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The clinical data of 6 acute promyelocytic leukemia (APL) patients with thrombosis as the first manifestation were retrospectively analyzed. Among 6 patients, 5 were males and 1 female.The median age at diagnosis was 55 years old. All patients had risk factors for cardiovascular disease (CVD), and 5 patients met the diagnostic criteria for disseminated intravascular coagulation (DIC). There were 3 patients at low risk (bcr1 subtype), 1 at intermediate risk (bcr2 subtype) and 2 at high risk (1 bcr3 subtype and 1 unknown). FLT3-ITD mutations were tested in 3 cases, all of whom showed negative results. Arterial thrombosis was found in all 6 cases, 4 cases had cerebral infarction, 1 had lower limb arterial embolism, and 1 had multiple arterial and venous thrombosis. Four patients with cerebral infarction received all-trans retinoic acid (ATRA) combined with arsenic trioxide (ATO)±chemotherapy and symptomatic treatment (1 patient at high risk did not receive ATRA), 2 patients achieved complete remission (CR), and the other 2 patients died of cerebral hemorrhage and cerebral infarction, respectively. One patient with lower extremity arterial thrombosis died suddenly within 12 h after admission likely due to acute myocardial infarction. One patient with mixed thrombosis received low molecular weight heparin and rivaroxaban successively after inferior vena cava filter implantation, and achieved CR after ATRA+ATO treatment. Thrombosis is a less common and under-recognized presentation in APL.Thrombosis patients with blood cells and/or coagulation abnormalities should consider the possibility of APL. APL patients complicated with thrombosis have a high probability of DIC and remain mostly intractable to existing treatments, who are at high risk of death and poor prognosis.
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OBJECTIVE@#To analyze the sequence of the F12 gene and molecular mechanism for 20 patients with coagulation factor Ⅻ (FⅫ) deficiency.@*METHODS@#The patients were selected from the outpatient department of the Second Hospital of Shanxi Medical University from July 2020 to January 2022. The activity of coagulation factor Ⅷ (FⅧ:C), factor Ⅸ (FⅨ:C), factor Ⅺ (FⅪ:C) and factor Ⅻ (FⅫ:C) were determined by using a one-stage clotting assay. All exons and 5' and 3' UTR of the F12 gene were analyzed by Sanger sequencing to detect the potential variants. Bioinformatic software was used to predict the pathogenicity of the variants, conservation of amino acids, and protein models.@*RESULTS@#The FⅫ:C of the 20 patients has ranged from 0.07% to 20.10%, which was far below the reference values, whilst the other coagulation indexes were all normal. Sanger sequencing has identified genetic variants in 10 patients, including 4 with missense variants [c.820C>T (p.Arg274Cys), c.1561G>A (p.Glu521Lys), c.181T>C (p.Cys61Arg) and c.566.G>C (p.Cys189Ser)], 4 deletional variants c.303_304delCA(p.His101GlnfsX36), 1 insertional variant c.1093_1094insC (p.Lys365GlnfsX69) and 1 nonsense variant c.1763C>A (p.Ser588*). The remaining 10 patients only harbored the 46C/T variant. The heterozygous c.820C>T(p.Arg274Cys) missense variant in patient 1 and the homozygous c.1763C>A (p.Ser588*) nonsense variant in patient 2 were not included in the ClinVar and the Human Gene Mutation Database. Bioinformatic analysis predicted that both variants were pathogenic, and the corresponding amino acids are highly conserved. The protein prediction models suggested that the c.820C>T (p.Arg274Cys) variant may affect the stability of the secondary structure of FⅫ protein by disrupting the original hydrogen bonding force and truncating the side chain, leading to changes in the vital domain. c.1763C>A (p.Ser588*) may produce a truncated C-terminus which may alter the spatial conformation of the protein domain and affect the serine protease cleavage site, resulting in extremely reduced FⅫ:C.@*CONCLUSION@#Among individuals with low low FⅫ:C detected by one-stage clotting assay, 50% have harbored variants of the F12 gene, among which the c.820C>T and c.1763C>A were novel variants underlying the reduced coagulating factor FⅫ.
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Humans , Factor XII/genetics , Pedigree , Mutation , Mutation, Missense , Heterozygote , Factor XII Deficiency/geneticsABSTRACT
Objective:To investigate the clinical characteristics, influencing factors and prevention and treatment measures of nosocomial infection in patients with acute myeloid leukemia (AML) (non-acute promyelocytic leukemia) after applying intermediate-high dose cytarabine (Ara-C) chemotherapy.Methods:The clinical data of 80 patients with AML treated with intermediate-high dose Ara-C in the Second Hospital of Shanxi Medical University from March 2013 to January 2020 were analyzed retrospectively. The clinical features of nosocomial infection were summarized and the influencing factors of infection were analyzed by using multivariate logistic regression.Results:A total of 80 patients received 198 times of chemotherapy, and the infection rate was 72.7% (144/198). Infection sites mainly included respiratory tract infection, pulmonary infection, gastrointestinal infection. A total of 45 strains of pathogenic bacterias were detected, among which Gram negative bacilli accounted for 55.6% (25/45), Gram positive cocci accounted for 24.4% (11/45), fungi accounted for 8.9% (4/45) and viruses accounted for 11.1% (5/45). There were no significant differences in infection rate, hospitalization time, neutrophils recovery time and hospitalization expenses between the sterile laminar flow ward and the general ward (all P > 0.05). Multivariate logistic regression analysis showed that infection during induction chemotherapy was independent risk factor of infection ( OR = 5.076, 95% CI 1.978-13.022, P =0.001), and antibiotic prevention was independent protective factor of nosocomial infection ( OR = 0.332, 95% CI 0.136-0.803, P = 0.014). Conclusions:The infection rate of AML patients receiving intermediate-high dose Ara-C chemotherapy is high. During the treatment, we should be alert to the infection during induction chemotherapy and use antibiotics to prevent it in time. For patients undergoing intermediate-high dose Ara-C chemotherapy, strengthening the environmental cleanliness of general wards may achieve the same preventive effect as that of sterile laminar flow wards.
