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    25 December 2023, Volume 22 Issue 06 Previous Issue   
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    Expert forum
    Diagnosis and treatment practice of chest tightness variant asthma in China
    HUA Wen, SHEN Huahao
    2023, 22 (06):  513-519.  DOI: 10.16150/j.1671-2870.2023.06.001
    Abstract ( 41 )   HTML ( 2 )   PDF (881KB) ( 18 )  

    Chest tightness variant asthma (CTVA) is an atypical form of asthma, characterized by chest tightness as the primary symptom, without the typical asthma symptoms, such as recurrent wheezing and shortness of breath. Since it was first reported and named internationally in 2013, CTVA has gradually been recognized by clinical physicians in China and has undergone extensive clinical practice. As for diagnosis of CTVA ,doctors should integrate symptoms of chest tightness and objective evidence of variable airflow restriction, exclude chest tightness caused by other diseases, and find effective results of asthma treatment in patients. The treatment principle of CTVA is similar to that of typical asthma. An appropriate initial treatment level should be selected based on the patient's chest tightness attack, and the stepwise treatment plan should be followed for upgrade and downgrade treatment. Based on clinical diagnosis and treatment practices, this article systematically introduces the diagnostic criteria, diagnostic procedures, management evaluation, and treatment plans of CTVA,which is to help clinical physicians in standardizing the diagnosis and treatment of CTVA.

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    Current status and countermeasures of diagnosis and treatment of mild bronchial asthma in China
    ZHOU Yan, ZHANG Min
    2023, 22 (06):  520-526.  DOI: 10.16150/j.1671-2870.2023.06.002
    Abstract ( 41 )   HTML ( 2 )   PDF (1139KB) ( 10 )  

    In China, bronchial asthma has emerged as the second most prevalent respiratory disease, with mild asthma constituting 50%-70% of the reported cases. The challenges in diagnosing mild asthma arise from the subtle or atypical symptoms exhibited by patients, coupled with the absence of characteristic wheezing sounds. Notably, pulmonary function tests frequently yield normal indices for large airway functions, contributing to a significant misdiagnosis rate exceeding 70%. This high misdiagnosis rate results in patients seeking medical care repeatedly, and some cases receiving inappropriate treatments, with incresed the risk of progressing to moderate or severe asthma, experiencing acute attacks, and facing the potential threat of mortalitydeath. About one-third of asthma deaths occur in mild asthma. Furthermore, the predominant focus on symptom relief rather than the effective control of airway inflammation raises significant concerns regar-ding the diagnosis and treatment of mild asthma in China. In response, the "Expert consensus on the diagnosis, treatment and management of mild bronchial asthma in China (2023 edition)" introduces two diagnostic pathways, namely definitive and presumptive diagnosis, with the aim of enhancing diagnostic accuracy and standardizing treatment approaches. Based on the limitations associated with methods for definitive diagnosis (such as a bronchodilator test's positive rate less than 10% in mild asthma patients and the low clinical implementation rate of bronchial provocation tests), and an understanding of the pathophysiological mechanisms of small airway dysfunction, a presumptive diagnosis pathway to address these inherent shortcomings is proposed. Initially, a singular pulmonary function test is employed for presumptive diagnosis, integrating small airway function with airway inflammation indices. The model is fine-tuned based on age and gender considerations. The assessment of large and small airway reversibility in patients with negative bronchodilator tests serves as a pivotal guide for diagnostic treatment, minimizing the risks of misdiagnosis and overdiagnosis. Treatment and management strategies are selected based on the level of symptom control, with an emphasis on anti-inflammatory approaches. Employing a patient-centered shared decision-making model and implementing an "assessment-adjustment-review" asthma management cycle to individualize precision management is executed to establish diagnostic and treatment strategies for mild asthma, enhancing the overall standard of care for mild asthma.

