1.Kidney transplantation from donors with Marfan syndrome: report of 2 cases and literature review
Meng ZHANG ; Yibin WANG ; Yuchen WANG ; Rumin LIU ; Ziyan YAN ; Renfei XIA ; Wenli ZENG ; Jialiang HUI ; Minjie ZHOU ; Jian XU ; Yun MIAO
Organ Transplantation 2024;15(2):257-262
Objective To investigate the feasibility and clinical experience of kidney transplantation from donors with Marfan syndrome (MFS). Methods Clinical data of 2 recipients undergoing kidney transplantation from the same MFS patient were retrospectively analyzed and literature review of 2 cases was conducted. Characteristics and clinical diagnosis and treatment of kidney transplantation from MFS patients were summarized. Results The Remuzzi scores of the left and right donor kidneys of the MFS patient during time-zero biopsy were 1 and 2. No significant difference was observed in the renal arteriole wall compared with other donors of brain death and cardiac death. Two recipients who received kidney transplantation from the MFS patient suffered from postoperative delayed graft function. After short-term hemodialysis, the graft function of the recipients received the left and right kidney began to gradually recover at postoperative 10 d and 20 d. After discharge, serum creatinine level of the recipient received the left kidney was ranged from 80 to 90 μmol/L, whereas that of the recipient received the right kidney kept declining, and the lowest serum creatinine level was 232 μmol/L before the submission date (at postoperative 43 d). Through literature review, two cases successfully undergoing kidney transplantation from the same MFS donor were reported. Both two recipients experienced delayed graft function, and then renal function was restored to normal. Until the publication date, 1 recipient has survived for 6 years, and the other recipient died of de novo cerebrovascular disease at postoperative 2 years. Conclusions MFS patients may serve as an acceptable source of kidney donors. However, the willingness and general conditions of the recipients should be carefully evaluated before kidney transplantation. Intraoperatively, potential risk of tear of renal arterial media should be properly treated. Extensive attention should be paid to the incidence of postoperative complications.
2.Effect of type of carrier material on the in vitro properties of solid dispersions of progesterone
Jing-nan QUAN ; Yi CHENG ; Jing-yu ZHOU ; Meng LI ; Zeng-ming WANG ; Nan LIU ; Zi-ming ZHAO ; Hui ZHANG ; Ai-ping ZHENG
Acta Pharmaceutica Sinica 2024;59(3):735-742
This study investigated the effect of different carrier materials on the
3.The evaluation of alpha-fetoprotein response on efficacy and prognosis in targeted therapy combined with immunotherapy for intermediate-to-advanced hepatocellular carcinoma: a multicenter clinical study
Kongying LIN ; Qingjing CHEN ; Luobin GUO ; Yun YANG ; Yufeng CHEN ; Jianxi ZHANG ; Fuqun WEI ; Hui ZHANG ; Zhiqing CHENG ; Yuntong LI ; Congren WANG ; Yabin JIANG ; Kecan LIN ; Weiping ZHOU ; Yongyi ZENG
Chinese Journal of Digestive Surgery 2024;23(2):248-256
Objective:To investigate the evaluation efficacy and predictive prognostic value of alpha-fetoprotein (AFP) response in tyrosine kinase inhibitors (TKIs) in combination with PD-1 inhibitors (α-PD-1) for intermediate-to-advanced hepatocellular carcinoma (HCC).Methods:The retrospective cohort study was conducted. The clinicopathological data of 205 patients with intermediate-to-advanced HCC who were admitted to 9 medical centers, including Mengchao Hepatobiliary Hospital of Fujian Medical University et al, from March 2020 to July 2022 were collected. There were 178 males and 27 females, aged (52±12)years. Based on AFP response at 6-8 weeks after treatment, patients were divided into the AFP response group (AFP level decreased by ≥50% compared to baseline) and the AFP no response group (AFP level decreased by <50% compared to baseline). Observation indicators: (1) AFP response evaluation of anti-tumor efficacy; (2) comparison of patient