[Type of CEBPA mutations in acute myeloid leukemia and their effect on prognosis].

Objective: To demonstrate the type of CEBPA gene mutations among patients with acute myeloid leukemia (AML), clinical characteristics, and prognostic effect on patient outcomes. Methods: Demographic data, clinical features, laboratory characteristics, and data about treatment and follow-up of 57 patients with CEBPA mutated AML diagnosed at Peking Union Medical College Hospital between April 2016 and November 2022 were collected and analyzed. Results: In total, 57 patients with CEBPA mutation accounted for 16.1% of all the 353 patients with AML, among which 28 patients had CEBPA-bZIPinf and 29 had CEBPA-other. Compared with the CEBPA-other group, the CEBPA-bZIPinf group was younger (54 vs 64 years, P=0.010), de novo AML was more common (P=0.001), and the level of bone marrow blast was higher (68.0% vs 36.3%, P=0.001). Moreover, 24 patients from the CEBPA-bZIPinf group and 19 from the CEBPA-other group received chemotherapy. The one-course complete remission (CR) rate of the CEBPA-bZIPinf group was significantly higher than that of the CEBPA-other (87.5% vs 47.4%, P=0.010) and CEBPA-wt (87.5% vs 50.3%, P=0.002) groups. After a median follow-up of 11 months, the median OS of the CEBPA-bZIPinf group was significantly longer than that of the CEBPA-wt group (not reached vs 22.1 months, P=0.012) . Conclusion: CEBPA-bZIPinf mutated AML is a unique clinical entity, with a younger age of diagnosis, better response to chemotherapy, and better prognosis.

[Primary nasopharyngeal carcinoma with Sjögren's syndrome and positive cerebrospinal fluid Epstein-Barr virus: a case report and literature review].

The study presents an analysis of the diagnostic and treatment protocol for a patient with a first episode of nasopharyngeal carcinoma who also has Sjogren's syndrome and Epstein-Barr Virus (EBV) positive cerebrospinal fluid, as detected through metagenomic next-generation sequencing (mNGS). It reviews existing literature to examine the connections between EBV and various conditions including Sjogren's syndrome, encephalitis or meningitis, and nasopharyngeal carcinoma, emphasizing the importance of EBV positive cerebrospinal fluid. The study focuses on a case from the Eighth Medical Center of the General Hospital of the People's Liberation Army, where a patient was admitted with headaches as the primary symptom on March 3, 2021. This patient had a history of Sjogren's syndrome and was later diagnosed with nasopharyngeal carcinoma. The research involved reviewing both domestic and international databases for cases related to cerebrospinal fluid EBV positive encephalitis or meningitis, and nasopharyngeal carcinoma. It aimed to aggregate data on demographics, initial symptoms, treatment methods, and patient outcomes. Findings suggest that positive cerebrospinal fluid EBV is linked to autoimmune diseases, viral encephalitis or meningitis, and nasopharyngeal carcinoma, albeit infrequently in the context of Sjogren's syndrome. Notably, EBV positive cerebrospinal fluid is commonly associated with recurrent nasopharyngeal carcinoma rather than initial episodes. The study concludes that for patients with an immune condition, exhibiting symptoms like headaches or cranial nerve issues, or in cases where nasopharyngeal carcinoma is suspected, early testing through cerebrospinal fluid mNGS or EBV DNA is recommended. This approach facilitates risk assessment, prognosis determination, and the creation of individualized treatment plans.

[Advances in non-invasive treatment of aortic diseases].

With the further development and long-term follow-up of endovascular treatment for aortic diseases, increasing evidence shows that in many cases, there are difficulties in the diagnosis of causes, decision-making of treatment timing, and lack of effective evaluation of treatment prognosis in endovascular treatments. Therefore, it is necessary to conduct in-depth research on non-invasive treatment including prevention, diagnosis, treatment, and prediction of aortic diseases. The non-invasive treatment of aortic disease is mainly applied to high-risk populations with aortic dissection, regulating key targets and mechanisms, and adopting drug intervention in advance to achieve the goal of controlling aortic dilation and preventing the occurrence of dissection. It also conducts precise multi omics analysis to determine the optimal intervention timing and treatment strategy, and aims at complications related to aortic disease or endovascular treatment for patients with a positive family history of aortic dilation and those who have developed aortic dissection. Precise regulation can control the progression of aortic aneurysm and aortic dissection, delay or achieve long-term stable coexistence with aortic disease, and even reverse disease progression and achieve benign aortic remodeling through new intervention vectors. Ultimately achieving the ideal state of complete thrombosis and mechanized healing of the aortic aneurysm or aortic dissection false lumen.

