prognosis. Patients younger than 1 year of age at diagnosis, intermediate-risk/high-risk group, male gender, and positive minimal residual disease (MRD) results at day 55, both in the univariate and multivariate analysis, were associated with significantly worse prognosis (P < .05). Patients with positive MRD at both day 35 and day 55 were related to a significantly poor outcome (P < .0001), but not for patients with negitive MRD at day 35. The overall outcomes for ALL patients treated with protocol SCMC-ALL-2005 in SCMC are lower than in developed countries. Factors including age, gender, risk group and MRD results at day 55 were associated with treatment outcomes in childhood ALL.


Subject(s)
Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Child , Child, Preschool , China/epidemiology , Female , Hematopoietic Stem Cell Transplantation , Humans , Infant , Male , Precursor Cell Lymphoblastic Leukemia-Lymphoma/blood , Precursor Cell Lymphoblastic Leukemia-Lymphoma/epidemiology , Registries , Retrospective Studies , Treatment Outcome
10.
Pediatr Blood Cancer ; 65(2)2018 Feb.
Article in English | MEDLINE | ID: mdl-28921877

ABSTRACT

BACKGROUND: Infantile fibrosarcoma (IFS) is a rare pediatric malignancy with relatively good prognosis, but the risk of progression or recurrence after therapy exists. To understand the immune microenvironment of IFS and determine if immunotherapy is a potential treatment, we analyzed T-cell responses in IFS tumors. PROCEDURE: IFS tumors were analyzed by immunohistochemistry and multicolor flow cytometry to characterize immune cell infiltration and function. Tumor infiltrating lymphocytes (TILs) were expanded in vitro and evaluated for recognition of autologous tumor cells. Real-time PCR was applied to evaluate tumor expression of chemokines/cytokines and tumor antigens. RESULTS: Significant infiltration of both CD4+ and CD8+ T cells was found in seven of 10 IFS but rarely found in age- and sex-matched rhabdomyosarcoma tumors. The TILs from recurrent IFS tumors expressed high levels of costimulatory molecules such as CD28, 4-1BB, and OX40, but little or no coinhibitory molecules such as PD-1 and CTLA4, Tim3, Lag3, and CD39. Upon activation, large portions of TILs produced IFN-γ and TNF-α. Eighteen out of 40 T cell lines generated from surgically removed tumors could recognize autologous tumor cells. Moreover, we found that IFS tumors expressed high levels of T-cell chemokines such as CXCL10 and CXCL16, and also classic tumor antigens such as CTAG2, GAGE, and NY-ESO-1, whose expression could be further enhanced by treatment with epigenetic modulator decitabine. CONCLUSIONS: IFS tumors are highly immunogenic and expansion of TILs followed by adoptive cell transfer could be a potential immunotherapy for IFS patients undergoing tumor recurrence.


Subject(s)
Antigens, Differentiation/immunology , CD4-Positive T-Lymphocytes/immunology , CD8-Positive T-Lymphocytes/immunology , Cytokines/immunology , Fibrosarcoma/immunology , Neoplasm Proteins/immunology , Adolescent , CD4-Positive T-Lymphocytes/pathology , CD8-Positive T-Lymphocytes/pathology , Child , Child, Preschool , Female , Fibrosarcoma/pathology , Fibrosarcoma/therapy , Humans , Immunotherapy , Infant , Male
11.
J Pediatr Hematol Oncol ; 40(1): e9-e12, 2018 01.
Article in English | MEDLINE | ID: mdl-29200145

ABSTRACT

We reported the outcome of 150 children newly diagnosed with multisystem langerhans cell histiocytosis following a langerhans cell histiocytosis-II-based protocol (arm B). However, the continuation treatment was extended to 56 weeks and etoposide was omitted from the continuation treatment. Risk organ (RO) involvement was defined as: liver (≥3 cm with or without functional impairment); spleen (≥2 cm below the costal margin in the midclavicular line); hematopoietic system (hemoglobin <100 g/L, and/or white blood cell count <4.0×10/L, and/or platelets <100×10/L). The lungs are not considered a RO in the current study. For the 59 patients with RO involvement (RO+), the rapid response rate (week 6) was 61.0% and the 3-year overall survival 73.4%±5.9%. Rapid responders had a better 3-year survival rate than poor responders (90.9%±5.0% vs. 45.7%±11.0%, P<0.001). Ninety-one patients without RO involvement (RO-) had a relatively low 3-year cumulative reactivation rate (10.7%). No deaths occurred in this subgroup and the 3-year overall survival of RO- patients was 100%. Poor responders of RO+ patients had an extremely poor prognosis. An effective salvage therapy is essential for this high-risk group. The initial treatment intensity and duration of continuation therapy both impact disease reactivation in RO- patients.


