OBJECTIVE: To explore the clinical characteristics and prognostic factors of patients with extranasal NK/T-cell lymphoma (NKTCL). METHODS: The clinical data of 138 patients with NKTCL diagnosed in 10 medical centers of Huaihai Lymphoma Working Group from June 2015 to April 2021 were collected and analyzed retrospectively. The differences in clinicopathological characteristics of patients with different involvement and efficacy of pegaspargase regimen were compared, as well as perform survival analysis. RESULTS: A total of 138 extranasal NKTCL patients were included, with a median age of 46 years, and the ratio of males to females was approximately 2∶1. There were 39 patients with gastrointestinal involvement, 32 patients with oropharyngeal involvement, 17 patients with skin involvement, 11 patients with lymph node involvement, 11 patients with orbital involvement, and 28 patients with other parts involvement. Patients with skin involvement had a higher proportion of advanced disease and a lower proportion of CD56 positive rate compared to those with oropharyngeal involvement. Among the patients with gastrointestinal involvement, the survival rate of patients who received pegaspargase regimen was significantly higher than those who were treated without pegaspargase (P < 0.01). Multivariate analysis showed that serum creatinine was an independent prognostic factor for patients with skin involvement ( HR =1.027, 95%CI : 1.001-1.054, P =0.040), ECOG PS and EBV DNA were independent prognostic factors for patients with gastrointestinal involvement ( HR =2.635, 95%CI : 1.096-6.338, P =0.030; HR =4.772, 95% CI : 1.092-20.854, P =0.038), and ECOG PS and CA stage were independent prognostic factors for patients with oropharyngeal involvement ( HR =13.875, 95%CI : 2.517-76.496, P =0.002; HR =20.261, 95%CI : 2.466-166.470, P =0.005). CONCLUSION The clinicopathological characteristics of extranasal NKTCL patients with different sites of involvement are vary, and effective individualized treatment need to be further explored.
Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Asparaginase/therapeutic use* ; Lymphoma, Extranodal NK-T-Cell/pathology* ; Prognosis ; Retrospective Studies ; Survival Rate ; Polyethylene Glycols

The clinical medicine postdoctoral training program embodies a pioneering initiative in China's pursuit of excellence in medical education in the contemporary era. The establishment of the Department of Rare Diseases at Peking Union Medical College Hospital in 2023 signifies a momentous milestone, making it the nation's inaugural clinical department exclusively dedicated to diagnosis and treatment of patients with rare diseases. Aligned with the Consensus on Core Competency Framework for Residency Education Among China Consortium of Elite Teaching Hospitals for Residency Education, the clinical postdoctoral program in the Department of Rare Diseases underscores a holistic advancement of fundamental competencies within the framework of "three basics and three stricts". This approach is further complemented by the refinement of teaching evaluation and feedback systems. The program aspires to serve as an educational paradigm for cultivating elite talents in the field of rare disease medicine in China and nurture multifaceted talents equipped with exceptional clinical insight and comprehensive capabilities, thereby alleviating the acute shortage of adept professionals in the domain of rare diseases and propelling the field's progression in China.