[Clinical study of the efficacies of ruxolitinib plus low-dose PTCY for acute GVHD prevention after haploidentical transplantation in malignant hematological diseases].

Objective: To investigate and verify a novel acute graft versus host disease (aGVHD) prevention protocol in the context of haploidentical hematopoietic stem cell transplantation (haplo-HSCT) . Methods: Patients who underwent haplo-HSCT in our center between January 2022 and December 2022 were included. All patients received reduced doses of cyclophosphamide, Rabbit anti-human tymoglobulin, ruxolitinib, methotrexate, cyclosporine, and MMF to prevent aGVHD. The transplantation outcomes, complications, and survival rate of all patients were investigated. Results: A total of 52 patients with haplo-HSCT were enrolled, 29 (55.8%) male and 23 (44.2%) female, with a median age of 28 (5-59) years. There were 25 cases of acute myeloid leukemia, 17 cases of acute lymphocyte leukemia, 6 cases of myelodysplastic syndrome, 2 cases of chronic myeloid leukemia and 2 cases of myeloproliferative neoplasms. 98.1% of patients had successful engraftment. The incidence of Ⅱ-Ⅳ aGVHD and Ⅲ-Ⅳ aGVHD was 19.2% (95% CI 8.2% -30.3% ) and 7.7% (95% CI 0.2% -15.2% ), respectively. No patients experienced severe gastrointestinal mucositis. The Epstein-Barr virus and CMV reactivation rates were 40.4% and 21.3%, respectively. 9.6% of patients relapsed during followup, with 1-year overall survival, progression-free survival, and non-relapse mortality rates of 86.5% (95% CI 76.9% -96.1% ), 78.8% (95% CI 67.4% -90.3% ) and 11.5% (95% CI 2.6% -20.5% ), respectively. Conclusion: Ruxolitinib combined with a low dose of PTCY is a safe and effective first-line aGVHD prevention strategy.

[Adeno-associated virus-mediated hepatocyte-specific NDUFA13 overexpression protects against CCl4-induced liver fibrosis in mice by inhibiting hepatic NLRP3 activation].

OBJECTIVE: To investigate the protective effect of NDUFA13 protein against acute liver injury and liver fibrosis in mice and explore the possible mechanisms. METHODS: BALB/C mice (7 to 8 weeks old) were divided into normal group, CCl4 group, CCl4+AAV-NC group and CCl4+AAV-NDU13 group (n=18). Mouse models of liver fibrosis were established by intraperitoneal injection of CCl4 twice a week for 3, 5 or 7 weeks, and the recombinant virus AAV8-TBG-NC or AAV8-TBG-NDUFA13 was injected via the tail vein 7-10 days prior to CCl4 injection. After the treatments, pathological changes in the liver of the mice were observed using HE and Masson staining. Hepatic expression levels of NDUFA13 and α-SMA were detected with Western blotting, and the coexpression of NDUFA13 and NLRP3, TNF-α and IL-1ß, and α-SMA and collagen Ⅲ was analyzed with immunofluorescence assay. RESULTS: HE and Masson staining showed deranged liver architecture, necrotic hepatocytes and obvious inflammatory infiltration and collagen fiber deposition in mice with CCl4 injection (P < 0.001). NDUFA13 expression markedly decreased in CCl4-treated mice (P < 0.001), while a significant reduction in inflammatory aggregation and fibrosis was observed in mice with AAV-mediated NDUFA13 overexpression (P < 0.001). In CCl4+AAV-NDU13 group, immunofluorescence assay revealed markedly weakened activation of NLRP3 inflammasomes (P < 0.001), significantly decreased TNF-α and IL-1ß secretion (P < 0.001), and inhibited hepatic stellate cell activation (P < 0.05) and collagen formation in the liver (P < 0.001). CONCLUSION: Mitochondrial NDUFA13 overexpression in hepatocytes protects against CCl4- induced liver fibrosis in mice by inhibiting activation of NLRP3 signaling.