Subject(s)
Histiocytosis, Langerhans-Cell/diagnosis , Histiocytosis, Langerhans-Cell/therapy , Risk Assessment , Adolescent , Child , Child, Preschool , China , Female , Hematopoietic System/pathology , Histiocytosis, Langerhans-Cell/mortality , Histiocytosis, Langerhans-Cell/pathology , Hospitals, Pediatric , Humans , Infant , Liver Diseases , Male , Salvage Therapy , Splenic Diseases , Survival Rate , Treatment Outcome
12.
Pediatr Hematol Oncol ; 35(7-8): 385-392, 2018.
Article in English | MEDLINE | ID: mdl-30693828

ABSTRACT

BACKGROUND: This is a descriptive review of the clinical patterns and outcomes of children with Langerhans cell histiocytosis and single-system involvement (SS-LCH) treated at Shanghai Children's Medical Center. PROCEDURE: 60 evaluable newly diagnosed patients (37 boys, 23 girls) with a median age of 3.9 years (range: 0.3-15.3 years) and histiopathology-confirmed SS-LCH were enrolled from 2010 to 2014. All patients received systemic chemotherapy using either the DAL HX-83 or LCH-II protocol as determined by the physician. RESULTS: Bone was the most frequently affected organ (56/60, 93.3%). Of the 56 patients suffering from SS-bone disease, 35 (62.5%) had unifocal disease and 21 (37.5%) had multifocal disease. CNS-risk lesions were seen in nine patients (16.1%, 9/56) at diagnosis. Thirty-two patients were treated with the LCH-II protocol and 28 received the DAL HX-83 protocol. No patient received intralesional steroid injection at the time of surgery. CNS-risk lesion correlated with an inferior event-free survival (EFS) for patients with bone disease (62.5 ± 17.1% vs. 90.7 ± 4.5%; p = 0.039). The difference in the 5-year EFS between patients with unifocal and multifocal SS-bone LCH did not reach the statistical significance (93.8 ± 4.3% vs. 75.0 ± 9.7%; p = 0.074). No deaths were observed, leading to a 5-year OS of 100% in the present cohort of patients. Permanent consequences and secondary malignancies were not observed but were also limited by short follow-up. CONCLUSIONS: Optimal therapy for patients with SS-bone LCH has not been established. Less toxic therapeutic approaches should be considered for these patients and tested in prospective trials.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Bone Neoplasms , Central Nervous System Neoplasms , Histiocytosis, Langerhans-Cell , Adolescent , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Bone Neoplasms/drug therapy , Bone Neoplasms/mortality , Central Nervous System Neoplasms/drug therapy , Central Nervous System Neoplasms/mortality , Child , Child, Preschool , China/epidemiology , Disease-Free Survival , Female , Histiocytosis, Langerhans-Cell/drug therapy , Histiocytosis, Langerhans-Cell/mortality , Humans , Infant , Male , Retrospective Studies , Survival Rate
13.
Opt Lett ; 39(8): 2511-3, 2014 Apr 15.
Article in English | MEDLINE | ID: mdl-24979031

ABSTRACT

Indium-tin-oxide nanowhiskers were employed as transparent electrodes in a liquid-crystal terahertz phase shifter. Transmittance of the device was as high as ∼75%. Phase shift exceeding π/2 at 1.0 THz is achieved in a ∼500 µm-thick cell. The driving voltage required for the device operating as a quarter-wave plate was as low as 17.68 V (rms), an improvement of nearly an order of magnitude over previous work.