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Objective:To study the ultrasonographic characteristics of embryos/fetuses with normal or abnormal central nervous system (CNS) from 7 to 13 +6 weeks of gestation using high resolution two-dimensional ultrasound combined with HD-live silhouette technology and provide a reference for early diagnosis of CNS abnormalities. Methods:Eighty normal embryos/fetuses during 7-13 +6 weeks and 41 fetuses with CNS malformations in early pregnancy during 11-13 +6 weeks were selected to observe the ultrasonographic features of embryos/fetuses with normal or abnormal CNS using transvaginal high resolution two-dimensional ultrasound and HD-live silhouette technology. Descriptive analysis was performed on the results. Results:From seven weeks of gestational age, high resolution two-dimensional ultrasound combined with HD-live silhouette technology can clearly and stereoscopically show the prosencephalon, mesencephalon and rhombencephalon. The rhombencephalon changed the most in the brain development of embryos. At nine weeks of gestation, cleared structures of pons curvature, the fourth ventricle and cisterna magna were observed. The developing cerebellum and the original Blake pouch cyst were seen at 10 weeks of gestation. From 11 to 13 +6 weeks, the most remarkable change was the choroid plexus of the fourth ventricle changed from perpendicular to parallel to the long axis of the neural tube. Of the 41 fetuses with CNS malformation, 16 (39.0%) were exencephaly, 11 (26.8%) were holoprosencephaly, five (12.2%) were encephalocele, four (9.7%) were anencephaly, three (7.3%) were fourth ventricle dilatation, and two (4.9%) were open spina bifida. Conclusions:High resolution two-dimensional ultrasound combined with HD-live silhouette technology can clearly and stereoscopically display the morphological changes in embryonic embryos/fetuses with development of normal CNS at 7-13 +6 weeks, which is helpful to better understand the origin of CNS embryonic abnormalities and provide diagnostic clues for the early detection of CNS abnormalities.
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Objective:To investigate the epidemiological characteristics, diagnosis, treat-ment and prognosis of gallbladder cancer in China from 2010 to 2017.Methods:The single disease retrospective registration cohort study was conducted. Based on the concept of the real world study, the clinicopathological data, from multicenter retrospective clinical data database of gallbladder cancer of Chinese Research Group of Gallbladder Cancer (CRGGC), of 6 159 patients with gallbladder cancer who were admitted to 42 hospitals from January 2010 to December 2017 were collected. Observation indicators: (1) case resources; (2) age and sex distribution; (3) diagnosis; (4) surgical treatment and prognosis; (5) multimodality therapy and prognosis. The follow-up data of the 42 hospitals were collected and analyzed by the CRGGC. The main outcome indicator was the overall survival time from date of operation for surgical patients or date of diagnosis for non-surgical patients to the end of outcome event or the last follow-up. Measurement data with normal distribu-tion were represented as Mean±SD, and comparison between groups was conducted using the t test. Measurement data with skewed distribution were represented as M( Q1, Q3) or M(range), and com-parison between groups was conducted using the U test. Count data were described as absolute numbers or percentages, and comparison between groups was conducted using the chi-square test. Univariate analysis was performed using the Logistic forced regression model, and variables with P<0.1 in the univariate analysis were included for multivariate analysis. Multivariate analysis was performed using the Logistic stepwise regression model. The life table method was used to calculate survival rates and the Kaplan-Meier method was used to draw survival curves. Log-rank test was used for survival analysis. Results:(1) Case resources: of the 42 hospitals, there were 35 class A of tertiary hospitals and 7 class B of tertiary hospitals, 16 hospitals with high admission of gallbladder cancer and 26 hospitals with low admission of gallbladder cancer, respectively. Geographical distribution of the 42 hospitals: there were 9 hospitals in central China, 5 hospitals in northeast China, 22 hospitals in eastern China and 6 hospitals in western China. Geographical distribution of the 6 159 patients: there were 2 154 cases(34.973%) from central China, 705 cases(11.447%) from northeast China, 1 969 cases(31.969%) from eastern China and 1 331 cases(21.611%) from western China. The total average number of cases undergoing diagnosis and treatment in hospitals of the 6 159 patients was 18.3±4.5 per year, in which the average number of cases undergoing diagnosis and treatment in hospitals of 4 974 patients(80.760%) from hospitals with high admission of gallbladder cancer was 38.8±8.9 per year and the average number of cases undergoing diagnosis and treatment in hospitals of 1 185 patients(19.240%) from hospitals with low admission of gallbladder cancer was 5.7±1.9 per year. (2) Age and sex distribution: the age of 6 159 patients diagnosed as gallbladder cancer was 64(56,71) years, in which the age of 2 247 male patients(36.483%) diagnosed as gallbladder cancer was 64(58,71)years and the age of 3 912 female patients(63.517%) diagnosed as gallbladder cancer was 63(55,71)years. The sex ratio of female to male was 1.74:1. Of 6 159 patients, 3 886 cases(63.095%) were diagnosed as gallbladder cancer at 56 to 75 years old. There was a significant difference on age at diagnosis between male and female patients ( Z=-3.99, P<0.001). (3) Diagnosis: of 6 159 patients, 2 503 cases(40.640%) were initially diagnosed as gallbladder cancer and 3 656 cases(59.360%) were initially diagnosed as non-gallbladder cancer. There were 2 110 patients(34.259%) not undergoing surgical treatment, of which 200 cases(9.479%) were initially diagnosed as gallbladder cancer and 1 910 cases(90.521%) were initially diagnosed as non-gallbladder cancer. There were 4 049 patients(65.741%) undergoing