Objective: To explore the prognostic factors of extracellular NK/T cell lymphoma (ENKTL) treated with pegaspargase/L-asparaginase. Methods: The clinical data of 656 ENKTL patients diagnosed at 11 medical centers in the Huaihai Lymphoma Working Group from March 2014 to April 2021 were retrospectively analyzed. The patients were randomly divided into two groups: a training set (460 cases) and a validation set (196 cases) at 7∶3, and the prognostic factors of the patients were analyzed. A prognostic scoring system was established, and the predictive performance of different models was compared. Results: Patients' median age was 46 (34, 57) years, with 456 males (69.5% ) and 561 nasal involvement (85.5% ). 203 patients (30.9% ) received a chemotherapy regimen based on L-asparaginase combined with anthracyclines, and the 5-year overall survival rate of patients treated with P-GEMOX regimen (pegaspargase+gemcitabine+oxaliplatin) was better than those treated with SMILE regimen (methotrexate+dexamethasone+cyclophosphamide+L-asparaginase+etoposide) (85.9% vs 63.8% ; P=0.004). The results of multivariate analysis showed that gender, CA stage, the Eastern Cooperative Oncology Group performance status (ECOG PS) score, HGB, and EB virus DNA were independent influencing factors for the prognosis of ENKTL patients (P<0.05). In this study, the predictive performance of the prognostic factors is superior to the international prognostic index, Korean prognostic index, and prognostic index of natural killer lymphoma. Conclusion: Gender, CA stage, ECOG PS score, HGB, and EB virus DNA are prognostic factors for ENKTL patients treated with pegaspargase/L-asparaginase.
Male ; Humans ; Middle Aged ; Asparaginase/therapeutic use* ; Prognosis ; Retrospective Studies ; Lymphoma, Extranodal NK-T-Cell/drug therapy* ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Etoposide ; Cyclophosphamide ; Methotrexate/therapeutic use* ; DNA/therapeutic use* ; Treatment Outcome

Homozygous familial hypercholesterolemia (HoFH) is a rare and serious autosomal genetic metabolic disease. Patients without intervention often die younger than 30 years old from early atherosclerotic cardiovascular disease (ASCVD)incurred by extremely high levels of low-density lipoprotein cholesterol (LDL-C). We present a case of HoFH, a child with compound heterozygous mutation in this study. The effect of conventional lipid-lowering therapy through diet control and lipid-lowering drugs was unsatisfactory. The blood-lipid purification proves effective but has poor compliance and difficult to maintain for a longer time. The patient received orthotopic liver transplantation and had been followed for 2 years, with the patient shows normal LDL-C, well growth and development. We hope the case will provide the clinician with better understanding of the diagnosis and treatment of the rare disease of HoFH.

Objective To investigate the predictive value of preoperative frailty for pulmonary com-plications(PPCs)after cardiac surgery in elderly patients.Methods A total of 162 elderly patients,109 males and 53 females,aged 65-83 years,BMI 18-36 kg/m2,ASA physical status Ⅱ-Ⅳ,underwent elec-tive open heart surgery from July 2022 to January 2023 were collected.The patients were divided into two groups according to the occurrence of PPCs:the PPCs group(n=57)and the non-PPCs group(n=105).General information,smoking history,alcohol consumption history,EuroSCORE Ⅱ,frailty,chronic comorbidities(hypertension,diabetes mellitus,myocardial infarction,pulmonary hypertension,chronic ob-structive pulmonary disease,sleep apnea syndrome,etc.),Hb,creatinine,albumin,pulmonary function indices,left ventricular ejection fraction,type of surgery,duration of surgery,aortic clamping time,and cardiopulmonary bypass time were collected.Factors with P＜0.2 and clinically significant in the univariate regression analysis were included in the multivariate logistic regression analysis,and the predictive efficacy of the Fried frailty scale and EuroSCORE Ⅱ for PPCs were compared by the area under the ROC curve(AUC).Results PPCs occurred in 57 patients(35.2％).Multifactorial Logistic regression analysis showed that frailty(OR=3.14,95％CI 1.05-9.37,P＜0.05)and EuroSCORE Ⅱ(OR=2.16,95％CI 1.01-4.60,P＜0.05)were risk factors for the development of PPCs.The predictive power of Fried frailty scale(AUC=0.76,95％CI 0.68-0.82)was significantly higher than that of EuroSCORE Ⅱ(AUC=0.65,95％CI 0.57-0.72)(P＜0.05).Conclusion Preoperative frailty is the independent risk factors for pulmonary complications after cardiac surgery in elderly patients,and the Fried frailty scale has a better predictive efficacy compared to EuroSCORE Ⅱ,a traditional risk predictor.