Subject(s)
Metal Nanoparticles/chemistry , Tin Compounds/chemistry , Electricity , Electrodes , Liquid Crystals , Optical Devices , Optical Phenomena
14.
Pediatr Blood Cancer ; 61(4): 659-63, 2014 Apr.
Article in English | MEDLINE | ID: mdl-24243691

ABSTRACT

BACKGROUND: This retrospective cohort study analysed the clinical characteristics and outcomes of patients with childhood lymphoblastic lymphoma (LBL) treated in Shanghai, China. PROCEDURE: From 2001 to 2010, 108 evaluable patients ≤16 years of age who were newly diagnosed with biopsy-proven LBL were treated with one of three treatment protocols: CCCG-99, SCMC-T-NHL-2002, or LBL-CHOF-2006. RESULTS: Two patients had Stage I disease, 5 had Stage II, 55 had Stage III, and 46 had Stage IV. The immunophenotype was T-cell LBL in 92 patients (85.2%) and precursor B-cell LBL in 16 (14.8%). The abandonment rate was 11.5%. Twenty-five patients (23.2%) suffered from resistant disease, including 1 with isolated central nervous system (CNS) relapse. At a median follow-up of 40.4 months (range, 0-114 months), the 5-year probability of event-free survival (pEFS) was 63.9 ± 4.6% in all patients. The 5-year pEFS for patients with pB-LBL was better than for patients with T-LBL (100% vs. 61.3 ± 5.1%, P = 0.007). Patients who had achieved complete remission on day 33 of induction had significantly better pEFS than those who had not (78.8 ± 4.6% vs. 28.2 ± 9.0%, P = 0.000). Three of 25 patients who experienced resistant disease were alive at the end of the study period. CONCLUSIONS: The abandonment rate was lower for patients with LBL than for patients with acute lymphoblastic leukemia. Prophylactic cranial radiation can be omitted for patients with LBL even when advanced-stage disease is present, as intensive systemic chemotherapy with intrathecal therapy is sufficient to prevent CNS relapse. The survival of patients with resistant disease was very poor.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Neoplasm Recurrence, Local/mortality , Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/mortality , Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/mortality , Adolescent , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Male , Neoplasm Recurrence, Local/drug therapy , Neoplasm Recurrence, Local/pathology , Neoplasm Staging , Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/pathology , Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/pathology , Prognosis , Retrospective Studies , Survival Rate , Time Factors , Young Adult
15.
J Pediatr Hematol Oncol ; 36(5): 374-8, 2014 Jul.
Article in English | MEDLINE | ID: mdl-24807005

ABSTRACT

For the first time, we conducted a 2-center retrospective study to show the efficacy of antithymocyte globulin (ATG)-Fresenius S plus cyclosporine treatment of children with severe aplastic anemia. From March 1997 to May 2011, a total of 124 patients (median age, 7.5 y; range, 1.5 to 16 y) from 2 centers with acquired AA treated with an immunosuppressive therapy (IST) regimen, consisting of ATG-Fresenius S (5 mg/kg per day for 5 d) and cyclosporine, were enrolled. The response rate was 55.6%. The median time between IST and response was 6 (0.5 to 18) months. After a median follow-up time of 29 (6 to 153) months, the rates of relapse and clonal evolution were 3.2% and 0.8%, respectively. Overall, 17 patients (13.7%) died in this study: 14 resulted from sepsis, 1 resulted from intracranial hemorrhage, 1 occurred after hematopoietic stem cell transplantation, and 1 resulted from clonal disease progression. The 5-year overall survival rate for the entire cohort was 74.7%. IST responders had a better survival rate (100%) than nonresponders (70.7%). The use of ATG-Fresenius S plus cyclosporine as a first-line immunosuppressive treatment appeared to be effective for children with severe aplastic anemia in our study. ATG-Fresenius S could be another option in the treatment arsenal, especially in countries where the other ATG products are harder to acquire.


Subject(s)
Anemia, Aplastic/drug therapy , Antilymphocyte Serum/therapeutic use , Cyclosporine/therapeutic use , Immunosuppressive Agents/therapeutic use , Secondary Prevention , Adolescent , Anemia, Aplastic/mortality , Child , Child, Preschool , Drug Therapy, Combination , Female , Follow-Up Studies , Humans , Infant , Male , Prognosis , Retrospective Studies , Survival Rate
16.
Zhonghua Yi Xue Za Zhi ; 94(20): 1547-52, 2014 May 27.
Article in Zh | MEDLINE | ID: mdl-25146742

