The 5th edition of the World Health Organization (WHO) classification of haematolymphoid tumours used the hierarchical system to classify T-cell and NK-cell lymphoid proliferations and lymphomas (T/NK-LPD/LYM) based on research advances and clinicopathological characteristics of the diseases. In this edition of classification, tumour-like lesions were included, some tumors were added/deleted, the names or terms of certain diseases were refined, and the diagnostic criteria or subtypes of some diseases were revised. This group of diseases was reintegrated from non-clonal hyperplasia to highly aggressive lymphoma, which would further reflect the nature of T/NK-LPD/LYM and benefit to clinical application.

Objective:To explore the predictive factors for prepatellar subfascial gas in patients with closed patellar fracture and their impacts on the early infection following internal fixation.Methods:A retrospective analysis was performed in the 148 patients with closed patellar fracture who had been treated at Department of Orthopaedic Surgery, Zhangjiagang Hospital Affiliated to Soochow University from January 2018 through December 2021. All patients underwent preoperative three-dimensional CT examination of the knee joint and was treated by open reduction and internal fixation of patellar fractures. According to the presence or absence of gas in the prepatellar fascia, the patients were divided into 2 groups. In the gas group of 18 patients, there were 12 males and 6 females with an age of (58.3±14.5) years; in the gas-free group of 130 patients, there were 57 males and 73 females with an age of (60.5±14.6) years. The risk factors for prepatellar subfascial gas were screened out by comparing the gender, age, body mass index, injury mechanism, AO/OTA classification, diabetes, primary hypertension, neutrophil percentage, lymphocyte percentage, white blood cell count, neutrophil count, lymphocyte count, C-reactive protein, erythrocyte sedimentation rate, procalcitonin, and albumin before operation between the 2 groups. A receiver operating characteristic (ROC) curve for risk factors were made to identify the best screening points. The impacts of prepatellar subfascial gas were analyzed on early infection after internal fixation.Results:The preoperative neutrophil percentage was the risk factor for prepatellar subfascial gas ( P<0.05). The area under the ROC curve of preoperative neutrophil percentage for prediction of prepatellar subfascial gas was 0.700 (95% CI: 0.554 to 0.847), the optimal critical value was 78.45%, and the sensitivity and specificity were 0.556 and 0.831, respectively ( P=0.006). In the gas group, the incidence of early postoperative infection was insignificantly higher ( P=0.058) , but the time for postoperative antibiotic use was significantly longer and the dressing changes were significantly more frequent than those in the gas-free group ( P<0.05). Conclusions:In patients with closed patellar fracture, preoperative neutrophil percentage >78.45% can be used as an effective non-imaging indicator for prepatellar subfascial gas. A patient with prepatellar subfascial gas could be more prone to early postoperative infection.

Under the background of the comprehensively implementing the rule of law, the construction of legal practice of medical institutions in China is directly related to the sustainable and high-quality development of the medical service industry. However, at present, each medical institution lacked a systematic plan for the construction of legal practice, and the health administrative department mostly implemented the measures of inspection and punishment for illegal practice, which led to the situation that the illegal practice of medical institutions was " investigated but not corrected, and changed but invalid" . This paper creatively put forward the application research of administrative reconciliation theory in the legal practice management of medical and health institutions, for promoting medical and health institutions and medical staff to strengthen legal practice management, standardizing medical behavior, ensuring medical safety, and achieving high-quality development through the institutional incentive and guidance.