Objective To determine the radiation dose of sensitive organs under different protective methods in lung CT scanning environment, and to explore the best protective scheme of corresponding organs. Methods Annealed thermoluminescence dose elements were placed in the stomach, liver, colon, and thyroid gland of a simulated human body model. The dose effect experiment of protective methods included non-protective group, half lead apron group, and full lead apron group. The dose effect experiment of protective thickness included 0.50 mmpb full lead apron group and 0.35 mmpb full lead apron group. The same exposure conditions of lung CT scan were used in the above experiments. Results Compared with the non-protective group, the exposure dose of the stomach, liver, colon, and thyroid gland increased significantly in the half lead apron group (P < 0.05), and the exposure dose of the thyroid gland and colon decreased significantly in the full lead apron group (P < 0.05). There were no significant differences in the exposure dose of the liver, stomach, and colon in the simulated human body model between the 0.35 mmpb full lead apron group and the 0.50 mmpb full lead apron group. Conclusion For lung CT scan, the protective measure of lead apron may not reduce the exposure dose of subjects. The protective thickness of lead apron does not necessarily have a substantial influence on the exposure dose of human body.

Although widely applied in treating hematopoietic malignancies, transplantation of hematopoietic stem/progenitor cells (HSPCs) is impeded by HSPC shortage. Whether circulating HSPCs (cHSPCs) in steady-state blood could be used as an alternative source remains largely elusive. Here we develop a three-dimensional culture system (3DCS) including arginine, glycine, aspartate, and a series of factors. Fourteen-day culture of peripheral blood mononuclear cells (PBMNCs) in 3DCS led to 125- and 70-fold increase of the frequency and number of CD34⁺ cells. Further, 3DCS-expanded cHSPCs exhibited the similar reconstitution rate compared to CD34⁺ HSPCs in bone marrow. Mechanistically, 3DCS fabricated an immunomodulatory niche, secreting cytokines as TNF to support cHSPC survival and proliferation. Finally, 3DCS could also promote the expansion of cHSPCs in patients who failed in HSPC mobilization. Our 3DCS successfully expands rare cHSPCs, providing an alternative source for the HSPC therapy, particularly for the patients/donors who have failed in HSPC mobilization.
Antigens, CD34/metabolism* ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells ; Humans ; Leukocytes, Mononuclear/metabolism* ; Peptides/metabolism*

Objective : To study the guidance effects of fibroblast growth factor 3 ( FGF3) on the prethalamic γ⁃aminobutyric acid (GABA) inhibitory axons, and to explore the effect of FGF3 on the formation of thalamic axonal network. Methods : Expression and localization of FGF3 and prethalamic GABA marker gene in chicken embryonic diencephalon were detected by immunofluorescence. Early orientation of GABA axons was tracked by DiI. Three⁃dimensional gel co⁃culture experiment was carried out to investigate guidance effects of FGF3 on early prethalamic GABA inhibitory axons. Results : Prethalamic GABA cells were adjacent to the FGF3 + hypothalamas, located above the hypothalamus. DiI tracing experiments revealed that prethalamic GABA inhibitory axons had already extended into the thalamus at E6. Compared with the blank control group, FGF3 not only significantly promoted the growth of prethalamic GABA inhibitory nerve fibers in the prethalamus, but also repelled the newborn prethalamic axons to the dorsal thalamus. The number of prethalamic axons was significantly less in proximal section (towards FGF3 beads) than that in distal section (away from FGF3 beads) (P < 0. 01) . Moreover, the guidance effects of FGF3 on prethalamic axons could be blocked by the FGF pathway inhibitor SU5402. Conclusion FGF3, an axon guidance molecule expressed in hypothalamus, exerts guidance effects on the pathway selection of adjacent prethalamic GABA inhibitory nerve fibers.

Peking Union Medical College Hospital, as one of the most stressful medical institutions in China, is facing the problem of emergency department overcrowding. In order to effectively alleviate the emergency overcrowding, improve the medical quality and patients′ medical experience, the hospital firmly grasped the two incremental links of " throughput" and " output" factors, established a multidisciplinary and multi-department cooperation team, constructed a close medical alliance cooperation mode, and innovated and explored a harmonious emergency overcrowding relief mode with the goal of unblocking the " exit" of patients. The practice showed that the comprehensive measures could effectively alleviate the problem of emergency overcrowding, and improve the medical environment and medical quality.