prognosis; (3) analysis of factors affecting patient prognosis. Measurement data with normal distrubution were represented as Mean± SD, and measurement data with skewed distribution were represented as M(range) and M( Q1, Q3). Count data were described as absolute numbers, and comparison between groups was conducted using the chi-square test. The Kaplan-Meier method was used to draw survival curve and calculate survival rate, and the Log-Rank test was used for survival analysis. The COX proportional risk model was used for univariate analysis and the COX stepwise regression model was used for multivariate analysis. Results:(1) AFP response evaluation of anti-tumor efficacy. Before treatment, all 205 patients were positive of AFP, with a baseline AFP level of 1 560(219,3 400)μg/L. All 205 patients were treated with TKIs in combination with α-PD-1, and the AFP level was 776(66,2 000)μg/L after 6 to 8 weeks of treatment. Of the 205 patients, 88 cases were classified as AFP response and 117 cases were classified as AFP no response. According to the response evaluation criteria in solid tumors version 1.1, the objective response rate (ORR) and disease control rate (DCR) were 42.05%(37/88) and 94.32%(83/88) in patients of the AFP response group and 16.24% (19/117) and 64.10% (75/117) in patients of the AFP no response group, showing significant differences between them ( χ2=16.846, 25.950, P<0.05). According to the modified response evaluation criteria in solid tumors, the ORR and DCR were 69.32% (61/88) and 94.32% (83/88) in patients of the AFP response group and 33.33% (39/117) and 64.10% (75/117) in patients of the AFP no response group, showing significant differences between them ( χ2=26.030, 25.950, P<0.05). (2) Comparison of patient prognosis. All 205 patients were followed up for 12.4(range, 2.4-34.0)months after treatment. The median progression free survival time and total survival time were 5.5 months and 17.8 months, respectively. The 1-year, 2-year progression free survival rates were 20.8% and 7.2%, and the 1-year, 2-year overall survival rates were 68.7% and 31.5%, respectively. The median progression free survival time, 1-year and 2-year progression free survival rates were 9.7 months, 39.6% and 14.2% in patients of the AFP response group and 3.7 months, 7.8% and 2.0% in patients of the AFP no response group, showing a significant difference in progression free survival between them ( χ2=43.154, P<0.05). The median overall survival time, 1-year and 2-year overall survival rates were not reached, 85.2% and 56.3% in patients of the AFP response group and 14.6 months, 56.3% and 14.5% in patients of the AFP no response group, showing a significant difference in overall survival between them ( χ2=33.899, P<0.05). (3) Analysis of factors affecting patient prognosis. Results of multivariate analysis showed that invasion of large blood vessels, extrahepatic metastasis, combined hepatic artery intervention therapy, and AFP response were independent factors influencing progression free survival in patients with intermediate-to-advanced HCC who were treated with TKIs in combination with α-PD-1 ( hazard ratio=1.474, 1.584, 0.631, 0.367, 95% confidence interval as 1.069-2.033, 1.159-2.167, 0.446-0.893, 0.261-0.516, P<0.05), and Eastern Cooperative Oncology Group score, invasion of large blood vessels, extrahepatic metastasis, and AFP response were independent factors influencing overall survival in patients with intermediate-to-advanced HCC who were treated with TKIs in combination with α-PD-1 ( hazard ratio= 1.347, 1.914, 1.673, 0.312, 95% confidence interval as 1.041-1.742, 1.293-2.833, 1.141-2.454, 0.197-0.492, P<0.05). Conclusions:AFP response at 6-8 weeks after treatment can effectively evaluate anti-tumor efficacy of TKIs in combination with α-PD-1 for intermediate-to-advanced HCC. AFP response is the independent factor influencing progression free survival and overall survival in patients with intermediate-to-advanced HCC who were treated with TKIs in combination with α-PD-1.
