Objective:To investigate clinical and laboratory characteristics of secondary hemophagocytic lymphohistiocytosis (sHLH) associated with secondary cutaneous T-cell lymphoma (CTCL) .Methods:CTCL patients with clinically suspected sHLH were collected from Department of Hematology, Wuhan No.1 Hospital from January 2016 to October 2021, and were evaluated according to the HLH-2004 diagnostic criteria and HScore.Results:Seven CTCL patients were confirmedly diagnosed with sHLH, including 2 with primary cutaneous γδT-cell lymphoma (PC-GDTCL) , 3 with cutaneous extranodal natural killer/T-cell lymphoma (C-ENKTCL) , and 2 with primary cutaneous anaplastic large cell lymphoma (PC-ALCL) . All the 7 patients received chemotherapy, but 6 died finally, and the median overall survival duration was 26.5 days (range: 14 - 60 days) after the confirmed diagnosis of CTCL complicated by sHLH. HLH-related gene mutations, which were located in the PRF1 and LYST genes, were identified in 2 patients; lymphoma-related gene mutations were identified in the KRAS and KMT2D genes in 1 PC-GDTCL patient,and in the JAK3 and SAMHD1 genes in another PC-GDTCL patient.Conclusions:CTCL complicated by sHLH usually progresses rapidly, so early diagnosis and treatment are needed. Bone marrow biopsy and mutation screening of lymphoma- and HLH-related genes at initial diagnosis and during disease progression may facilitate early diagnosis.

Objective:To develop an online interactive cytopathological training program, and to evaluate it for improving the cytopathological diagnostic ability of endoscopists in endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) of pancreas.Methods:A total of 5 500 cytopathological images were collected from 194 patients with pancreatic solid mass who underwent EUS-FNA in Nanjing Drum Tower Hospital from August 2018 to August 2019. The cell type in each cytopathological picture was labeled by senior cellular pathologists, which was used to build a learning and testing platform for online interactive cytopathological training. Five endoscopists without cytopathological background were invited to participate in this training. Sensitivity, specificity, positive predictive value and negative predictive value of endoscopists in differential diagnosis of cancer and non-cancer before and after training were compared to evaluate the effect of the online interactive cytopathological training program on improving the ability of endoscopists in diagnosis of cytopathology.Results:A cytopathological training platform for endoscopists to learn and take online test was successfully built. Before training, sensitivity, specificity, positive predictive value, negative predictive value and accuracy of diagnosis of cancer and non-cancer for endoscopists were 0.55 (95% CI: 0.53-0.58), 0.32 (95% CI: 0.30-0.35), 0.43 (95% CI: 0.41-0.45), 0.44 (95% CI: 0.41-0.47) and 0.43 (95% CI: 0.42-0.45), respectively. After training, the above indicators were 0.96 (95% CI: 0.95-0.97), 0.70 (95% CI: 0.68-0.73), 0.74 (95% CI: 0.72-0.76), 0.95 (95% CI: 0.94-0.96) and 0.81 (95% CI: 0.80-0.83), respectively, which were significantly improved compared with those before ( P<0.001). Conclusion:The online interactive cytopathological training program can improve the understanding and diagnostic ability of endoscopists in pancreatic cytopathology, help to implement rapid on-site evaluation in the process of EUS-FNA, and improve the diagnostic efficiency of EUS-FNA.

The main clinical phenotypes, imaging features and genetic test results of a child with Joubert syndrome treated in Shenzhen Children′s Hospital in July 2020 were analyzed retrospectively, and the literature on Joubert syndrome was summarized.The main manifestations of the protester during infancy were respiratory abnormalities and developmental retardation.The brain magnetic resonance imaging (MRI) showed a " molar sign" , which was consistent with the diagnosis of Joubert syndrome.Genetic testing suggested that the protestor carried complex heterozygous variations of KIAA0586 gene.Two variants were not reported previously, one of which was synonymous mutation.The child is the first case of Joubert syndrome caused by KIAA0586 gene in China.Joubert syndrome is a rare congenital brain development malformation characterized by high clinical heterogeneity and MRI molar signs.It may involve multiple systems.Early identification and intervention can improve outcomes.