surgical treatment, of which 2 303 cases(56.878%) were initially diagnosed as gallbladder cancer and 1 746 cases(43.122%) were initial diagnosed as non-gallbladder cancer. Of the 1 746 patients who were initially diagnosed as non-gallbladder cancer, there were 774 cases(19.116%) diagnosed as gallbladder cancer during operation and 972 cases(24.006%) diagnosed as gallbladder cancer after operation. Of 6 159 patients, there were 2 521 cases(40.932%), 2 335 cases(37.912%) and 1 114 cases(18.087%) undergoing ultrasound, computed tomography (CT) or magnetic resonance imaging (MRI) examination before initial diagnosis, respec-tively, and there were 3 259 cases(52.914%), 3 172 cases(51.502%) and 4 016 cases(65.205%) undergoing serum carcinoembryonic antigen, CA19-9 or CA125 examination before initially diagnosis, respectively. One patient may underwent multiple examinations. Results of univariate analysis showed that geographical distribution of hospitals (eastern China or western China), age ≥72 years, gallbladder cancer annual admission of hospitals, whether undergoing ultrasound, CT, MRI, serum carcinoembryonic antigen, CA19-9 or CA125 examination before initially diagnosis were related factors influencing initial diagnosis of gallbladder cancer patients ( odds ratio=1.45, 1.98, 0.69, 0.68, 2.43, 0.41, 1.63, 0.41, 0.39, 0.42, 95% confidence interval as 1.21-1.74, 1.64-2.40, 0.59-0.80, 0.60-0.78, 2.19-2.70, 0.37-0.45, 1.43-1.86, 0.37-0.45, 0.35-0.43, 0.38-0.47, P<0.05). Results of multivariate analysis showed that geographical distribution of hospitals (eastern China or western China), sex, age ≥72 years, gallbladder cancer annual admission of hospitals and cases undergoing ultrasound, CT, serum CA19-9 examination before initially diagnosis were indepen-dent influencing factors influencing initial diagnosis of gallbladder cancer patients ( odds ratio=1.36, 1.42, 0.89, 0.67, 1.85, 1.56, 1.57, 0.39, 95% confidence interval as 1.13-1.64, 1.16-1.73, 0.79-0.99, 0.57-0.78, 1.60-2.14, 1.38-1.77, 1.38-1.79, 0.35-0.43, P<0.05). (4) Surgical treatment and prognosis. Of the 4 049 patients undergoing surgical treatment, there were 2 447 cases(60.435%) with complete pathological staging data and follow-up data. Cases with pathological staging as stage 0, stage Ⅰ, stage Ⅱ, stage Ⅲa, stage Ⅲb, stage Ⅳa and stage Ⅳb were 85(3.474%), 201(8.214%), 71(2.902%), 890(36.371%), 382(15.611%), 33(1.348%) and 785(32.080%), respectively. The median follow-up time and median postoperative overall survival time of the 2 447 cases were 55.75 months (95% confidence interval as 52.78-58.35) and 23.46 months (95% confidence interval as 21.23-25.71), respectively. There was a significant difference in the overall survival between cases with pathological staging as stage 0, stage Ⅰ, stage Ⅱ, stage Ⅲa, stage Ⅲb, stage Ⅳa and stage Ⅳb ( χ2=512.47, P<0.001). Of the 4 049 patients undergoing surgical treatment, there were 2 988 cases(73.796%) with resectable tumor, 177 cases(4.371%) with unresectable tumor and 884 cases(21.833%) with tumor unassessable for resectabi-lity. Of the 2 988 cases with resectable tumor, there were 2 036 cases(68.139%) undergoing radical resection, 504 cases(16.867%) undergoing non-radical resection and 448 cases(14.994%) with operation unassessable for curative effect. Of the 2 447 cases with complete pathological staging data and follow-up data who underwent surgical treatment, there were 53 cases(2.166%) with unresectable tumor, 300 cases(12.260%) with resectable tumor and receiving non-radical resection, 1 441 cases(58.888%) with resectable tumor and receiving radical resection, 653 cases(26.686%) with resectable tumor and receiving operation unassessable for curative effect. There were 733 cases not undergoing surgical treatment with complete pathological staging data and follow-up data. There was a significant difference in the overall survival between cases not undergoing surgical treatment, cases undergoing surgical treatment for unresectable tumor, cases undergoing non-radical resection for resectable tumor and cases undergoing radical resection for resectable tumor ( χ2=121.04, P<0.001). (5) Multimodality therapy and prognosis: of 6 159 patients, there were 541 cases(8.784%) under-going postoperative adjuvant chemotherapy and advanced chemotherapy, 76 cases(1.234%) under-going radiotherapy. There were 1 170 advanced gallbladder cancer (pathological staging ≥stage Ⅲa) patients undergoing radical resection, including 126 cases(10.769%) with post-operative adjuvant chemotherapy and 1 044 cases(89.231%) without postoperative adjuvant chemo-therapy. There was no significant difference in the overall survival between cases with post-operative adjuvant chemotherapy and cases without postoperative adjuvant chemotherapy ( χ2=0.23, P=0.629). There were 658 patients with pathological staging as stage Ⅲa who underwent radical resection, including 66 cases(10.030%) with postoperative adjuvant chemotherapy and 592 cases(89.970%) without postoperative adjuvant chemotherapy. There was no significant difference in the overall survival between cases with postoperative adjuvant chemotherapy and cases without postoperative adjuvant chemotherapy ( χ2=0.05, P=0.817). There were 512 patients with pathological staging ≥stage Ⅲb who underwent radical resection, including 60 cases(11.719%) with postoperative adjuvant chemotherapy and 452 cases(88.281%) without postoperative adjuvant chemotherapy. There was no significant difference in the overall survival between cases with postoperative adjuvant chemo-therapy and cases without post-operative adjuvant chemo-therapy ( χ2=1.50, P=0.220). Conclusions:There are more women than men with gallbladder cancer in China and more than half of patients are diagnosed at the age of 56 to 75 years. Cases undergoing ultrasound, CT, serum CA19-9 examination before initial diagnosis are independent influencing factors influencing initial diagnosis of gallbladder cancer patients. Preoperative resectability evaluation can improve the therapy strategy and patient prognosis. Adjuvant chemotherapy for gallbladder cancer is not standardized and in low proportion in China.