4.Effects of IL-37a expression up-regulation on lung function and Th1/Th2 balance in rats with acute respiratory distress syndrome
Bo XIONG ; Yijing CEN ; Linhai PAN ; Ming JIAN ; Qianglin ZENG ; Hui ZHOU
Immunological Journal 2024;40(3):272-278
This study was designed to explore the effect of up-regulation of interleukin(IL)-37a expression on lung function and T helper type 1(Th1)/Th2 cytokine balance in rats with acute respiratory distress syndrome(ARDS).Mesenchymal stem cells(MSCs)overexpressing IL-37a were constructed and co-cultured with mononuclear cells,then flow cytometry was used to detect the effect of IL-37a overexpression on Th1 and Th2 expression,and ELISA was used to detect IFN-γ and IL-4 levels in supernatant of the culture medium.Total of 30 SD rats were recruited and randomly divided into control group(sham surgery),ARDS group(lipopolysaccharideinduced establishment of ARDS model),and IL-37a group(ARDS model+tail vein injection of 10 μg/kg IL-37a),with 10 rats in each group.After 12 hours of modeling,arterial oxygen pressure(PaO2)and oxygenation index(PaO2/FiO2)were measured using a blood gas analyzer,and the dry/wet(W/D)ratio of the lungs was measured.HE staining was used to observe lung tissue pathology and evaluate lung pathological injury scores;ELISA was used to detect alveolar lavage fluid(BALF)and serum IL-4 and IFN-γ expression;flow cytometry was used to detect spleen Th1/Th2;while Western blot was used to detect the protein expression of NF-κB and NLRP3 in lung tissue.Data showed that the levels of Th1,Th1/Th2,and IL-4 in the peripheral cells of the overexpression IL-37a group were lower than those in the negative control group cells,while the expression of Th2 and IFN-γ were higher than those in the negative control group cells(P<0.05).Compared with the control group,the ARDS group showed lower levels of PaO2,PaO2/FiO2,BALF and serum IL-4,but higher levels of W/D,lung pathological injury score,BALF and serum IFN-γ,spleen Th1/Th2,and lung tissue NF-κB and NLRP3 proteins.IL-37a could reverse the changes mentioned above in ARDS rats(P<0.05).Taken together,up-regulation of IL-37a expression can ameliorate lung injury in ARDS rats,which may be related to the role of IL-37a in inducing MSC differentiation and promoting the restoration of Th1/Th2 balance.
5.Accuracy and clinical efficacy of robot-assisted transiliac-transsacral screw placement for posterior pelvic ring frac-tures
Xue-Lin WANG ; Zheng-Jie WU ; Yan-Hui ZENG ; Can-Hui LI ; Jian-Sheng ZHOU ; Shi HONG
China Journal of Orthopaedics and Traumatology 2024;37(6):605-608
Objective To explore accuracy and clinical effect of robot-assisted implantation of sacroiliac penetrating screw in orthopedic surgery for posterior pelvic ring fracture.Methods The clinical data of 24 patients with posterior pelvic ring frac-ture treated with robot-assisted sacroiliac penetration screws from August 2022 to August 2023 were retrospectively analyzed,including 10 males and 14 females;aged from 21 to 73 years old with an average of(49.29±14.48)years old;according to Tile pelvic fractures,13 patients were type B and 11 were type C.The effect of screw placement was evaluated according to Gras criteria based on postoperative CT scan results.At the final follow-up,fracture healing was evaluated according to Matta score,and functional recovery was evaluated by Majeed score.Results All patients were followed up for 3 to 13 months with an aver-age of(6.00±3.28)months.Totally 36 sacroiliac penetrating screws,18 S1 penetrating screws,18 S2 penetrating screws were inserted,a total of 29 were excellent and 7 good according to Gras standard.Screw adjustment times was 0.00(0.00,0.75)times.At the final follow-up,Matta score was excellent in 18 patients,5 good and 1 moderate,and the maximum displacement distance was 2.55(0.00,5.65)mm.Majeed score was 84.37±8.38,15 patients were excellent,7 good and 2 moderate.Con-clusion Robot could accurately and safely assist in the placement of sacroiliac joint screws for the treatment of posterior pelvic ring fractures,and promote postoperative functional recovery of patients.