ABSTRACT

OBJECTIVE: To evaluate the long-term outcomes of childhood stage 4 neuroblastoma (NB) and its correlative prognostic factors. METHODS: Comprehensive protocols including tumor resection, intensive chemotherapy, radiotherapy, autologous bone marrow transplantation and 13-cis-retinoid were designed and implemented. A total of 112 newly diagnosed NB stage 4 patients at Shanghai Children's Medical Center collected from June 1998 to December 2010 were treated. Their clinical features, therapeutic efficacies, long-term outcomes and prognostic factors were analyzed. RESULTS: There were 69 males and 43 females with an age range of 4 months to 15 years. Among them, 12 improving patients didn't complete treatment because of parental decisions. Among those completing the comprehensive protocols, 62 cases (62.0%) achieved very good partial remission (VGPR), 20 (20.0%) achieved partial remission (PR) while another 18 (18.0%) progressed during treatment. The efficiency rate (including VGPR+PR) of treatment was 82.0 % (n = 82). The median follow-up period was 78 (56, 120) months. And 13 cases were lost after a median follow-up of 16 months. The 2, 3, 5-year event-free survival (EFS) was 56.2% (59/105) , 40.8% (40/98) and 21.1% (19/90) respectively. Age (>18 months), poor curative effect (achieving no VGPR at the end of treatment), elevated level of lactate dehydrogenase (LDH) (> 5 times normal value), bone marrow involvement and brain metastasis were poor prognostic factors (χ(2) = 12.01, 13.66, 6.29, 5.44, 16.18, all P < 0.05) . According to the multivariate estimates of hazards, age, high levels of LDH, poor curative effect and brain metastasis were associated with a worse survival (OR = 3.54, 1.89, 3.08, 3.45, all P < 0.05) . Brain metastasis predicted a worse outcome with 100% mortality rate (n = 6). Compared to traditional chemotherapy, topotecan-based chemotherapy could not improved the efficiency (52.6% (10/19) vs 63.2% (36/57) , P > 0.05) and long-term outcome (2 ys-EFS 42.1% (8/19) vs 56.4% (31/55) , P > 0.05). CONCLUSIONS: The prognosis remains poor for neuroblastoma of stage 4. Age (>18 months), poor curative effect (achieving no VGPR at the end of treatment), elevated LDH level (>5 times normal value) and bone marrow infiltration are associated with worse prognosis. Brain metastasis predicts the worst with 100% death rate. Topotecan included chemotherapy can not be proved more effective in this study.


Subject(s)
Neuroblastoma/therapy , Adolescent , Child , Child, Preschool , Combined Modality Therapy , Female , Follow-Up Studies , Humans , Infant , Male , Neoplasm Staging , Neuroblastoma/diagnosis , Prognosis , Retrospective Studies , Treatment Outcome
17.
Front Pediatr ; 12: 1348342, 2024.
Article in English | MEDLINE | ID: mdl-38496369

ABSTRACT

Introduction: Patients with systemic lupus erythematosus (SLE) are at a higher risk of developing cancer, particularly hematological malignancies such as lymphoma and leukemia. However, existing studies on this topic that assess cancer incidence following SLE diagnosis are limited. In addition, SLE can be diagnosed after cancer, although such cases in children have been rarely reported. Case report: We present the case of a 2.6-year-old boy who presented to our institute with fever and abdominal pain. His physical examination revealed a periumbilical mass, which was pathologically diagnosed as Burkitt's lymphoma. Autologous stem cell transplantation was performed to consolidate the effect of chemotherapy and reduce the risk of cancer relapse. He was diagnosed with SLE 5 years later, following the presentation of a fever with rash, positive autoantibodies, decreased complement, and kidney involvement. At the final follow-up, the patient was still alive and showed no recurrence of Burkitt's lymphoma or disease activity of SLE. Conclusion: Despite the low frequency of SLE in children with lymphoma, cancer and SLE may be induced by a common mechanism involving B-cell cloning and proliferation. Therefore, hematologists and rheumatologists should be aware of the occurrence of these two conditions during patient follow-up.