Objective:To investigate the clinical characteristics of the disease spectrum of pediatric hyper blood immunoglobulin E (IgE).Methods:A total of 498 children with total serum IgE ≥ 5×10 5 IU/L admitted to the Department of Pediatrics, Sun Yat-Sen Memorial Hospital of Sun Yat-Sen University from January 2012 to December 2018 were enrolled.Their clinical data, etiology distribution, clinical manifestations, laboratory results, treatment and outcomes were retrospectively analyzed.According to serum total IgE level, patients were divided into mildly increased IgE group (5×10 5-<10×10 5 IU/L), moderately increased group (10×10 5-<20×10 5 IU/L), and severely increased group (≥20×10 5 IU/L). The distribution of disease types among the 3 groups were compared. Results:(1) Allergic disease (213 cases) was the most common etiology in children with hyper blood IgE, and infectious disease (163 cases), mycoplasma pneumoniae (109 cases) and EB virus (120 cases) were common pathogens.(2) The incidence of allergic diseases (45.0%) and infectious diseases (42.2%) in the mildly increased group was significantly higher than that in the moderately increased group (40.8%, 26.2%, respectively) and the severely increased group(38.9%, 12.2%, respectively) (all P<0.001). The incidence of immune diseases(18.5%), tumors and hematological diseases (5.4%) in the moderately increased group was significantly higher than that of the mildly increased group (4.4%, 2.0%, respectively) (all P<0.001). The incidence of immune diseases (34.4%), tumors and hematological diseases (11.1%) in the severely increased group was significantly higher than that of the mildly increased group(4.4%, 2.0%, respectively) and the moderately increased group (18.5%, 5.4%, respectively) (all P<0.001). (3) The main clinical manifestations were fever (63.5%), respiratory symptoms (53.7%) and lympha-denopathy (53.7%), 47.5% of the children with hyper blood IgE had an increased white blood cell count, and 12.1% of them had an increased eosinophil count.(4) The most common specific allergens were dust mite combination (32.0%), milk (17.0%), and egg white (16.0%). There was no difference in disease distribution among the 3 groups of hyper blood IgE children with positive specific IgE ( P=0.164). Conclusions:Hyper blood IgE in children are most commonly caused by allergic and infectious diseases.The etiological distributions of hyper blood IgE in children at varying severities differ a lot.The higher the total IgE level, the higher the incidence of immunodeficiency disease, rheumatic disease, tumor and hematological disease.

The current document is based on a consensus reached by a panel of experts from the Chinese Society of Allergy and the Chinese Society of Otorhinolaryngology-Head and Neck Surgery, Rhinology Group. Chronic rhinosinusitis (CRS) affects approximately 8% of Chinese adults. The inflammatory and remodeling mechanisms of CRS in the Chinese population differ from those observed in the populations of European descent. Recently, precision medicine has been used to treat inflammation by targeting key biomarkers that are involved in the process. However, there are no CRS guidelines or a consensus available from China that can be shared with the international academia. The guidelines presented in this paper cover the epidemiology, economic burden, genetics and epigenetics, mechanisms, phenotypes and endotypes, diagnosis and differential diagnosis, management, and the current status of CRS in China. These guidelines—with a focus on China—will improve the abilities of clinical and medical staff during the treatment of CRS. Additionally, they will help international agencies in improving the verification of CRS endotypes, mapping of eosinophilic shifts, the identification of suitable biomarkers for endotyping, and predicting responses to therapies. In conclusion, these guidelines will help select therapies, such as pharmacotherapy, surgical approaches and innovative biotherapeutics, which are tailored to each of the individual CRS endotypes.
Adult ; Asian Continental Ancestry Group ; Biomarkers ; China ; Consensus ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Eosinophils ; Epidemiology ; Epigenomics ; Genetics ; Humans ; Hypersensitivity ; Inflammation ; International Agencies ; Medical Staff ; Neck ; Phenotype ; Precision Medicine

Objective To analyze the clinical manifestations and gene variations of tumor necrosis factor receptor-associated periodic syndrome (TRAPS).Methods Clinical data and gene testing of four children and three adult relatives in a family from Puning,Guangdong were retrospectively analyzed.CD4+T cells,CD8+T cells,B cells,monocytes and NK cells were assessed by flow cytometry.Plasma level of TNFR receptors were assessed by enzyme linked immunosorbent assay (ELISA).TNFRSF1A gene variation was identified by second generation sequencing.Swiss-Model was used to analyze the potential impact of TNFRSF1A gene variation on its protein tertiary structure.Results For all the patients,periodic fever was the main clinical feature,combined with arthralgia,myalgia,multiple serositis,periorbital edema and migratory cutaneous rash,accompanied with elevated level of acute-phase reactants and increased white blood cell counts during each episode.This disease was found in both gender and every generation in this family.The median age of onset was 2 years,ranging from 6 months to 30 years.The plasma level of TNFR1 of the patients range from 0 to 12.4 ng/L,which was lower than that of the normal controls range from 18.0～ 22.2 ng/L,while the level of TNFR2 was normal.Also,the numbers of T cells,B cells and monocytes were within normal range;however,number of NK cells in the patients (0.070±0.034) was lower than that in the normal controls (0.152±0.122).The TNFRSF 1A variation,located in exon 3:c.295T＞A (p.C99S),was found in the proband as well as the other 6 family members,which could induce change of the side chain of amino acid according to the prediction of the three-dimensional structure,subsequently affecting the binding to the receptor.Conclusions TRAPS is characterized by periodic fever,arthralgia,myalgia,multiple serositis,periorbital edema and migratory cutaneous rash,with a significant decrease in plasma level of TNFR1 and NK cells.The gene sequencing analysis revealed a pathogenic variation in TNFRSF1A gene.