Objective:To analyze the risk factors of bronchopulmonary dysplasia(BPD)in very preterm infants(VPI), and to provide scientific basis for the prevention and treatment of BPD in VPI.Methods:A prospective multicenter study was designed to collect the clinical data of VPI in department of neonatology of 28 hospitals in 7 regions from September 2019 to December 2020.According to the continuous oxygen dependence at 28 days after birth, VPI were divided into non BPD group and BPD group, and the risk factors of BPD in VPI were analyzed.Results:A total of 2 514 cases of VPI including 1 364 cases without BPD and 1 150 cases with BPD were enrolled.The incidence of BPD was 45.7%.The smaller the gestational age and weight, the higher the incidence of BPD( P<0.001). Compared with non BPD group, the average birth age, weight and cesarean section rate in BPD group were lower, and the incidence of male infants, small for gestational age and 5-minute apgar score≤7 were higher( P<0.01). In BPD group, the incidences of neonatal respiratory distress syndrome(NRDS), hemodynamically significant patent ductus arteriosus, retinopathy of prematurity, feeding intolerance, extrauterine growth restriction, grade Ⅲ~Ⅳ intracranial hemorrhage, anemia, early-onset and late-onset sepsis, nosocomial infection, parenteral nutrition-associated cholestasis were higher( P<0.05), the use of pulmonary surfactant(PS), postnatal hormone exposure, anemia and blood transfusion were also higher, and the time of invasive and non-invasive mechanical ventilation, oxygen use and total hospital stay were longer( P<0.001). The time of starting enteral nutrition, cumulative fasting days, days of reaching total enteral nutrition, days of continuous parenteral nutrition, days of reaching 110 kcal/(kg·d) total calorie, days of reaching 110 kcal/(kg·d) oral calorie were longer and the breastfeeding rate was lower in BPD group than those in non BPD group( P<0.001). The cumulative doses of amino acid and fat emulsion during the first week of hospitalization were higher in BPD group( P<0.001). Multivariate Logistic regression analysis showed that NRDS, invasive mechanical ventilation, age of reaching total enteral nutrition, anemia and blood transfusion were the independent risk factors for BPD in VPI, and older gestational age was the protective factor for BPD. Conclusion:Strengthening perinatal management, avoiding premature delivery and severe NRDS, shortening the time of invasive mechanical ventilation, paying attention to enteral nutrition management, reaching whole intestinal feeding as soon as possible, and strictly mastering the indications of blood transfusion are very important to reduce the incidence of BPD in VPI.

【Objective】 To clarify the hematological characteristics and current situation of chronic mountain sickness among Tibetan residents in extreme high altitude area (more than 5 000 m above the sea level) of Ali district based on the analysis of physical examination and blood test results. 【Methods】 Totally 250 Tibetan residents were selected by convenient sampling for blood oxygen saturation (SpO2), heart rate, and blood routine examination. Chronic mountain sickness was determined according to the hemoglobin (Hb) level and SpO2. 【Results】 The red blood cell (RBC), Hb and hematocrit (HCT) of the Tibetan residents at 5 200-meter altitude were all higher than the normal physiological reference range of China. Mean red blood cell volume (MCV), mean red blood cell hemoglobin content (MCH), mean red blood cell hemoglobin concentration (MCHC), white blood cell (WBC) and platelet (PLT) were in the upper limit of the reference value. The RBC, Hb, HCT and MCHC of male Tibetan residents were higher than those of females, while PLT was lower than that of females, with significant differences. There were no statistical differences in MCV, MCH or WBC among different genders of Tibetan residents. The SpO2 of the Tibetan residents was about 85% of the normal value, and the males had higher SpO2 than the females in the same age group, and the difference was statistically significant, but the heart rate did not differ significantly. The prevalence rate of chronic mountain sickness in this area was as high as 16.4%, and the prevalence rate of heavy manual workers was significantly higher than that of light manual workers, with significant differences. 【Conclusion】 The high-altitude anoxic environment causes the changes in red blood cells, hemoglobin, and oxygen saturation of local residents, and the prevalence of chronic mountain sickness increases significantly. Labor intensity is one of the risk factors for chronic mountain sickness in high-altitude areas.

【Objective】 To demonstrate the real incidence and clinical features of acute transfusion reactions (ATRs) and analyze its association with transfusion effectiveness. 【Methods】 The blood transfusion process of patients in the Hematology Department in our hospital from March 16, 2018 to March 16, 2019 was observed and followed up. The vital signs and clinical features were recorded, the clinical data and relevant laboratory examination results were collected, and the correlation between ATRs, clinical features and transfusion effectiveness was statistically analyzed. 【Results】 A total of 2500 transfusions were observed in the Hematology Department, out of which 138 patients developed 242 ATRs. The overall incidence of ATRs was 9.68% (95% CI, 8.52%~10.94%) and the incidence of ATRs during platelets transfusion was the highest. The clinical features of most ATRs were mild and no fatal consequences occurred. No significant difference was found in the association between transfusion effectiveness with ATRs in platelets and red blood cells transfusion (P>0.05). 【Conclusion】 None of the ATRs observed in this study resulted in serious consequences or had effect on the transfusion effectiveness. The characteristics presented in these reactions may provide certain references for clinical practice.