[Determination of Perchloroethylene in blood by headspace gas chromatography-mass spectrometry].

Objective: To establish a method for determination of Perchloroethylene (PCE) in blood by headspace gas chromatography-mass spectrometry (HS/GC-MS) . Methods: From Dctober to December 2021, A total of 3 mL blood samples were taken into a 10 mL headspace bottle, after heated at 60 ℃ for 30 mins, PCE in the top air was separated by VF-WAXms capillary column and detected by GC-MS. The retention time and external standard method were used for qualitative and quantitative analysis of PCE in samples, respectively. Results: There was good linear relationship in the range of 5.09-200.17 µg/L. The linear correlation coefficient was 0.9993.The detection limit was 0.21 µg/L and the lower limit of quantitation was 0.70 µg/L. The recovery rates of samples with different concentrations were 95.3%-103.8%. The intra-batch relative standard deviations (RSD) were 3.2%-4.6%, and inter-batch RSD was 4.0%-6.1%. The samples can be stored at 4 ℃ for three days and at -20 ℃ for seven days. Conclusion: This method is proved to be simple, practical and highly sensitive, which is suitable for the determination of PCE in blood.

[Diagnosis and orthodontic treatment strategy of complete canine transposition].

Tooth transposition is a challenge for orthodontists, especially in correcting the order of teeth. At present, the literature on transposition canines mainly focuses on epidemiological studies and case reports, and no systematic treatment guidance has been formed. In this article, the definition and classification, epidemiology and etiology, imaging diagnosis, treatment and risk control of transposed canines are systematically described in order to provide reference for clinical practice.

[Plurihormonal PIT1-lineage pituitary neuroendocrine tumors: a clinicopathological study].

Objective: To investigate the clinicopathological characteristics of plurihormonal PIT1-lineage pituitary neuroendocrine tumors. Methods: Forty-eight plurihormonal PIT1-lineage tumors were collected between January 2018 and April 2022 from the pathological database of Sanbo Brain Hospital, Capital Medical University. The related clinical and imaging data were retrieved. H&E, immunohistochemical and special stains were performed. Results: Out of the 48 plurihormonal PIT1-lineage tumors included, 13 cases were mature PIT1-lineage tumors and 35 cases were immature PIT1-lineage tumors. There were some obvious clinicopathological differences between the two groups. Clinically, the mature plurihormonal PIT1-lineage tumor mostly had endocrine symptoms due to increased hormone production, while a small number of immature PIT1-lineage tumors had endocrine symptoms accompanied by low-level increased serum pituitary hormone; patients with the immature PIT1-lineage tumors were younger than the mature PIT1-lineage tumors; the immature PIT1-lineage tumors were larger in size and more likely invasive in imaging. Histopathologically, the mature PIT1-lineage tumors were composed of large eosinophilic cells with high proportion of growth hormone expression, while the immature PIT1-lineage tumors consisted of chromophobe cells with a relatively higher expression of prolactin; the mature PIT1-lineage tumors had consistently diffuse cytoplasmic positive staining for keratin, while the immature PIT1-lineage tumors had various expression for keratin; the immature PIT1-lineage tumors showed more mitotic figures and higher Ki-67 proliferation index; in addition, 25.0% (12/48) of PIT1-positive plurihormonal tumors showed abnormal positive staining for gonadotropin hormones. There was no significant difference in the progression-free survival between the two groups (P=0.648) by Kaplan-Meier analysis. Conclusions: Plurihormonal PIT1-lineage tumor belongs to a rare type of PIT1-lineage pituitary neuroendocrine tumors, most of which are of immature lineage. Clinically increased symptoms owing to pituitary hormone secretion, histopathologically increased number of eosinophilic tumor cells with high proportion of growth hormone expression, diffusely cytoplasmic keratin staining and low proliferative activity can help differentiate the mature plurihormonal PIT1-lineage tumors from the immature PIT1-lineage tumors. The immature PIT1-lineage tumors have more complicated clinicopathological characteristics.