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Objective:To investigate the pathogen distribution and antimicrobial resistance among lower respiratory tract infections in patients with hematological malignancies.Methods:Sputum samples were collected from 967 patients with hematological malignancies and lower respiratory tract infections in Department of Hematology,the Second Hospital of Shanxi Medical University from January 2017 to July 2020. The pathogens and drug sensitivity reports were carried out by automatic bacterial identification instruments. WHONET 5.6 and SPSS 20.0 softwares were used for statistical analysis.Results:A total of 961 strains of pathogens were isolated, 516 (53.7%) pathogens were Gram-negative bacteria, mainly 118 strains of Klebsiella pneumonia (12.3%), 68 strains of Pseudomonas aeruginosa (7.1%), 67 strains of Acinetobacter baumannii (7.0%),52 strains of Stenotrophomonas maltophilia (5.4%), 43 strains of Escherichia coli (4.5%), and 42 strains of Enterbacter cloacae (4.4%). There were 171 (17.8%) strains of Gram-positive bacteria and 274 (28.5%) fungi. The drug resistance rates of Pseudomonas aeruginosa and Acinetobacter baumannii to carbapenem were 22.1%-31.3%. Stenotrophomonas maltophilia was sensitive to levofloxacin, compound sulfamethoxazole and minocycline. The antimicrobial resistance rates of these three enterobacteria to carbapenems, cefoperazone/sulbactam, piperacillin/tazobactam were low (<10%). The resistant Gram-positive bacteria to ticoplanin, vancomycin and linazolamide were not detected.Conclusion:The major pathogens related to lower respiratory tract infections in patients with hematological malignancies are gram-negative bacteria in our centre. Different pathogens appear different characteristics of antimicrobial resistance.
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Objective:To investigate the effect of miRNA-618 (miR-618) on the cell proliferation and apoptosis of acute monocyte leukemia THP-1 cells.Methods:Real-time polymerase chain reaction (PCR) was used to detect the relative expression level of miR-618 in THP-1 cells and monocytes isolated from peripheral blood of the healthy people. Overexpression of miR-618 plasimid vector was constructed and empty vector was treated as the negative control; and then the two vectors were transfected with THP-1 cells; finally, miR-618 overexpression group and negative control group were set. THP-1 cell proliferation and apoptosis of both groups were detected by using CCK-8 method and flow cytometry, respectively. TargetScan was used to predict the target gene of miR-618 and it was verified by using luciferase reporter assay.Western blot was used to detect the protein levels of THP-1 cells in miR-618 overexpression group and negative control group, and predicted miR-618 target gene in peripheral blood monocytes of the healthy people.Results:PCR showed that the expression level of miR-618 was lower in THP-1 cells compared with that in monocytes isolated from peripheral blood of the healthy people ( P < 0.05). CCK-8 assay showed that compared with the negative control group, the proliferation ability of THP-1 cells in miR-618 overexpression group was decreased (the absorbance values at 0, 24, 48 and 72 h after transfection: 0.20±0.03 vs. 0.20±0.03, 0.28±0.02 vs. 0.35±0.03, 0.34±0.03 vs. 0.43±0.04, 0.39±0.02 vs. 0.53±0.05, all P < 0.05), and the late apoptosis rate was increased [(27.1±0.1)% vs. (14.9±0.1)%, t=2.13, P=0.03]. The target gene of miR-618 was ARPP19 predicted by using TargetScan software. Luciferase reporter assay showed that the relative luciferase activity of THP-1 cells in group transfected with wild-type ARPP19 gene plasmid+miR-618 gene plasmid was higher than that in the blank control group and group transfected with wild-type ARPP19 gene plasmid+miR-618 empty vector (0.170±0.003 vs. 0.100±0.004, 0.100±0.001, all P < 0.05). Western blot indicated the expression level of ARPP19 protein in THP-1 cells of miR-618 overexpression group was lower than that of the negative control group, while the expression levels of ARPP19 protein of peripheral blood monocytes of the healthy people in both groups were similar. Conclusion:miR-618 can inhibit the cell proliferation and promote apoptosis of THP-1 cells by inhibiting the expression of of THP-1 cells ARPP19 in acute monocyte leukemia.
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Objective To study the impact of peripheral blood absolute lymphocyte count ( ALC) on survival in adult patients with acute lymphoblastic leukemia ( ALL ) at the end of induction chemotherapy. Methods 122 adult patients with ALL from January 2011 to December 2017 in the Second Hospital of Shanxi Medical University were retrospectively analyzed. The impact of ALC at the end of induction and ALC ratios for relapse-free( RFS) and overall survival ( OS ) of adult ALL were analyzed. Results The study cohort was divided into the two groups according ALC-28 values ( <675cells/μL and ≥675cells/μL). The median RFS (8 months vs 12 months,P=0. 001),median OS (16 months vs 23 months,P=0. 010) between the two groups had statistically significant differ-ences. ALC-28/ALC-1 was bordered by 43％. The RFS and OS in the low ratio group were significantly shorter than those in the high ratio group (P<0. 05). ALC-28/ALC-8 was compared with 86％ cut-off. Longer RFS and OS in the higher ALC-28/ALC-8 group (all P<0. 05). Low ALC-28 was independent risk factor for RFS. Low ALC-28,and low ALC-28/ALC-1 were independent risk factors of OS. Conclusion ALC at the end of induction and ALC ratios are associated with RFS and OS of adult ALL. Higher the value,the longer the survival time.