6.Role of neoadjuvant rectal score in prognosis and adjuvant chemotherapy decision-making in locally advanced rectal cancer following neoadjuvant short-course radiotherapy and consolidation chemotherapy
Qiang ZENG ; Yuan TANG ; Haitao ZHOU ; Ning LI ; Wenyang LIU ; Silin CHEN ; Shuai LI ; Ningning LU ; Hui FANG ; Shulian WANG ; Yueping LIU ; Yongwen SONG ; Yexiong LI ; Jing JIN
Chinese Journal of Oncology 2024;46(4):335-343
Objectives:To assess the prognostic impact of the neoadjuvant rectal (NAR) score following neoadjuvant short-course radiotherapy and consolidation chemotherapy in locally advanced rectal cancer (LARC), as well as its value in guiding decisions for adjuvant chemotherapy.Methods:Between August 2015 and August 2018, patients were eligible from the STELLAR phase III trial (NCT02533271) who received short-course radiotherapy plus consolidation chemotherapy and for whom the NAR score could be calculated. Based on the NAR score, patients were categorized into low (<8), intermediate (8-16), and high (>16) groups. The Kaplan-Meier method, log rank tests, and multivariate Cox proportional hazard regression models were used to evaluate the impact of the NAR score on disease-free survival (DFS).Results:Out of the 232 patients, 24.1%, 48.7%, and 27.2% had low (56 cases), intermediate (113 cases), and high NAR scores (63 cases), respectively. The median follow-up period was 37 months, with 3-year DFS rates of 87.3%, 68.3%, and 53.4% ( P<0.001) for the low, intermediate, and high NAR score groups. Multivariate analysis demonstrated that the NAR score (intermediate NAR score: HR, 3.10, 95% CI, 1.30-7.37, P=0.011; high NAR scores: HR=5.44, 95% CI, 2.26-13.09, P<0.001), resection status ( HR, 3.00, 95% CI, 1.64-5.52, P<0.001), and adjuvant chemotherapy ( HR, 3.25, 95% CI, 2.01-5.27, P<0.001) were independent prognostic factors for DFS. In patients with R0 resection, the 3-year DFS rates were 97.8% and 78.0% for those with low and intermediate NAR scores who received adjuvant chemotherapy, significantly higher than the 43.2% and 50.6% for those who did not ( P<0.001, P=0.002). There was no significant difference in the 3-year DFS rate (54.2% vs 53.3%, P=0.214) among high NAR score patients, regardless of adjuvant chemotherapy. Conclusions:The NAR score is a robust prognostic indicator in LARC following neoadjuvant short-course radiotherapy and consolidation chemotherapy, with potential implications for subsequent decisions regarding adjuvant chemotherapy. These findings warrant further validation in studies with larger sample sizes.
7.Role of neoadjuvant rectal score in prognosis and adjuvant chemotherapy decision-making in locally advanced rectal cancer following neoadjuvant short-course radiotherapy and consolidation chemotherapy
Qiang ZENG ; Yuan TANG ; Haitao ZHOU ; Ning LI ; Wenyang LIU ; Silin CHEN ; Shuai LI ; Ningning LU ; Hui FANG ; Shulian WANG ; Yueping LIU ; Yongwen SONG ; Yexiong LI ; Jing JIN
Chinese Journal of Oncology 2024;46(4):335-343
Objectives:To assess the prognostic impact of the neoadjuvant rectal (NAR) score following neoadjuvant short-course radiotherapy and consolidation chemotherapy in locally advanced rectal cancer (LARC), as well as its value in guiding decisions for adjuvant chemotherapy.Methods:Between August 2015 and August 2018, patients were eligible from the STELLAR phase III trial (NCT02533271) who received short-course radiotherapy plus consolidation chemotherapy and for whom the NAR score could be calculated. Based on the NAR score, patients were categorized into low (<8), intermediate (8-16), and high (>16) groups. The Kaplan-Meier method, log rank tests, and multivariate Cox proportional hazard regression models were used to evaluate the impact of the NAR score on disease-free survival (DFS).Results:Out of the 232 patients, 24.1%, 48.7%, and 27.2% had low (56 cases), intermediate (113 cases), and high NAR scores (63 cases), respectively. The median follow-up period was 37 months, with 3-year DFS rates of 87.3%, 68.3%, and 53.4% ( P<0.001) for the low, intermediate, and high NAR score groups. Multivariate analysis demonstrated that the NAR score (intermediate NAR score: HR, 3.10, 95% CI, 1.30-7.37, P=0.011; high NAR scores: HR=5.44, 95% CI, 2.26-13.09, P<0.001), resection status ( HR, 3.00, 95% CI, 1.64-5.52, P<0.001), and adjuvant chemotherapy ( HR, 3.25, 95% CI, 2.01-5.27, P<0.001) were independent prognostic factors for DFS. In patients with R0 resection, the 3-year DFS rates were 97.8% and 78.0% for those with low and intermediate NAR scores who received adjuvant chemotherapy, significantly higher than the 43.2% and 50.6% for those who did not ( P<0.001, P=0.002). There was no significant difference in the 3-year DFS rate (54.2% vs 53.3%, P=0.214) among high NAR score patients, regardless of adjuvant chemotherapy. Conclusions:The NAR score is a robust prognostic indicator in LARC following neoadjuvant short-course radiotherapy and consolidation chemotherapy, with potential implications for subsequent decisions regarding adjuvant chemotherapy. These findings warrant further validation in studies with larger sample sizes.