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    Pay attention to standardized diagnosis and treatment of corticosteroid-responsive cough
    SHI Cuiqin, YU Li
    2023, 22 (06):  527-533.  DOI: 10.16150/j.1671-2870.2023.06.003
    Abstract ( 20 )   HTML ( 1 )   PDF (1908KB) ( 10 )  

    The global prevalence of chronic cough in adults is 9.6%.In 2008, the cough special issue of Lancet published a diagnosis and treatment plan to distinguish the causes of chronic cough based on eosinophilic airway inflammation or sensitivity to glucocorticoids therapy. In 2014, Professor Lai Kefang of China first proposed the Chinese name “corticosteroid-responsive cough” (CRC). CRC refers to chronic cough that is effectively treated with glucocorticoids, accounting for 63% of the causes of chronic cough in China, including cough variant asthma, eosinophilic bronchitis, and atopic cough. Cough variant asthma is the most common cause of chronic cough in China, accounting for 30% to 50% Chronic cough has a complex etiology and involves numerous examinations, especially bronchial provocation tests, induced sputum cell analysis, multi-channel esophageal impedance pH monitoring, etc., which have not been carried out in many tertiary hospitals, and not all chronic patients can undergo relevant examinations to confirm diagnosis and receive treatment. The concept of corticosteroid-responsive cough has important guiding significance for the empirical treatment of chronic cough, and is suitable for promotion in grassroots hospitals. In clinical practice, it is only necessary to determine whether it is a CRC based on the clinical characteristics of the patient without the need for further tests, such as induced sputum cell analysis and bronchial provocation tests to distinguish cough variant asthma, eosinophilic bronchitis, and allergic cough, and then empirical treatment can be given. However, there is currently insufficient understanding of the standardized diagnosis and treatment of CRC. For example, there is still a lack of consensus on determining whether it is CRC, how to select hormone treatment and the duration of hormone treatment. It is necessary to further emphasize and promote the relevant concepts and diagnosis and treatment plans of corticosteroid-responsive cough.

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    Current status and strategies of asthma questionnaire application in China
    BAO Wuping, LIN Yanmei, ZHANG Min
    2023, 22 (06):  534-540.  DOI: 10.16150/j.1671-2870.2023.06.004
    Abstract ( 23 )   HTML ( 1 )   PDF (873KB) ( 10 )  

    The prevalence of bronchial asthma in China is 4.2%, similar to other countries around the world, but the mortality rate of asthma in China is 36.7 /100 000, which is four times as high as that of Japan and seven times as that of the United States. Therefore, It is urgent for China to strengthen the effectiveness of asthma management and minimize the risk of related deaths. Pivotal aspects of asthma management involve screening and assessing symptom control. Simple screening tools expedite rapid identification of patients who require more detailed assessment in primary medical care. Numerical tools like ACT and ACQ are widely used in clinical practice and clinical research. Categorical symptom control tools are extensively used in clinical studies. For example,asthma APGAR (Activities, Persistence, Triggers, Asthma medications, Response to therapy) questionnaire not only assists in assessing asthma, but also in enhancing treatment adherence, improving asthma control. Currently, some asthma questionnaires face limitations in their application in China, particularly concerning sociocultural factors, specific age groups, and heterogeneous phenotypes. More attention should be paid to individualized factors (age diverse, clinical phenotypes), and use of biomarkers and intelligent technologies in questionnaire design for enhancing management of asthma.

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    Academic trend at home and abroad
    Analysis of global burden of bacterial infections: an interpretation of Global mortality associated with 33 bacterial pathogens in 2019
    ZHANG Ying, JIANG Xiaofei
    2023, 22 (06):  541-549.  DOI: 10.16150/j.1671-2870.2023.06.005
    Abstract ( 111 )   HTML ( 1 )   PDF (1585KB) ( 45 )  