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Objective To explore the role of E2A﹣HLF fusion gene in the prognosis evaluation of B﹣cell acute lymphoblastic leukemia (B﹣ALL), and to improve the accuracy of stratified treatment. Methods The clinical characteristics, treatment effect and survival time of two B﹣ALL patients with E2A﹣HLF fusion gene who were admitted to the Second Hospital of Shanxi Medical University were retrospectively analyzed, and the related literature was reviewed. Results The first patient was an 8 years old girl, developed with fever, abdominal pain, and slightly increased white blood cells (WBC), and also accompanied by hypercalcemia. Another patient was a 27 years old man, developed with jaw pain and anemia, WBC was normal. Precursor B﹣ALL (pre﹣B﹣ALL) was identified by flow cytometry (FCM) in the two cases. E2A﹣HLF fusion gene was screened out at first diagnosis for the girl, but found after relapse for the man. Both patients early received intensive treatment with high﹣dose methotrexate after the first complete remission, but relapsed after 3 and 6 months respectively. The girl did not receive allogeneic hematopoietic stem cell transplantation (allo﹣HSCT) after relapse and died of severe infection. The man received allo﹣HSCT complete remission, and maintained complete remission within 5 months after HSCT, E2A﹣HLF fusion gene was also negative, but eventually died of multiple transplantation﹣related complications. Conclusions E2A﹣HLF fusion gene occurs mostly in the pre﹣B﹣ALL patients with hypercalcemia, and it shows extremely poor prognosis with a high multidrug resistance rate and high early recurrence rate. The allo﹣HSCT can increase the leukemia﹣free survival rate, and the relapse and overall survival may be improved when these patients received allo﹣HSCT in the first complete remission.
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Objective To summarize the diagnosis and treatment of biliary pancreatic duct dilatation.Methods The retrospective and descriptive study was conducted.The clinical data of 22 patients with biliary pancreatic duct dilatation who were admitted to Renji Hospital of Shanghai Jiaotong University School of Medicine between October 2013 to September 2017 were collected.There were 6 males and 16 females,aged from 33 to 82years,with an average age of 66 years.Surgical exploration was decided according to clinical symptoms,results of laboratory test and imaging examinations.For patients with space occupying lesions,surgical procedure was selected based on results of pathological examination.Patients without surgical exploration or space occupying lesions were allocated into follow-up.Observation indicators:(1) surgical exploration;(2) relationship of clinical symptoms and preoperative examinations with surgical exploration positive for space occupying lesions;(3) surgical treatment;(4) follow-up.Follow-up using outpatient examination was performed on patients up to October 2018.Follow-up was performed on patients with positive surgical exploration to detect postoperative complications.For patients with positive results of imaging examinations,no jaundice,normal laboratory indicators or mild abnormality,liver function,tumor markers and B-ultrasound were re-examined each month,and computed tomography (CT) and magnetic resonance imaging (MRI) was performed once every 3 months.Surgical exploration was performed when total bilirubin (TBil) or tumor markers showed a progressive increase.Follow-up was performed on patients with negative results of imaging examination,jaundice,and mildly elevated CA19-9.TBil and CA19-9 were re-examined monthly,and if they were progressively elevated,patients were transferred to surgical exploration.For patients with negative results of imaging examination,no symptoms,and negative laboratory test,liver function,tumor markers,and B-ultrasound were re-examined once every 3 months,and enhanced CT and MRI were re-examined once every 6 months within one year.Follow-up was performed once every 6 months during the second year,and once a year after two years.Measurement data with normal distribution were represented as Mean±SD,and comparison between groups was analyzed using the t test.Count data were descibed as absolute numbers,and they were analyzed using the chi-square test under R×C chart or Fisher exact probability.Results (1) Surgical exploration:of 22 patients,11 underwent surgical exploration,and 11 underwent followup.Of the 11 patients with surgical exploration,4 were positive for space occupying lesions including 1 of false negative,and 7 were negative for space occupying lesions.(2) Relationship of clinical symptoms and preoperative examinations with surgical exploration positive for space occupying lesions.① Relationship of clinical symptoms and laboratory test with surgical exploration positive for space occupying lesions:juandice was significantly associated with surgical exploration positive for space occupying lesions (P＜0.05),and elevated TBil and DBil were significantly associated with surgical exploration positive for space occupying lesions (x2 =0,0,P＜0.05),with a sensitivity of 75.0％ and specificity of 100.0％.(② Relationship between imaging examination and surgical exploration positive for space occupying lesions:results of CT,MRI,endoscopic retrograde cholangiopancreatography,endoscopic ultrasonography,PET-CT,and combined imaging examinations had no significant association with surgical exploration positive for space occupying lesions (x2 =0,0.77,0,0,1.00,0,0,0,0,P＞0.05).PET-CT had no significant association with surgical exploration positive for space occupying lesions (P＞0.05).③ Relationship of imaging examination and laboratory test with surgical exploration positive for space occupying lesions:positive imaging examination combined with elevated TBil and CA19-9 was significantly associated with surgical exploration positive for space occupying lesions (P＜0.05),with a sensitivity of 50.0％ and specificity of 100.0％.④ Relationship of preoperative diameters of biliary ducts and pancreatic ducts with surgical exploration positive for space occupying lesions:of 22 patients,the diameters of biliary ducts and pancreatic ducts were (13.8±4.3)mm and (4.6±1.5)mm for patients with positive surgical exploration,(13.0±2.8)mm and (3.5±0.5) mm for patients with negative surgical exploration,(11.6±2.4) mm and (3.2±0.4) mm for patients with follow-up,respectively,showing no significant difference between them (t =0.22,0.36,P＞0.05).(3) Surgical treatment:9 of 11 patients with surgical exploration followed the standard procedure.Of the 9 patients,4 were found space-occupying lesions at the choledocho-pancreatico-duodenal junction (3 undergoing pancreaticoduodenectomy and 1 undergoing duodenal papilla partial resection),5 with negative exploration underwent common bile duct incision and T-tube drainage (one patient was unable to pinch the T-tube one month after operation and detected obstruction at the lower end of the bile duct by radiography,and was confirmed pancreatic head cancer by reoperation 3 months after the first operation).Two patients didn't follow the exploratory procedure,and underwent the child operation only based on the preoperative imaging findings,without intraoperative pathological examination.Postoperative pathological examination showed chronic ampulla and chronic pancreatitis,respectively.(4) Follow-up:22 patients were followed up for 12-60 months,with a median followup time of 36 months.Two of 11 patients with surgical exploration had postoperative gastroplegia,1 had bile leakage,1 had incisional infection,and they were improved after symptomatic treatment.Four patients undergoing surgeries for positive exploration had no recurrence during follow-up.Of 5 patients with negative exploration undergoing common bile duct incision and T-tube drainage,1 was confirmed pancreatic head cancer and underwent pancreaticoduodenectomy,4 were removed T-tube after by T-tube cholangiography at 2 months after surgery.During the follow-up,no positive signs showed in laboratory test or imaging examination.No recurrence occurred in the two patients undergoing pancreaticoduodenectomy.Of 11 patients with follow-up,10 had abdominal pain before surgery,including 3 with pain during follow-up and 7 with symptoms disappeared.There was no abnormalities in the laboratory test.Conclusions The positive imaging examinations combined with jaundice and elevated CA19-9 is an absolute indication for surgical exploration in patients with biliary duct dilatation.Those patients who do not meet this criteria should be distributed into the follow-up.If no positive pathological results were obtained during the operation,the surgery should be terminated and the patients should be transferred into follow-up.The reckless biliary anastomosis or biliary stents placement is opposed.