Objective: To study the clinicopathologic features of lymphoproliferative disease by lymph node core needle biopsy(CNB)and to evaluate the diagnostic significance of CNB for lymphoproliferative disease. Methods: The annual distribution, entity constitute, clinical finding, gross feature, morphologic change, affiliate study and repeat biopsy diagnosis of 1 013 cases of lymph node CNB diagnosed at West China Hospital of Sichuan University from January 2009 to December 2015 were investigated. Results: (1) Proportion of lymph node CNB in total amount of biopsy specimens increased from 0.2% in 2009 to 0.8% in 2015.(2) The study cohort included 471 lymphomas, 12 atypical lymphoid hyperplasia (ALH), 136 suspected lymphomas, 372 benign lesions, and 22 cases of descriptive diagnoses. The most common types were diffuse large B cell lymphoma and T-lymphoblastic lymphoma. (3) Majority of patients were adolescents and children younger than 20 years or the elderly older than 60 years. 53.1% CNB tumor specimen consisted of ≥4 tissue cores and 40.5% were >2 cm in length. (4) 104 CNB cases with previous history of excision biopsy was included 45 carcinomas(no metastatic carcinoma was found), 32 lymphomas for treatment observation.1/14 suspicious lymphomas, 1/1 ALH and 3/22 cases benign lesions were diagnosed as lymphoma by repeat biopsy respectively. (5) 217 CNB cases were diagnosed as lymphoma by subsequent CNB (70), or subsequent excision biopsy (147) including 78.5%(73/93) suspected lymphomas, 5/7 ALH and 32.3%(20/62)benign lesions. Conclusions Lymph node CNB has certain clinical indications, although limited for the diagnosis of lymphoproliferative disorders. Suspected lymphomas and ALH diagnosed by CNB should be followed by repeat tissue biopsy. For the benign lesions by CNB it does not rule out additional biopsy to further investigate the lesion.

Objective: To investigate the feasibility and value of interphase fluorescence in situ hybridization (FISH) in the pathological diagnosis, differential diagnosis and therapeutic assessment of B-cell lymphomas. Methods: The cohort included 604 cases of B-cell lymphoma which were collected at West China Hospital from May 2010 to December 2016.And all were subjected to interphase FISH using 11 break apart or fusion probes (MYC, bcl-2, bcl-6, IRF4, MYC/IgH, bcl-2/IgH, CCND1/IgH, IgH, API2/MALT1, p53/ATM, and D13S319/CEP12). Results: The median age of the 604 B-cell lymphoma patients was 47.7 (aged 2-90) years including 372 men and 232 women. All the cases was divided into 463 large B cell lymphomas(LBL) and 141 small B cell lymphomas, and the total interphase FISH positive rate was 59.8% (361/604). Among the 463 LBL, 12.5% (58/463), 9.5% (44/463) and 2.2% (10/463) of cases showed MYC, bcl-6 and bcl-2 gene rearrangements respectively; and 363 diffuse large B cell lymphoma (DLBCLs) were reclassified as germinal center B-cell (GCB) subtype (38.6%, 140/363) and non-GCB subtype (61.4%, 223/363) by Hans algorithm. The rearrangement rates in GCB and non-GCB DLBCL were 45.7%(64/140)and 21.5%(48/223; P=0.001), respectively. Compared to the non-GCB DLBCL, GCB DLBCL showed higher MYC and bcl-2 gene rearrangements (P=0.001). Eleven (2.4%, 11/463) cases had MYC and bcl-6 or bcl-2 gene rearrangement (double-hit lymphoma); one (0.2%, 1/463) case had MYC, bcl-6 and bcl-2 gene rearrangements (triple-hit lymphoma); two (0.4%, 2/463) cases had bcl-2 and bcl-6 gene rearrangements. MYC translocation and MYC/IgH fusion were detected in 94.2%(81/86) and 83.7%(72/86) cases of Burkitt lymphomas. IRF4 rearrangement was detected in two cases of IRF4+ LBCL. Genetic abnormalities were detected in 9/19, 100%(29/29), 30.8%(12/39) and 68.5%(37/54) cases of follicular lymphoma, mantle cell lymphoma, MALT lymphoma and chronic lymphocytic leukemia, respectively. Conclusions Interphase FISH can rapidly and accurately detect the genetic changes in B-cell lymphomas. Different genetic changes are specifically valuable to the diagnosis, differential diagnosis, prognosis evaluation and treatment guidance of various B-cell lymphomas.