18.
Int J Ophthalmol ; 17(6): 1049-1057, 2024.
Article in English | MEDLINE | ID: mdl-38895667

ABSTRACT

AIM: To investigate ocular surface disorders and tear function changes in patients with acne vulgaris and explore the potential relationship between acne vulgaris and dry eye. METHODS: This cross-sectional study included right eyes of 53 patients with acne vulgaris and 54 healthy controls. The participants completed the Ocular Surface Disease Index (OSDI) questionnaire. The following ocular surface-related parameters were measured: tear meniscus height (TMH), noninvasive tear breakup time (NIBUT), Schirmer I test (SIT), lipid layer thickness (LLT) score of the tear film, meibum score, meibomian gland orifice obstruction score, the ratio of meibomian gland loss, conjunctival hyperemia score, and corneal fluorescein staining (CFS) score. RESULTS: The stability of the tear film decreased in acne vulgaris patients. In the acne group, the TMH and NIBUT were lower, whereas the OSDI, meibum score, meibomian gland orifice obstruction score, ratio of meibomian gland loss, and conjunctival hyperemia score were higher compared with controls (P<0.05). There were no significant differences in the CFS score, SIT, or LLT score between the groups (P>0.05). In two dry eye groups, the TMH, NIBUT, and LLT score were lower in the acne with dry eye (acne-DE) group, and the meibum score, meibomian gland orifice obstruction score, ratio of meibomian gland loss and conjunctival hyperemia score in the acne-DE group were higher (P<0.05). There were no significant differences between OSDI, SIT, and CFS score (P>0.05). CONCLUSION: Patients with moderate-to-severe acne vulgaris are more likely to experience dry eye than those without acne vulgaris. Reduced tear film stability and meibomian gland structure dysfunction are more pronounced in patients with moderate-to-severe acne and dry eye.

19.
Opt Express ; 21(14): 16670-82, 2013 Jul 15.
Article in English | MEDLINE | ID: mdl-23938519

ABSTRACT

Indium-tin-oxide (ITO) nanorods (NRs) and nanowhiskers (NWhs) were fabricated by an electron-beam glancing-angle deposition (GLAD) system. These nanomaterials are of interests as transparent conducting electrodes in various devices. Two terahertz (THz) time-domain spectrometers (TDS) with combined spectral coverage from 0.15 to 9.00 THz were used. These allow accurate determination of the optical and electrical properties of such ITO nanomaterials in the frequency range from 0.20 to 4.00 THz. Together with Fourier transform infrared spectroscopic (FTIR) measurements, we found that the THz and far-infrared transmittance of these nanomaterials can be as high as 70% up to 15 THz, as opposed to about 9% for sputtered ITO thin films. The complex conductivities of ITO NRs, NWhs as well films are well fitted by the Drude-Smith model. Taking into account that the volume filling factors of both type of nanomaterials are nearly same, mobilities, and DC conductivities of ITO NWhs are higher than those of NRs due to less severe carrier localization effects in the former. On the other hand, mobilities of sputtered ITO thin films are poorer than ITO nanomaterials because of larger concentration of dopant ions in films, which causes stronger carrier scattering. We note further that consideration of the extreme values of Re{σ} and Im{σ} as well the inflection points, which are functions of the carrier scattering time (τ) and the expectation value of cosine of the scattering angle (γ), provide additional criteria for accessing the accuracy of the extraction of electrical parameters of non-Drude-like materials using THz-TDS. Our studies so far indicate ITO NWhs with heights of ~1000 nm show outstanding transmittance and good electrical characteristics for applications such as transparent conducting electrodes of THz Devices.


Subject(s)
Nanoparticles/chemistry , Surface Plasmon Resonance/methods , Tin Compounds/chemistry , Electric Conductivity , Light , Materials Testing , Nanoparticles/radiation effects , Terahertz Radiation , Tin Compounds/radiation effects
20.
Opt Express ; 21(13): 16056-62, 2013 Jul 01.
Article in English | MEDLINE | ID: mdl-23842392

ABSTRACT

We report generation of broadband supercontinuum (SC) by noise-like pulses (NLPs) with a central wavelength of 1070 nm propagating through a long piece of standard single-mode fibers (~100 meters) in normal dispersion region far from the zero-dispersion point. Theoretical simulations indicate that the physical mechanism of SC generation is due to nonlinear effects in fibers. The cascaded Raman scattering is responsible for significant spectral broadening in the longer wavelength regions whereas the Kerr effect results in smoothing of SC generated spectrum. The SC exhibits low threshold (43 nJ) and a flat spectrum over 1050-1250 nm.

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