    The Global Burden of Bacterial Infections report showed that in 2019, there were approximately 13.7 million deaths worldwide due to infection (95% UI ranging from 10.9 million to 17.1 million), and bacterial infection related deaths accounted for 13.6% (10.1%-18.1%) of global deaths, making it the second leading cause of death globally, second only to ischemic heart disease. Eleven infectious syndromes involve 33 bacterial pathogens and are associated with 7.7 million deaths worldwide (5.7 million to 10.2 million). Lower respiratory tract infections have caused approximately 4 million deaths worldwide (3.33 million to 4.89 million), with Streptococcus pneumoniae causing the most deaths at 653 000 (553 000 to 777 000); Blood flow infections have caused 2.91 million people (1.74 million to 4.53 million), with Staphylococcus aureus causing the most deaths, at 299 000 people (166 000 to 485 000); Peritoneal and intra-abdominal infections have caused 1.28 million deaths (826 000 to 1.86 million), with Escherichia coli causing the most deaths, reaching 290000 (188 000 to 423 000). The Global Burden of Bacterial Infections report also shows that in 2019, Staphylococcus aureus was associated with over 1 million deaths (resulting in 1.105 million deaths); Escherichia coli causes 450 000 (329 000 to 602 000) female and 500 000 (355 000 to 684 000) male deaths worldwide, while Streptococcus pneumoniae causes over 750 000 deaths worldwide; Klebsiella pneumoniae causes over 750 000 deaths worldwide; Pseudomonas aeruginosa has caused over 500 000 deaths worldwide. The Global Burden of Bacterial Infections report data shows that the pathogens most associated with mortality worldwide vary by age. Staphylococcus aureus is the deadliest pathogen among people aged 15 and above, with 940 000 deaths (682 000 to 1 276 000) in this age group; Salmonella typhi is most associated with mortality in children aged 5-14, with a death toll of 49 000 (23 000-86 000); Streptococcus pneumoniae is most associated with the death of newborns to 4-year-old children, with a death toll of 225 000 (180 000 to 281 000). The Global Burden of Bacterial Infections report estimates the mortality rates of various pathogens and infectious syndromes, some of which were previously unknown and some were severely underestimated, especially in low-and middle-income countries where the burden is disproportionately high. However, there is a lack of corresponding data and research in China, and it is necessary to conduct corresponding surveys and research to develop innovative strategies.

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    Original articles
    Analysis of uterine blood flow parameters in immune-related recurrent spontaneous abortion
    KONG Xiaoxiao, CHEN Ping, NIU Jianmei, LÜ Mingli, WANG Hui
    2023, 22 (06):  550-554.  DOI: 10.16150/j.1671-2870.2023.06.006
    Abstract ( 23 )   HTML ( 2 )   PDF (807KB) ( 13 )  

    Objective: To analyze the uterine artery and endometrial blood flow parameters by in patients with autoimmune-related recurrent spontaneous abortion (RSA) for providing clinical referene. Methods: A total of 48 patients with more than 2 consecutive miscarriages from March 2021 to March 2022 were selected. Transvaginal three-dimensional ultrasound were used to measure endometrial thickness, endometrial volume, hemodynamic parameters of the endometrium [vascularization index (VI) and vascularization flow index (VFI)], and blood flow parameters [resistance index (RI), pulsatility index (PI), and peak systolic to lowest diastolic velocity ratio (S/D)] of bilateral uterine arteries were assessed in 6-9 days after ovulation. According to the autoimmune-related indexes(antinuclear antibody, anti-cardiolipin antibody、, beta 2 glycoprotein Ⅰ, anti-double strand DNA antibody), patients were divided into observation group and control group. Patients with all negative results were classified as non-autoimmune-related RSA (control group), while those with one or more abnormal indexes were considered RSA (observation group). Ultrasonographic parameters were compared between the two groups, and receiver operating characteristic (ROC) curves were used to evaluate the predictive value of ultrasonographic parameters for autoimmune-related RSA. Results: Autoimmune-related RSA accounted for 25% of clinically recurrent miscarriages. The VI and VFI values in the observation group were lower than those in the control group(P=0.005,0.017). The average levels of PI and RI of bilateral uterine arteries in the observation group were significantly higher than those in the control group(P≤0.001,0.001).There was no statistical difference in endometrial thickness and endometrial volume between the two groups. ROC curve analysis showed that uterine artery PI > 2.895, RI > 0.885, endometrial VI < 1.518%, and VFI < 0.140 predicted autoimmune-related RSA with area under the curve (AUC) values of 0.738,0.746,0.809,0.774,respectively. Conclusions: Compared to other RSA cases, patients with autoimmune-related RSA have increased uterine artery PI and RI values and reduced endometrial blood flow perfusion. Clinicians should be vigilant in screening for autoimmune-related indexes in such patients during clinical ultrasound examinations.