OBJECTIVETo study the clinicopathologic features and significance of aberrant CD56 expression in diffuse large B-cell lymphoma (DLBCL).

METHODSThe clinical and pathologic profiles of 10 cases of DLBCL with aberrant expression of CD56 were investigated. Immunohistochemical staining, in-situ hybridization for Epstein-Barr virus encoded RNA and gene rearrangement for IgH and Igκ were carried out.

RESULTSThere were 6 male and 4 female patients. The medium age of patients was 46 years. All of them presented with extranodal lymphoma involvement, with gastrointestinal tract being the commonest site (5/10). Histologic examination showed that most of the atypical lymphoid cells were centroblast-like and demonstrated a diffuse growth pattern. Apoptosis and necrosis were identified in some cases. Immunohistochemical study showed that the tumor cells were positive for CD20 or CD79α and aberrantly expressed CD56. Five cases had the GCB phenotype while the remaining cases had the non-GCB phenotype, according to Hans classification. Bcl-6 was positive in most cases (9/10). All cases showed a high proliferation index by Ki-67. The tumor cells were negative for CD3ε, CD138 and granzyme B. In-situ hybridization for Epstein-Barr virus encoded RNA was performed in 7 cases and none of them showed positive signals. IgH gene rearranged bands were detected in 4 cases (4/6) and Igκ was detected in 3 cases (3/6). Follow-up data were available in 8 patients. Two patients died of disease progression within 5 to 13 months after diagnosis and the other 6 patients were alive 8 to 60 months after therapy.

CONCLUSIONSDLBCL with aberrant expression of CD56 is rare. Most of them present with extranodal involvement, show high frequency of bcl-6 expression and high proliferation index. The patients often have good response to chemotherapy.

Antigens, CD20 ; metabolism ; Apoptosis ; CD56 Antigen ; metabolism ; CD79 Antigens ; metabolism ; Disease Progression ; Female ; Gene Rearrangement ; Granzymes ; metabolism ; Herpesvirus 4, Human ; genetics ; Humans ; Immunophenotyping ; In Situ Hybridization ; Lymphoma, Large B-Cell, Diffuse ; genetics ; metabolism ; pathology ; Male ; Middle Aged ; Necrosis ; Phenotype ; Proto-Oncogene Proteins c-bcl-6 ; metabolism ; RNA, Viral ; analysis

Objective To study the etiology,relevant factors and endoscopic characteristics of acute lower gastrointestinal tract hemorrhage.Methods A total of 938 patients receiving emergency colonoscopy for acute lower gastrointestinal tract hemorrhage were collected to study the etiological profiles.Different causes between young group and elderly group were also recorded.Results The total detectable rate of acute lower gastrointestinal tract hemorrhage in respect of causes was 96.16％ (902/938).The five leading causes were colorectal malignancy (41.26％),erosive colorectal polypus (12.37％),inflammatory bowel disease (9.59％),erosive and ulcer colitis (10.34％) and hemorrhoids (8.64％).Compared with youth and middle age patients,the incidence of colorectal malignancy (x2 =68.116,df =2,P =0.000) and colorectal polyps (x2 =9.101,df =2,P =0.011) were significantly increased in elderly patients,while incidence of inflammatory bowel disease (x2 =62.150,df=2,P =0.000) and erosive and ulcer colitis (x2 =20.292,df =2,P =0.000) were significantly decreased in those patients.Conclusions Emergency colonoscopy for diagnosis of acute lower gastrointestinal tract hemorrhage is an important measure.The major manifestation is bleeding resulting from colorectal malignancy,erosive colorectal polypus,inflammatory bowel disease,erosive and ulcer colitis and anal hemorrhoid.Age is associated with the cause of lower gastrointestinal tract bleeding induced by different kinds of lesions at the colorectum.