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Objective@#To summarize the diagnosis and treatment of biliary pancreatic duct dilatation.@*Methods@#The retrospective and descriptive study was conducted. The clinical data of 22 patients with biliary pancreatic duct dilatation who were admitted to Renji Hospital of Shanghai Jiaotong University School of Medicine between October 2013 to September 2017 were collected. There were 6 males and 16 females, aged from 33 to 82 years, with an average age of 66 years. Surgical exploration was decided according to clinical symptoms, results of laboratory test and imaging examinations. For patients with space occupying lesions, surgical procedure was selected based on results of pathological examination. Patients without surgical exploration or space occupying lesions were allocated into follow-up. Observation indicators: (1) surgical exploration; (2) relationship of clinical symptoms and preoperative examinations with surgical exploration positive for space occupying lesions; (3) surgical treatment; (4) follow-up. Follow-up using outpatient examination was performed on patients up to October 2018. Follow-up was performed on patients with positive surgical exploration to detect postoperative complications.For patients with positive results of imaging examinations, no jaundice, normal laboratory indicators or mild abnormality, liver function, tumor markers and B-ultrasound were re-examined each month, and computed tomography (CT) and magnetic resonance imaging (MRI) was performed once every 3 months. Surgical exploration was performed when total bilirubin (TBil) or tumor markers showed a progressive increase. Follow-up was performed on patients with negative results of imaging examination, jaundice, and mildly elevated CA19-9. TBil and CA19-9 were re-examined monthly, and if they were progressively elevated, patients were transferred to surgical exploration. For patients with negative results of imaging examination, no symptoms, and negative laboratory test, liver function, tumor markers, and B-ultrasound were re-examined once every 3 months, and enhanced CT and MRI were re-examined once every 6 months within one year. Follow-up was performed once every 6 months during the second year, and once a year after two years. Measurement data with normal distribution were represented as Mean±SD, and comparison between groups was analyzed using the t test. Count data were descibed as absolute numbers, and they were analyzed using the chi-square test under R×C chart or Fisher exact probability.@*Results@#(1) Surgical exploration: of 22 patients, 11 underwent surgical exploration, and 11 underwent follow-up. Of the 11 patients with surgical exploration, 4 were positive for space occupying lesions including 1 of false negative, and 7 were negative for space occupying lesions. (2) Relationship of clinical symptoms and preoperative examinations with surgical exploration positive for space occupying lesions. ① Relationship of clinical symptoms and laboratory test with surgical exploration positive for space occupying lesions: juandice was significantly associated with surgical exploration positive for space occupying lesions (P<0.05), and elevated TBil and DBil were significantly associated with surgical exploration positive for space occupying lesions (χ2=0, 0, P<0.05), with a sensitivity of 75.0% and specificity of 100.0%. ② Relationship between imaging examination and surgical exploration positive for space occupying lesions: results of CT, MRI, endoscopic retrograde cholangio-pancreatography, endoscopic ultrasonography, PET-CT, and combined imaging examinations had no significant association with surgical exploration positive for space occupying lesions (χ2=0, 0.77, 0, 0, 1.00, 0, 0, 0, 0, P>0.05). PET-CT had no significant association with surgical exploration positive for space occupying lesions (P>0.05). ③ Relationship of imaging examination and laboratory test with surgical exploration positive for space occupying lesions: positive imaging examination combined with elevated TBil and CA19-9 was significantly associated with surgical exploration positive for space occupying lesions (P<0.05), with a sensitivity of 50.0% and specificity of 100.0%. ④ Relationship of preoperative diameters of biliary ducts and pancreatic ducts with surgical exploration positive for space occupying lesions: of 22 patients, the diameters of biliary ducts and pancreatic ducts were (13.8±4.3)mm and (4.6±1.5)mm for patients with positive surgical exploration, (13.0±2.8)mm and (3.5±0.5)mm for patients with negative surgical exploration, (11.6±2.4)mm and (3.2±0.4)mm for patients with follow-up, respectively, showing no significant difference between them (t=0.22, 0.36, P>0.05). (3) Surgical treatment: 9 of 11 patients with surgical exploration followed the standard procedure. Of the 9 patients, 4 were found space-occupying lesions at the choledocho-pancreatico-duodenal junction (3 undergoing pancreaticoduodenectomy and 1 undergoing duodenal papilla partial resection), 5 with negative exploration underwent common bile duct incision and T-tube drainage (one patient was unable to pinch the T-tube one month after operation and detected obstruction at the lower end of the bile duct by radiography, and was confirmed pancreatic head cancer by reoperation 3 months