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    Value of Kyoto gastritis score and modified prediction model in diagnosing Helicobacter pylori infection status under gastroscopy in Chinese population
    QIAN Danye, MENG Xiangjun, ZHU Liming
    2023, 22 (06):  555-561.  DOI: 10.16150/j.1671-2870.2023.06.007
    Abstract ( 42 )   HTML ( 1 )   PDF (2569KB) ( 24 )  

    Objective: To validate the application value of Kyoto gastritis score in diagnosing Helicobacter pylori (Hp) infection status under gastroscopy in Chinses population, and to construct a modified prediction model to improve the diagnostic value of Hp infection. Methods: A total of 749 cases who underwent gastroscopy at the Digestive Endoscopy Center of the Ninth People's Hospital (North), Shanghai Jiao Tong University School of Medicine, from January 1, 2021 to June 1, 2023 were retrospectively collected. Patients were divided into Hp-positive group and Hp-negative group based on histopathological biopsy or 13C breath test results. The endoscopic mucosal manifestations were recorded according to the Kyoto classification of gastritis, including atrophy, intestinal metaplasia, fold enlargement, diffuse redness, nodularity, RAC, red streak, and other 18 manifestations to verify the diagnostic value of Kyoto gastritis score for Hp infection. The mucosal manifestations with significant differences in univariate analysis were included in binary Logistic regression, and a modified prediction model was constructed according to the regression coefficients. ROC analysis was used to analyze the diagnostic value of Kyoto gastritis score and the modified prediction model for Hp infection. Results: The Hp infection rate was 54.47%. Intestinal metaplasia, fold enlargement, nodularity, diffuse redness, spotty redness, and sticky mucus were independent predictors of Hp infection. RAC, red streak, white and flat elevated lesion were independent predictors of Hp non-infection. The area under the curve was 0.861 (95%CI was 0.835~0.887), the accuracy was 75.2%, and the sensitivity and specificity were 88.5% and 65.1%. Mucosal manifestations such as spotty redness, sticky mucus, and RAC were incorporated into the modified prediction model. The results showed that the specificity (85.9%), positive predictive value (88.2%), negative predictive value (85.4%) and AUC [0.929 (95%CI was 0.910-0.947)] of the modified prediction model for Hp infection were higher than those of Kyoto gastritis score [65.1%, 75.2%, 82.5% and 0.861 (95%CI was 0.835-0.887), respectively]. Conclusions: Kyoto gastritis score has a certain application value for Hp in the Chinese population, and the modified prediction model has a higher diagnostic value for Hp infection.

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    Analysis of all-cause mortality data of residents in Shannan City, Tibet, 2021
    ZHAN Dui, NIMA Zhuoma, DOU Ji, PINGCUO Zhuoma, QI Jinlei
    2023, 22 (06):  562-566.  DOI: 10.16150/j.1671-2870.2023.06.008
    Abstract ( 20 )   HTML ( 0 )   PDF (864KB) ( 7 )  