8.Randomized controlled study on treatment of intertrochanteric fracture of femur with proximal femoral nail antirotation assisted by robot navigation
Xi-Cong CHEN ; Jian CAI ; Hui-Liang ZENG ; Guo-Cai CHEN ; Guan-Ming ZHOU
Journal of Regional Anatomy and Operative Surgery 2024;33(9):768-772
Objective To investigate the therapeutic effects of proximal femoral nail antirotation(PFNA)assisted by robot navigation and PFNA guided by C-arm X-ray machine for the treatment of intertrochanteric fracture of femur(IFOF).Methods The 100 patients with unilateral IFOF in our hospital were selected as the study subjects.They were divided into the control group(50 cases)and the robot-assisted group(50 cases)according to random number table method.The control group was treated with PFNA internal fixation under C-arm X-ray machine fluoroscopy,and the robot-assisted group was treated with PFNA internal fixation assisted by robot navigation.Surgical indicators,fracture reduction and fracture healing,stress response indexes,hip joint function,quality of life score and incidence of surgery related complications were compared between the two groups.Results The operation time,total fluoroscopy time,nail placement time and fracture healing time of the robot-assisted group were shorter than those of the control group(P<0.05);and the intraoperative blood loss,guide needle adjustment frequency,and fluoroscopy frequency of the robot-assisted group were less than those of the control group(P<0.05).The fracture reduction and fracture healing in the robot-assisted group were better than those in the control group(P<0.05).Two days after operation,the levels of serum norepinephrine(NE),angiotensin Ⅱ(Ang Ⅱ)and superoxide dismutase(SOD)in the two groups were higher than those before operation(P<0.05),and the levels of serum NE,Ang Ⅱ and SOD in the robot-assisted group were lower than those in the control group(P<0.05).The Harris score and 36-item short-form(SF-36)score of patients 7 days and 3 months after surgery in the two groups were higher than those before surgery(P<0.05),and the above scores 3 months after surgery were higher than those 7 days after surgery(P<0.05).Harris score and SF-36 score 7 days and 3 months after operation in the robot-assisted group were higher than those in the control group(P<0.05).The incidence of complications in the robot-assisted group was lower than that in the control group(P<0.05).Conclusion Compared with the C-arm X-ray machine fluoroscopy,the treatment of IFOF with PFNA assisted by robot navigation can further shorten the operation time,reduce the surgical trauma and the incidence of complications,achieve better effect of fracture reduction and healing,reduce stress response of the body,and improve hip joint function and quality of life for patients.
9.Treatment status of tyrosine kinase inhibitor for newly-diagnosed chronic myeloid leukemia: a domestic multi-centre retrospective real-world study
Xiaoshuai ZHANG ; Bingcheng LIU ; Xin DU ; Yanli ZHANG ; Na XU ; Xiaoli LIU ; Weiming LI ; Hai LIN ; Rong LIANG ; Chunyan CHEN ; Jian HUANG ; Yunfan YANG ; Huanling ZHU ; Ling PAN ; Xiaodong WANG ; Guohui LI ; Zhuogang LIU ; Yanqing ZHANG ; Zhenfang LIU ; Jianda HU ; Chunshui LIU ; Fei LI ; Wei YANG ; Li MENG ; Yanqiu HAN ; Li'e LIN ; Zhenyu ZHAO ; Chuanqing TU ; Caifeng ZHENG ; Yanliang BAI ; Zeping ZHOU ; Suning CHEN ; Huiying QIU ; Lijie YANG ; Xiuli SUN ; Hui SUN ; Li ZHOU ; Zelin LIU ; Danyu WANG ; Jianxin GUO ; Liping PANG ; Qingshu ZENG ; Xiaohui SUO ; Weihua ZHANG ; Yuanjun ZHENG ; Qian JIANG
Chinese Journal of Hematology 2024;45(3):215-224