A patient with spinal cavernous vascular malformation: case report and review of the literature.

BACKGROUND: Spinal cavernous vascular malformation (SCM) is a rare type of spinal vascular malformation that can be easily misdiagnosed and overlooked, accounting for 5%-12% of all spinal vascular malformations. To date, surgical resection has been the gold standard for treating SCM, particularly in symptomatic patients. The risk of secondary hemorrhage in SCM is as high as 66%. Therefore, early, timely, and accurate diagnosis is crucial for patients with SCM. CASE REPORT: In this report, we describe a 50-year-old female patient who was admitted to the hospital with recurrent bilateral lower extremity pain and numbness for 10 years, with recurring symptoms for 4 months. The patient's symptoms initially improved after conservative treatment but then worsened again. An MRI revealed a spinal cord hemorrhage, and after surgical treatment, the patient's symptoms improved significantly. A postoperative pathological examination confirmed the diagnosis of SCM. CONCLUSIONS: This case, along with a review of the literature, suggests that for SCM, early surgery using techniques such as microsurgery and intraoperative evoked potential monitoring may result in better outcomes for the patient.

An mHealth-based school health education system designed to scale up salt reduction in China (EduSaltS): A development and preliminary implementation study.

Background: High-salt diet is an important risk factor for several non-communicable diseases. School-based health education has been found effective in reducing salt intake among children and their families in China. However, no such interventions have been scaled up in the real world. For this purpose, a study was launched to support the development and scale-up of an mHealth-based system (EduSaltS) that integrated routine health education and salt reduction and was delivered through primary schools. This study aims to elaborate the framework, development process, features, and preliminary scaling-up of the EduSaltS system. Methods: The EduSaltS system evolved from previously successfully tested interventions to reduce family salt intake by empowering schoolchildren through school health education. EduSaltS was designed by following the WHO's conceptual framework for developing a scaling-up strategy which accounted for the nature of the innovation, the capacity of the implementing organizations, the characteristics of the environment, the resources available, and type of scaling up. The system was then developed step by step from determination of online platform architecture, definition of component interventions and activities, development of specific educational materials and tools, to the development of the online/offline hybridized system. The system was tested and refined by a pilot in two schools and a preliminary scale-up in two cities in China. Results: EduSaltS was developed as an innovative health education system, including an online WeChat-based education platform, a set of offline activities, and an actual administrative website showing the progress and setting the system. The WeChat platform could be installed on users' smartphones to automatically deliver 20 sessions of five-minute well-structured cartoon video classes, followed by other online interactive activities. It also helps support project implementation and real-time performance evaluation. As a first-stage roll-out, a one-year course has been successfully implemented among 54,538 children and their families from 209 schools in two cities, and the average course completion rate was 89.1%. Conclusion: As an innovative mHealth-based health education system, EduSaltS was developed based on successfully tested interventions and an appropriate framework for scaling up. The early-stage roll-out has shown its preliminary scalability, and further evaluation is ongoing.

Comprehensive workplace intervention for cancer prevention in China (WECAN): protocol for a stepped-wedge, cluster-randomised controlled trial.