    Objective: To analyze the death characteristics of residents in Shannan City, Tibet autonomous region in 2021 for understanding the causes of death and the sequence of causes of death, and provide a basis for formulating re-levant prevention and control strategies. Methods: Indicators of mortality rate, standardized mortality rate, years of life lost (YLL), probability of premature death, and life expectancy to reflect causes of death were analyzed in Shannan City in 2021. Results: In 2021, the total mortality rate of residents in Shannan City was 500.3/100 000 (standardized rate 1060.9/100,000); the male mortality rate was 539.2/100 000 (standardized rate 1 282.5/100, 000); the female mortality rate was 462.5/100 000 (standardized rate 889.5/100 000). The mortality rate of males was higher than that of females. The total mortality YLL was 35 998.8, among which there was 20 827.0 for males and 15171.8 for females. The top five causes of death among residents were cerebrovascular disease, heart disease, malignant tumor, respiratory system disease, and infectious disease, accounting for 71.7% of the reported deaths. Chronic non-communicable diseases accounted for 68.4% of all deaths. The probability of premature death from the four main chronic diseases was 17.5%, among which there was 21.9% for men and 13.5% for women. The life expectancy of residents in Shannan was 75.3 years, 72.9 years for men and 77.7 years for women. Conclusions: The probability of premature death among Shannan city residents for four major categories of chronic diseases is higher than the national level, and the four major categories of chronic diseases are still the main causes of death in Shannan City residents. Reducing premature death and YLL from chronic diseases is the main goal of preventing and controlling chronic diseases.

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    Value of 18F-PSMA PET/MRI for early diagnosis of recurrence and metastasis in prostate cancer patients after radical prostatectomy
    ZHOU Yilei, ZHANG Miao, GUO Rui, ZHOU Jinxin, LI Biao, LI Xiang
    2023, 22 (06):  567-572.  DOI: 10.16150/j.1671-2870.2023.06.009
    Abstract ( 26 )   HTML ( 1 )   PDF (2687KB) ( 5 )  

    Objective: To investigate the value and significance of 18F-prostate-specific membrane antigen (18F-PSMA)-1007 PET/MR for early diagnosis of recurrence and metastasis in prostate cancer patients after radical prostatectomy (RP). Methods: A total of 143 patients with prostate cancer after radical prostatectomy were enrolled during June 2019 to January 2022 in Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine. All the patients underwent 18F-PSMA-1007 PET/MR imaging within 12 to 60 months after surgery. According to the serum prostate-specific antigen (PSA) levels, patients were divided into 5 groups(PSA≤0.2 ng/mL, 0.2 ng/mL<PSA≤0.5 ng/mL, 0.5 ng/mL<PSA≤4.0 ng/mL, 4.0 ng/mL<PSA<10.0 ng/mL and PSA≥10.0 ng/mL). The detection rates of recurrence or metastasis were compared between different groups. Results: The detection rates of 18F-PSMA-1007 PET/MR for recurrence or metastasis in PSA≤0.2 ng/mL, 0.2 ng/mL<PSA≤0.5 ng/mL, 0.5 ng/mL< PSA ≤ 4.0 ng/mL, 4.0 ng/mL<PSA<10.0 ng/mL and PSA ≥ 10.0 ng/mL groups were 25.00% (6/24),70.00% (7/10),66.67% (16/24),74.07% (20/27) and 94.83% (55/58) respectively, and bilateral comparison between groups revealed that difference in detection rate was statistically significant (P<0.01 or P<0.05). In terms of treatment,34 patients (27.64%) changed their pre-examination treatment regimen based on the results of 18F-PSMA-1007 PET/MR imaging. Conclusions: It reveals that prostate patients with detected recurrence or metastasis account for 25% of those with normal PSA level.The detection rate increases with the increase of PSA levels. It suggests that 18F-PSMA PET/MR can detect recurrence and metastasis in the early stage, which can effectively guide the formulation of treatment plans.