Objective:To retrospectively analyze the treatment status of tyrosine kinase inhibitors (TKI) in newly diagnosed patients with chronic myeloid leukemia (CML) in China.Methods:Data of chronic phase (CP) and accelerated phase (AP) CML patients diagnosed from January 2006 to December 2022 from 77 centers, ≥18 years old, and receiving initial imatinib, nilotinib, dasatinib or flumatinib-therapy within 6 months after diagnosis in China with complete data were retrospectively interrogated. The choice of initial TKI, current TKI medications, treatment switch and reasons, treatment responses and outcomes as well as the variables associated with them were analyzed.Results:6 893 patients in CP ( n=6 453, 93.6%) or AP ( n=440, 6.4%) receiving initial imatinib ( n=4 906, 71.2%), nilotinib ( n=1 157, 16.8%), dasatinib ( n=298, 4.3%) or flumatinib ( n=532, 7.2%) -therapy. With the median follow-up of 43 ( IQR 22-75) months, 1 581 (22.9%) patients switched TKI due to resistance ( n=1 055, 15.3%), intolerance ( n=248, 3.6%), pursuit of better efficacy ( n=168, 2.4%), economic or other reasons ( n=110, 1.6%). The frequency of switching TKI in AP patients was significantly-higher than that in CP patients (44.1% vs 21.5%, P<0.001), and more AP patients switched TKI due to resistance than CP patients (75.3% vs 66.1%, P=0.011). Multi-variable analyses showed that male, lower HGB concentration and ELTS intermediate/high-risk cohort were associated with lower cytogenetic and molecular responses rate and poor outcomes in CP patients; higher WBC count and initial the second-generation TKI treatment, the higher response rates; Ph + ACA at diagnosis, poor PFS. However, Sokal intermediate/high-risk cohort was only significantly-associated with lower CCyR and MMR rates and the poor PFS. Lower HGB concentration and larger spleen size were significantly-associated with the lower cytogenetic and molecular response rates in AP patients; initial the second-generation TKI treatment, the higher treatment response rates; lower PLT count, higher blasts and Ph + ACA, poorer TFS; Ph + ACA, poorer OS. Conclusion:At present, the vast majority of newly-diagnosed CML-CP or AP patients could benefit from TKI treatment in the long term with the good treatment responses and survival outcomes.
10.Analysis and summary of clinical characteristics of 289 patients with paroxysmal nocturnal hemoglobinuria in Zhejiang Province
Gaixiang XU ; Weimei JIN ; Baodong YE ; Songfu JIANG ; Chao HU ; Xin HUANG ; Bingshou XIE ; Huifang JIANG ; Lili CHEN ; Rongxin YAO ; Ying LU ; Linjie LI ; Jin ZHANG ; Guifang OUYANG ; Yongwei HONG ; Hongwei KONG ; Zhejun QIU ; Wenji LUO ; Binbin CHU ; Huiqi ZHANG ; Hui ZENG ; Xiujie ZHOU ; Pengfei SHI ; Ying XU ; Jie JIN ; Hongyan TONG
Chinese Journal of Hematology 2024;45(6):549-555
Objective:To further improve the understanding of paroxysmal nocturnal hemoglobinuria (PNH), we retrospectively analyzed and summarized the clinical characteristics, treatment status, and survival status of patients with PNH in Zhejiang Province.Methods:This study included 289 patients with PNH who visited 20 hospitals in Zhejiang Province. Their clinical characteristics, comorbidity, laboratory test results, and medications were analyzed and summarized.Results:Among the 289 patients with PNH, 148 males and 141 females, with a median onset age of 45 (16-87) years and a peak onset age of 20-49 years (57.8% ). The median lactic dehydrogenase (LDH) level was 1 142 (604-1 925) U/L. Classified by type, 70.9% (166/234) were classical, 24.4% (57/234) were PNH/bone marrow failure (BMF), and 4.7% (11/234) were subclinical. The main clinical manifestations included fatigue or weakness (80.8%, 235/289), dizziness (73.4%, 212/289), darkened urine color (66.2%, 179/272), and jaundice (46.2%, 126/270). Common comorbidities were hemoglobinuria (58.7% ), renal dysfunction (17.6% ), and thrombosis (15.0% ). Moreover, 82.3% of the patients received glucocorticoid therapy, 70.9% required blood transfusion, 30.7% used immunosuppressive agents, 13.8% received anticoagulant therapy, and 6.3% received allogeneic hematopoietic stem cell transplantation. The 10-year overall survival (OS) rate was 84.4% (95% CI 78.0% -91.3% ) . Conclusion:Patients with PNH are more common in young and middle-aged people, with a similar incidence rate between men and women. Common clinical manifestations include fatigue, hemoglobinuria, jaundice, renal dysfunction, and recurrent thrombosis. The 10-year OS of this group is similar to reports from other centers in China.

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