Objective To determine the diagnostic value of endoscopic ultrasound-guided fine needle aspiration ( EUS-FNA ) for gastrointestinal lesions with inconclusive endoscopic biopsies. Methods A retrospective analysis was performed in 65 patients who were found to have gastrointestinal lesions with inconclusive endoscopic biopsies and underwent EUS-FNA in Drum Tower Hospital. Diagnostic value of EUS-FNA was determined by comparing with surgical histopathology and follow-up results. Results This study included 41 males ( 63％) and 24 females ( 37％) with median age of 60 years. The most common lesion was diffuse infiltrative lesions ( 37, 56. 9％) , followed by submucosal protrusion types ( 17, 26. 2％) . Fifty-four cases ( 83. 1％) were malignant lesions, and 11 cases ( 16. 9％) were benign. The overall sensitivity, specificity, and accuracy of EUS-FNA for gastrointestinal lesions with inconclusive biopsies were 76. 8％ ( 95％CI: 65. 7％-87. 8％) , 100. 0％ ( 95％CI: 66. 4％-100. 0％) , and 80. 0％( 95％CI: 70. 3％-89. 7％) , respectively. Sub-group analysis showed the sensitivity, specificity, and accuracy of EUS-FNA for diffuse infiltrative lesions were 70. 6％ ( 95％CI: 55. 3％-85. 9％, 100. 0％( 95％CI:29. 2％-100. 0％) , and 73. 0％ ( 95％CI: 58. 7％-87. 3％) , respectively. For submucosal protrusions, the sensitivity, specificity, and accuracy of EUS-FNA were 68. 8％ ( 95％CI: 46. 0％ -91. 5％) , 100. 0％ ( 95％CI: 2. 5％-100. 0％) , and 70. 6％ ( 95％CI: 44. 0％-89. 7％) , respectively. Conclusion EUS-FNA has moderate diagnostic value for endoscopic biopsy-inconclusive gastrointestinal lesions. It can be an alternative option when standard methods, such as endoscopic mucosal forceps biopsy, fail to provide a definitive diagnosis.

Objective To evaluate the feasibility and safety of endoscopic ultrasound-guided portal pressure gradient (EUS-PPG) measurement in the normal porcine model.Methods Four pigs,2 male and 2 female,aged 8-12 months,weighing 20-30 kg were selected in the experiment.Under general anesthesia and EUS guidance,a 22 G fine needle connected to electrocardiograph monitor with a central vein pressure manometer was used to puncture and measure pressures in the portal vein (PV) and hepatic vein (HV) or inferior vena cava (IVC).Pressures were measured three times for each vessel and the mean pressure was recorded.The PPG was recorded as the difference between the PV pressure and HV or IVC pressure.Vital signs during and after the procedure and operation-related complications were monitored.Results EUS-PPG measurement was successful in all targeted vessels.The PV pressure,HV or IVC pressure,and PPG was 11.0±1.0 mmHg(1 mmHg=0.133 kPa),7.3±1.1 mmHg and 3.8±0.9 mmHg,respectively.No adverse event occurred.Conclusion EUS-PPG measurement has a high successful rate and reliable accuracy and safety reflecting the portal vein pressure.

Objective: To study the relationship between STAT3 gene (rs2293152, rs957970) polymorphism and susceptibility to bronchial asthma in children, and to lay the foundation for search of the candidate pathogenic gene of asthma and early prevention of the disease. Methods: One hundred and thirty hospitalized asthmatic children or out-patients with asthma at Affiliated Hospital of Southwest Medical University from September 2013 to September 2017 were selected as asthmatic group, and at the same time 145 healthy children were selected from the Affiliated Hospital of Southwest Medical University as the healthy control group.Apply sequence specific primers-polymerase chain reaction (SSP-PCR) was applied to detect susceptibility single nucleotide polymorphism (SNP) of STAT3 gene (rs2293152, rs9579700). The statistical differences in distribution between genotype and allele were analyzed between the asthma group and the healthy control group. Results: The distribution frequency of CC genotype of rs2293152 in STAT3 gene was significantly higher in the asthmatic group (19.2%)than that in the healthy control group (9.0%) (χ²=6.065, P=0.014). C allele was significantly higher in asthmatic group (41.9% vs.33.8%) (χ²=3.861, P=0.049). There was no significant difference in the distribution of rs957970 in STAT3 gene between the asthma group and the healthy control group. Conclusions The SNP of rs2293152 in STAT3 gene is associated with susceptibility to asthma in children in South Sichuan.C allele may be the susceptible gene for childhood asthma, while the SNP of rs957970 in STAT3 gene has no significant correlation with childhood asthma in Southern Sichuan.

Objective: To study the feasibility of establishing human 3D pancreatic cancer organoids with endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) specimen. Methods: A total of 9 patients with suspected pancreatic masses were prospectively included in this study from June 2017 to January 2018. EUS-FNA was performed for initial diagnosis. The biopsy tissues were obtained from a COOK 22-gauge FNA needle for organoid establishment, and the growth status in vitro was observed. Results: Nine specimens of pancreatic lesions obtained from EUS-FNA were enrolled. Successful establishment of organoids was achieved in 5 cases, which were all confirmed to be pancreatic cancer histopathologically. In the process of generating, the growth rate of organoids increased correspondingly. The pathological morphology of these organoids was similar to the corresponding pancreatic tissues in HE staining. Conclusion Pancreatic cancer organoids can be successfully created by means of EUS-FNA. Establishment of these organoids can potentially provide excellent models for patients with pancreatic cancer in guiding precision treatment.