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    Study on the diagnostic efficacy of ADNEX model in differentiating metastatic and primary ovarian cancer
    NI Zhongxin, CHEN Hui
    2023, 22 (06):  573-578.  DOI: 10.16150/j.1671-2870.2023.06.010
    Abstract ( 30 )   HTML ( 0 )   PDF (854KB) ( 13 )  

    Objective: To study the clinical value of the International Ovarian Tumor Analysis(IOTA) ADNEX(the Assessment of Differential NEopasia in the adneXa) model in differentiating metastatic ovarian cancer from primary ovarian cancer. Methods: Patients who underwent transvaginal ultrasonography and operation in our hospital from March 2016 to April 2021 were selected. Preoperative ultrasound examination was performed, and the model prediction results were recorded. Based on the postoperative pathological results, the sensitivity, specificity and area under receiver operating characteristic (ROC) curve of the ADNEX model with and without CA125 in distinguishing metastatic and primary ovarian cancer was calculated. Results: A total of 197 patients with ovarian cancer were enrolled, including 153 patients with primary ovarian cancer (36 patients with stage Ⅰ ovarian cancer, 117 patients with stage Ⅰ-Ⅳ ovarian cancer) and 44 patients with metastatic ovarian cancer. The area under the ROC curve of ADNEX model without CA125 in differentiating metastatic and primary ovarian cancer was 0.621 (95% confidence interval 0.534-0.708), with a sensitivity of 93.2% and a specificity of 31.4%.The area under the ROC curve of ADNEX model with CA125 was 0.810 (95% confidence interval 0.747-0.872), with a sensitivity of 79.5% and a specificity of 69.3%. The difference between the two was statistically significant (P<0.001). Conclusions: ADNEX model has good clinical value in the differentiation of metastatic and primary ovarian cancer. Use of CA125 in ADNEX can improve the diagnostic efficiency of the model.

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    Review articles
    Clinical research on progress of antinuclear antibody in rheumatoid arthritis
    LI Yuan, ZHANG Le, YIN Hanlin, ZHENG Bing, LÜ Liangjing
    2023, 22 (06):  579-586.  DOI: 10.16150/j.1671-2870.2023.06.011
    Abstract ( 29 )   HTML ( 0 )   PDF (900KB) ( 18 )  

    Rheumatoid arthritis (RA) is a common chronic, systemic autoimmune disease characterized by erosive arthritis, with a global prevalence of 0.25% to 1.00%, which may ultimately lead to joint deformity and loss of function. Antinuclear antibody (ANA) refers to auto-antibodies that target various components of eukaryotic cells. Although ANA may be frequently detected in RA patients, with a positive rate of 30% to 60%, the significance of ANA in the diagnosis and treatment of RA remains unclear. Nuclear patterns in RA population are dominated by speckled and homogeneous patterns, mainly at low titres. The detection rate of extractable nuclear antibodies is usually lower than that of ANA. The serum levels of rheumatoid factor and anti-cyclic citrullinated peptide antibody in ANA-positive RA patients are much higher than ANA-negative ones, and joint erosion of ANA-positive RA patients had more severe joint erosion on imaging as well. Therefore, ANA is a potential predictor of severe joint injury. ANA-positive RA patients are more likely to have extra-articular manifestations [such as subcutaneous nodules(82% vs 46%), ocular lesions(38% vs 6%), and infections(38% vs 12%)].In terms of RA comorbidities, compared to the ANA negative group, the ANA positive group had higher levels of moderate and severe anemia (16.04% vs 6.9%; 6.6% vs 0.07%), Sjogren's syndrome (19.5% vs 4.1%), and vasculitis (29% vs 7%). In addition, ANA positivity is an independent risk factor for elderly RA patients with carotid intimal thickening (HR=4.089) and adverse pregnancy outcomes in female RA (OR=3.268, P=0.045), respectively. Attention should be paid to high-titre ANA-positive RA patients for preventive measures. In terms of treatment, RA patients who are ANA-positive when given tumour necrosis factor-α inhibitor (TNFi) treatment got much poorer therapeutic response than ANA-negative ones, especially those with high titres were more prone to generating anti-drug antibodies. Increase of ANA titer induced by TNFi is associated with no response to TNFi treatment, thus monitoring the change of ANA may predict the long-term efficacy of TNFi. The most common adverse event of TNFi is drug-induced lupus. Although it has been confirmed that the induction of anti-double-stranded DNA antibodies is associated with poor efficacy of TNFi, large-scale studies are still needed to confirm the correlation between the increase of ANA titer or ocurence of dsDNA antibody and the induction of drug-induced lupus. In conclusion, this paper reviews the research progress on ANA characteristics in RA population, clinical manifestations of ANA-positive RA, and the association between ANA and TNFi prognosis. The potential of ANA as a new biomarker of RA needs to be further studied.