INTRODUCTION: Cancer is the second leading cause of death across the globe with the majority of deaths occurring in low-income and middle-income countries. Evidence has shown that the cancer burden can be substantially reduced by avoiding behavioural risk factors through comprehensive intervention strategies, including workplace health promotion, which has shown to be cost-effective in developed countries while rarely conducted in developing countries. This study aims to explore a feasible and sustainable approach to the prevention and control of cancer in China by developing an evidence-based comprehensive workplace health model equipped with a smartphone application for implementation. METHODS AND ANALYSIS: This study is designed as a stepped-wedge, cluster-randomised controlled trial. We will recruit 15 workplaces from three cities in China. A total of 750 employees will be randomly selected for evaluation that includes five rounds of survey conducted every 6 months. After the second evaluation, workplaces will be randomly allocated to start the intervention sequentially every 6 months in three steps with five workplaces per step. A mobile application 'Healthy Workplace' will be developed to support the intervention. On-line and off-line health-related activities will be carried out among employees. Employers will provide supportive policies, environment and benefits to facilitate the adoption of healthy behaviours. The primary outcome is the change of Healthy Lifestyle Index Score, which consists of five components including smoking, alcohol drinking, physical activity, diet and body mass index. ETHICS AND DISSEMINATION: The study has been approved by Queen Mary University of London Ethics of Research Committee (QMERC22.257) and Chinese Centre for Disease Control and Prevention Institutional Review Board (202210). Written informed consent is required from all participants. Results will be disseminated through presentations, publications and social media. TRIAL REGISTRATION NUMBER: ChiCTR2200058680.

[Reverse partial pulmonary resection: a new surgical approach for pediatric pulmonary cysts].

OBJECTIVE: To evaluate the safety and efficacy of reverse partial lung resection for treatment of pediatric pulmonary cysts combined with lung abscesses or thoracic abscess. METHODS: We retrospectively analyzed the clinical data of children undergoing reverse partial lung resection for complex pulmonary cysts in our hospital between June, 2020 and June, 2021.During the surgery, the patients lay in a lateral position, and a 3-5 cm intercostal incision was made at the center of the lesion, through which the pleura was incised and the fluid or necrotic tissues were removed.The anesthesiologist was instructed to aspirate the sputum in the trachea to prevent entry of the necrotic tissues in the trachea.The cystic lung tissue was separated till reaching normal lung tissue on the hilar side.The proximal end of the striated tissue in the lesion was first double ligated with No.4 silk thread, the distal end was disconnected, and the proximal end was reinforced with continuous sutures with 4-0 Prolene thread.The compromised lung tissues were separated, and the thoracic cavity was thoroughly flushed followed by pulmonary inflation, air leakage management and incision suture. RESULTS: Sixteen children aged from 3 day to 2 years underwent the surgery, including 3 with simple pulmonary cysts, 11 with pulmonary cysts combined with pulmonary or thoracic abscess, 1 with pulmonary cysts combined with tension pneumothorax and left upper lung bronchial defect, and 1 with pulmonary herpes combined with brain tissue heterotaxy.All the operations were completed smoothly, with a mean operation time of 129 min, an mean hospital stay of 11 days, and a mean drainage removal time of 7 days.All the children recovered well after the operation, and 11 of them had mild air leakage.None of the children had serious complications or residual lesions or experienced recurrence of infection after the operation. CONCLUSION: Reverse partial lung resection is safe and less invasive for treatment of complex pediatric pulmonary cysts complicated by infections.

A computational model for the transit of a cancer cell through a constricted microchannel.

We propose a three-dimensional computational model to simulate the transient deformation of suspended cancer cells flowing through a constricted microchannel. We model the cell as a liquid droplet enclosed by a viscoelastic membrane, and its nucleus as a smaller stiffer capsule. The cell deformation and its interaction with the suspending fluid are solved through a well-tested immersed boundary lattice Boltzmann method. To identify a minimal mechanical model that can quantitatively predict the transient cell deformation in a constricted channel, we conduct extensive parametric studies of the effects of the rheology of the cell membrane, cytoplasm and nucleus and compare the results with a recent experiment conducted on human leukaemia cells. We find that excellent agreement with the experiment can be achieved by employing a viscoelastic cell membrane model with the membrane viscosity depending on its mode of deformation (shear versus elongation). The cell nucleus limits the overall deformation of the whole cell, and its effect increases with the nucleus size. The present computational model may be used to guide the design of microfluidic devices to sort cancer cells, or to inversely infer cell mechanical properties from their flow-induced deformation.

Estimated Dietary and Health Impact of the World Health Organization's Global Sodium Benchmarks on Packaged Foods in Australia: a Modeling Study.