after the first operation). Two patients didn′t follow the exploratory procedure, and underwent the child operation only based on the preoperative imaging findings, without intraoperative pathological examination. Postoperative pathological examination showed chronic ampulla and chronic pancreatitis, respectively. (4) Follow-up: 22 patients were followed up for 12-60 months, with a median follow-up time of 36 months. Two of 11 patients with surgical exploration had postoperative gastroplegia, 1 had bile leakage, 1 had incisional infection, and they were improved after symptomatic treatment. Four patients undergoing surgeries for positive exploration had no recurrence during follow-up. Of 5 patients with negative exploration undergoing common bile duct incision and T-tube drainage, 1 was confirmed pancreatic head cancer and underwent pancreaticoduodenectomy, 4 were removed T-tube after by T-tube cholangiography at 2 months after surgery. During the follow-up, no positive signs showed in laboratory test or imaging examination. No recurrence occurred in the two patients undergoing pancreaticoduodenectomy. Of 11 patients with follow-up, 10 had abdominal pain before surgery, including 3 with pain during follow-up and 7 with symptoms disappeared. There was no abnormalities in the laboratory test.@*Conclusions@#The positive imaging examinations combined with jaundice and elevated CA19-9 is an absolute indication for surgical exploration in patients with biliary duct dilatation. Those patients who do not meet this criteria should be distributed into the follow-up. If no positive pathological results were obtained during the operation, the surgery should be terminated and the patients should be transferred into follow-up. The reckless biliary anastomosis or biliary stents placement is opposed.
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Objective To explore the clinical features, curative effects and prognosis of patients with psoriasis related acute leukemia. Methods The clinical data of 39 cases in the Second Hospital Shanxi Medical University from January 2011 to June 2016 were collected, and their clinical features and prognosis were analyzed retrospectively. Results Of 39 patients, 28 were males and 11 were females, with the median age of 42 years (13-76 years), the median time that suffering from psoriasis were 10 years (1-30 years); There were 23 (59.0 ％) patients with acute promyelocytic leukemia (APL), 13 (33.3 ％) patients with acute myeloid leukemia (AML) and 3 (7.7 ％) patients with B-cell acute lymphocytic leukemia (B-ALL). In patients with recurrence, there were 4 patients with APL, 4 patients with AML, and 1 patient with B-ALL. Single factor analysis showed that the type of PML-RARαfusion gene isoform and the time of suffering from psoriasis were the influencing factors of patients with APL recurrence (both P<0.05), but they were not the independent risk factors (both P>0.05) based on multivariate analysis. The complete remission (CR) rates of patients with psoriasis related APL and AML were 96％(22/23) and 46％(6/13), the 3-year overall survival (OS) rates were 96％and 44％, 3-year relapse free survival (RFS) rates were 77％and 38％, respectively. Conclusions In patients with psoriasis related acute leukemia, the largest population is APL patients, and they have a better prognosis. However, patients with psoriasis related AML and ALL have low CR rate and OS rate, and the allogeneic hematopoietic stem cell transplantation should be considered after remission.
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Objective To evaluate the efficacy and safety of the mobilization and collection of unrelated allogeneic peripheral blood stem cells. Methods The suitable stem cell mobilization plan was made in accordance with the hematopoietic stem cell mobilization plan of China Marrow Donor Program, the ruler of the hospital, and the donor's constitution. The unrelated allogeneic peripheral blood stem cells of 64 healthy donors were collected in the second Hospital of Shanxi Medical University from May 2012 to January 2017. The donor was infected one or several times with the mobilization agent granulocyte colony stimulating factor (G-CSF) by 5-10 μg·kg-1·d-1. After 3-4 days, peripheral blood hematopoietic stem cells were collected using COBE Spectra blood cell separator. Then, the effect and adverse reaction of donors were analyzed from different age and sex. Results It can achieve the acquisition requirements using 3 or 4 days of mobilization programs, mononuclear cells≥5.0×108/kg, CD34+cells≥2.0×106/kg. The single acquisition success rate (the target acquisition of the number of mononuclear cells and CD34+) up to 65 ％, collection efficiency reached 52％, which could reduce the risk of donor and the cost of patients. The quality of donor stem cell of young was better than that of older persons. Sixteen donors (25％) had mild adverse reactions, and no special treatment was required. Conclusions Allogeneic stem cell mobilization is safe. Starting from save medical resources and the interests of the donor the 3 day or 4 days of mobilization scheme could improve the success rate of the single mobilization. During the collection process, the condition of donor hypocalcemia should be observed and health education should be given to relieve the tension of donor.