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    Advances in molecular pathology of Ewing sarcoma
    LIU Hengan, WANG Chaofu
    2023, 22 (06):  587-592.  DOI: 10.16150/j.1671-2870.2023.06.012
    Abstract ( 27 )   HTML ( 2 )   PDF (814KB) ( 17 )  

    Ewing sarcoma is a rare malignant small round cell mesenchymal neoplasm with a characteristic FET family-ETS family fusion gene, and is classified as undifferentiated small round cell sarcoma in the fifth revision of the WHO Classification of Tumors of Soft Tissue and Bone (2020). Microscopically, Ewing sarcoma is composed of a monomorphic round cell population, accompanied by different degrees of neuroectodermal differentiation. Although surgery combined with chemotherapy has increased the 5-year survival rate of Ewing sarcoma to about 70%, the 5-year survival rate of patients with metastases at diagnosis is still less than 30%. With the rapid development of molecular pathology, the molecular mechanism of Ewing sarcoma has also become a focus of research. This article reviews the latest research progress on the unique pathological features, molecular mechanisms and genetic diagnostic criteria of Ewing sarcoma, in order to provide a new direction for the clinical diagnosis, treatment and prognosis of Ewing sarcoma.

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    Case reports
    Hydroa vacciniforme lymphoproliferative disorder:a case report
    ZHU Xia, JIN Jingjing, WANG Xin, MIAO Fei, MA Jiexian, ZHANG Jiechen, XIAO Li
    2023, 22 (06):  593-597.  DOI: 10.16150/j.1671-2870.2023.06.013
    Abstract ( 36 )   HTML ( 1 )   PDF (11056KB) ( 18 )  

    In this study, we report a case of hydroa vacciniforme lymphoproliferative disorder (HVLPD). The patient, who was 21 years old at the time of initial consultation,suffering from recurrent papules, vesicular rashes, bleeding and black scabs on the neck, face and trunk. Serum EBV-DNA was significantly increased (2.88×107 copies/mL). The patient underwent skin biopsies twice within 2 years. The pathology of the first skin biopsy showed partial degeneration and loosening of the epidermal stratum spinosum, intraepidermal blister formation, partial epidermal detachment, and multifocal small abscesses seen in the blisters and stratum spinosum. Patchy infiltration of small lymphocytes, plasma cells, histiocytes, and eosinophils in the dermis, with no significant atypia of lymphocytes, EBER in situ hybridization was negative, which made it difficult to make a definitive diagnosis on pathology. The pathology of the second skin biopsy showed blisters visible within the patient's epidermis, and atypical lymphoid cells infiltrate around the hair follicles, sweat glands and blood vessels in the dermis. The immunohistochemical analysis indicated that lymphoid cells were positive for CD3, CD5, CD4, CD8, granzyme B and TIA-1, while CD56 and Perforin were negative, and the proliferation rate of Ki-67 was approximately 10%. EBER was positive by in situ hybridization consistent with clinicopathologic features of HVLPD. More than 1 year after receiving symptomatic treatment, the patient's rash worsened, with sometimes fever and left eyelid edema. The third skin biopsy performed in the other hospital showed that atypical lymphoid cells infiltrated the subcutaneous adipose tissue, and the proliferation rate of Ki-67 was 60%. The disease progressed to EBER-positive T-cell lymphoma. After 2 courses of chemotherapy with gemcitabine, cisplatin, dexamethasone and pegaspargase, the patient's edema subsided and the rash healed. This report demonstrates the clinical and pathologic features of the disease during its evolution and progression, with a view to enriching its diagnostic and therapeutic experience.

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    Medical education