BACKGROUND: In 2021, the World Health Organization (WHO) set sodium benchmarks for packaged foods to guide countries in setting feasible and effective sodium reformulation programs. We modeled the dietary and health impact of full compliance with the WHO's sodium benchmarks in Australia and compared it to the potential impact of Australia's 2020 sodium reformulation targets. METHODS: We used nationally representative data on food and sodium intake, sodium levels in packaged foods, and food sales volume to estimate sodium intake pre- and post-implementation of the WHO and Australia's sodium benchmarks for 24 age-sex groups. Using comparative risk assessment models, we then estimated the potential deaths, incidence, and disability-adjusted life years averted from cardiovascular disease, chronic kidney disease, and stomach cancer based on the reductions in sodium intake. RESULTS: Compliance with the WHO's sodium benchmarks for packaged foods in Australia could lower mean adult sodium intake by 404 mg/day, corresponding to a 12% reduction. This could prevent about 1770 deaths/year (95% uncertainty interval 1168-2587), corresponding to 3% of all cardiovascular disease, chronic kidney disease, and stomach cancer deaths in Australia, and prevent some 6900 (4603-9513) new cases, and 25 700 (17 655-35 796) disability-adjusted life years/year. Compared with Australian targets, the WHO benchmarks will avert around 3 and a half times more deaths each year (1770 versus 510). CONCLUSIONS: Substantially greater health impact could be achieved if the Australian government strengthened its current sodium reformulation program by adopting WHO's more stringent and comprehensive sodium benchmarks.

Microarray profiling identifies hsa_circ_0082003 as a novel tumor promoter for papillary thyroid carcinoma.

BACKGROUND: Circular RNAs (circRNAs) are non-coding RNAs that have essential regulatory roles in the development of various tumors. This study explored whether circRNAs are involved in the progression of papillary thyroid carcinoma (PTC). METHODS: Differentially expressed circRNAs (DECs) in four pairs of PTC and matched normal thyroid tissues were screened using a circRNA microarray. The potential functions of dysregulated circRNAs were predicted by bioinformatic analyses. Reverse transcription quantitative polymerase chain reaction (RT-qPCR) was used to determine hsa_circ_0082003 expression in 80 pairs of PTC and matched normal thyroid tissues. Cell counting kit-8, colony formation, wound healing, and Transwell assays were performed to evaluate the biological functions of hsa_circ_0082003 in PTC cells. The role of hsa_circ_0082003 in PTC tumorigenesis in vivo was validated in nude mice. RESULTS: In total, 3150 DECs (2317 upregulated and 833 downregulated) were identified. Pathway enrichment analyses indicated that the dysregulated circRNAs may play roles in PTC development. RT-qPCR validation demonstrated that hsa_circ_0082003 expression was significantly increased in PTC tissues and correlated with poor clinicopathological parameters. Receiver operating characteristic curve analysis showed that hsa_circ_0082003 had good performance for diagnosing PTC and judging whether it was accompanied by lymph node metastasis. Knockdown of hsa_circ_0082003 inhibited PTC cell proliferation, migration, and invasion. Tumor formation assays in vivo showed that downregulation of hsa_circ_0082003 significantly suppressed the growth of PTC. CONCLUSION: Hsa_circ_0082003 may serve as a novel diagnostic biomarker and potential therapeutic target for PTC.

[Clinicopathological characteristics of H3K27-altered diffuse midline glioma and evaluation of NTRK as its therapeutic target].

Objective: To investigate the clinicopathological characteristics of H3K27-altered diffuse midline glioma (DMG), and to analyze DMG's prognostic factors, and subsequently, to study the possibility of using NTRK as a therapeutic target for DMG. Methods: A total of 232 DMG diagnosed at the Sanbo Brain Hospital, Capital Medical University, Beijing, China from July 2016 to March 2021 were collected. Their clinical, radiological and pathological features, the ratio of MGMT promoter methylation, expression of NTRK, and characteristics of NTRK gene fusion were analyzed. The prognostic values of different factors were also studied, including age, tumor location, histological grade, gene and protein expression of NTRK, and postoperative adjuvant therapy. Results: Among the 232 DMG cases, there were 8 patients with both primary and relapse tumors on the record. Thus, a total of 224 patients were analyzed, including 118 males and 106 females. There were 126 adults (>18 years of age) and 98 children (≤18 years of age). Notably, the most frequent location was thalamus (41/126, 32.5%) in adults, but brainstem (59/96, 60.2%) in children. The lesions showed T1 hypointensity or isointensity, and T2 hyperintensity. However, contrast enhancement patterns of the tumors varied, with many tumors lacking contrast-enhancing. The histological grades included grade 2 (9/224, 4.0%), grade 3 (41/224, 18.3%) and grade 4 (174/224, 77.7%). Two hundred and twenty-four DMGs were diffusely positive for H3K27M and negative for H3K27me3. The ratio of MGMT promoter methylation was low (1/45, 2.2%). One hundred and seventy-seven of the 224 cases (177/224, 79.0%) were positive for NTRK. Fifty cases were analyzed using fluorescence in situ hybridization. Among them, five DMGs (positive rate, 10.0%) were NTRK fusion positive. This study showed that there were no differences between adult and pediatric DMGs in histological grading, expression of NTRK, and NTRK gene fusion. One hundred and fifty-nine patients were included in the follow-up analysis (P>0.05). During the follow-up period, 109/159 patients (69.6%) died of the disease, with a median survival time of 12 months (range 1 to 55 months). Univariate log-rank analysis showed that age, location, surgical procedure and postoperative adjuvant therapy were associated with overall survivals of the DMG patients (P<0.05). Conclusions: The prognosis of DMG is poor overall. There are differences between adult and pediatric DMGs in anatomic location and prognosis, but not in other features. NTRK1 gene fusion is detected in 10.0% of the tumors. It suggests that TRK inhibitor might be a choice for treating DMG.

[Prognostic value of translocation t(11;14) in primary light-chain amyloidosis treated with bortezomib-based regimen].

Objective: To investigate the prognostic value of translocation t(11;14) in newly-diagnosed primary light-chain (AL) amyloidosis patients treated with bortezomib-based regimen. Method: Clinical information of newly-diagnosed AL amyloidosis patients in Peking Union Medical College Hospital who had baseline t(11;14) data and accepted bortezomib-combined therapies from September, 2015 to September, 2021 was collected. The relationships between t(11;14) status and baseline characteristics, hematological response, organ response and prognosis were analyzed. Results: A total of 152 patients were included, aged (59.5±9.1) years and 93 cases were male (61.2%). Forty-six patients carried t(11;14) (30.3%). There was no statistical difference in the proportion of organ involved, distribution of Mayo 2004 and 2012 stages and laboratory indexes between patients with and without t(11;14) (all P>0.05). For hematological response, the difference in the rates of ≥very good partial response (VGPR) between those with t(11;14) and without after the first cycle [28.2%(11/39) vs 37.4%(34/91), P>0.05] was not statistically significant. After 3 cycles, the difference in the rates of ≥VGPR between two groups was not statistically significant [35.9%(14/39) vs 51.1%(46/90), P>0.05]. The difference in the ratio of the best hematological response reaching ≥VGPR between two groups during the first-line treatment was not statistically significant [52.2%(24/46) vs 64.2%(68/106), P>0.05]. But patients with t(11;14) had lower cardiac response rate at 3 months [15.2%(5/33) vs 34.6%(28/81), P=0.038] and 6 months [19.4%(6/31) vs 50.6%(42/83),P=0.003] than those without, but the difference in cardiac response rates at 12 months was not statistically significant [41.7%(10/24) vs 53.5%(38/71),P>0.05]. For survival, the differences in overall survival (not reached vs 50.1 months, P>0.05) and hematological event-free survival (36.2 months vs 39.9 months, P>0.05) between patients carrying t(11;14) and those without were not statistically significant. Conclusion: Patients with t(11;14) had lower cardiac response rate than those without, but their hematological response and survival are not significantly different from those free from t(11;14).