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Objective To explore the biological characteristics of primary CD5 positive diffuse large B-cell lymphoma (DLBCL) and treatment effects of rituximab combined with different chemotherapy regimens. Methods The clinical features of 2 CD5 positive DLBCL patients (1 case with early report and 1 case with newly treated) in department of hematology of the Second Hospital of Shanxi Medical University were summarized, including bone marrow, lymph nodes morphology, flow cytometry, immunohistochemistry, and molecular biology characteristics. The treatment response of rituximab combined with different chemotherapy regimens was evaluated, and the literatures were reviewed. Results First patient was diagnosed as primary CD5 positive DLBCL with complex karyotype (leukemia stage). After the first induction chemotherapy with rituximab and VCTP regimens (4 weeks), the patient achieved complete remission of bone marrow. Spleen and lymph nodes also were reduced significantly. Eight consolidation therapy including R-VCTP regimen (2 weeks), R-Hyper-CVAD regimens for 3 courses and R-HD-MTX regimens for 4 courses were sequentially given. Cytarabine combined with and methotrexate (MTX) was performed 10 times of intrathecal injection. After the follow-up of 21 months, the disease sustained remission without central nervous system (CNS) invasiveness. Second elderly patient was diagnosed as primary CD5 positive DLBCL (nasal type), who received induction chemotherapy regimen of rituximab combined with vincristine and prednisone for 3 courses. Nasal tumor symptoms completely relieved after subsequent treatments and the local sclerotin restored. However, multiple skin lesions appeared after half a year, which were quickly alleviated after treatment of rituximab combined with COP regimen. Magnetic resonance imaging (MRI) showed that the brain parenchymal infiltration appeared. Related symptoms gradually relieved after radiotherapy and imageology also showed that infiltrates were mostly absorbed. The patient was followed up for 21 months and had the stable disease. Conclusions Primary CD5 positive DLBCL is a unique subset presented with more extranodal lesions, which occur commonly in bone marrow, skin and CNS. Rituximab combined with acute lymphoblastic leukemia chemotherapy and intrathecal chemotherapy may reduce incidence of metastatic central lesions , and improve the disease free survival rate.
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Objective To summarize the prenatal ultrasonographic characteristics of unroofed coronary sinus( URCS) for improving the prenatal echocardiography diagnostic accuracy of URCS . Methods The retrospective review for prenatal ultrasonogram of 7 fetuses was applied ,which were diagnosed as URCS . The prenatal ultrasonographic characteristics of 7 fetuses with URCS were reviewed and comparatively analyzed with postpartum autopsy findings or postnatal echocardiography . The relevant literatures referring to the prenatal diagnosis of URCS were reviewed . The experience of prenatal diagnosis of URCS were summarized . Results In 7 cases with URCS ,5 cases of completely URCS were associated with right atrial isomerism syndrome and confirmed by postpartum autopsy ,and 2 cases of partially URCS were only with persistent left superior vena cava and confirmed by postnatal echocardiography . The karyotype analysis of 2 cases of partially URCS on amniotic fluid was normal . Conclusions There are some characteristic signs in ultrasonic features of URCS . URCS can be diagnosed by prenatally ultrasonography . A long axial section of the coronary sinus is the most important view for prenatal ultrasonic diagnosis of U RCS .
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Objective To summarize the prenatal ultrasonographic characteristics of anomalous origin of one pulmonary artery from the aorta( AOPA ) ,and describe the diagnostic and clinical outcomes of fetal AOPA . Methods Echocardiographic characteristics of 3 fetuses with AOPA were reviewed . The ultrasonographic features were comparatively analyzed with postpartum autopsy findings . The relevant literature were reviewed and the experience of prenatal diagnosis of AOPA were summarized . Results In 3 cases with AOPA ,2 cases were the proximal type and anomalous origin of right pulmonary artery from the aorta ,of them ,one was accompanied with coarctation of the aorta , the other was accompanied with aortopulmonary window . One case was the distal type and anomalous origin of left pulmonary artery from the left innominate aorta ,it was only associated with mirror right aortic arch and right ductus arteriosus . Ultrasound characteristics were no bifurcation in distal pulmonary artery ,main trunk of pulmonary artery extends directly to one branch ,the other branch originated from the ascending aorta or left innominate artery . Three cases were confirmed by postpartum autopsy . Conclusions There are some characteristic signs on ultrasonic features of AOPA . Prenatal ultrasound has important value for diagnosis of AOPA .
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Objective:To revise the Chinese version of the Parental Psychological Flexibility Questionnaire (PPFQ) and test its validity and reliability.Methods:Totally 1015 parents from 2 primary school of Beijing were recruited and asked to complete the Chinese version of PPFQ.Item analysis was conducted to identify valid items.The parents were randomly allocated into two groups where one subset (n =510) was used for exploratory factor analysis (EFA),and the other (n =505) for confirmatory factor analysis (CFA) and discriminant validity.In addition,responses of 389 participants selected by simple random sampling was assessed in criterion validity with the Cognitive Fusion Questionnaire (CFQ),Acceptance and Action Questionnaire-Second Edition (AAQ-Ⅱ]),Self-Rating Anxiety Scale (SAS),Self-Rating Depression Scale (SDS),Mindful Attention Awareness Scale (MAAS),Trait Coping Style Questionnaire(TCSQ) and Conners Child Behavior Rating Scale (CBRS).Seven weeks later,225 of the participants were retested.Results:The exploratory factor analysis extracted 3 components,and explained 58.1％ of the total variance.The factor loading of items ranged between 0.63 and 0.86.The confirmatory factor analysis verified the 3-factors model (x2/df=1.77,NFI =0.94,IFI =0.97,GFI =0.96,TLI =0.96,CFI =0.97,RMSEA =0.04).Discriminant validity testing demonstrated the three revised factors had adequate discriminant validity in relation to one another (Ps ＜ 0.01).Criterion validity results showed that the total and subscale scores of the PPFQ were negatively correlated with the scores of CFQ,AAQ-Ⅱ,SAS,SDS,TCSQ-NC,CBRS (r =-0.16--0.52,Ps ＜ 0.01),and were positively correlated with the scores of MAAS,TCSQ-PC (r =0.14-0.37,Ps ＜ 0.01).The total and subscale scores of the PPFQ demonstrated good internal consistency Cronbach's α were 0.77-0.86 and test-retest reliability coefficient were 0.55-0.75.Conclusion:It suggests tthe validity and reliability of the adapted Chinese version of the Parental Psychological Flexibility Questionnaire be satisfactory,and could be used in the clinical and research work in China.
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Objective@#To evaluate the efficacy and safety of a domestic human plasma derived coagulation Factor Ⅸ concentrate (pd-FⅨ) in patients with hemophilia B.@*Methods@#The study was a multicenter, open-label and single-arm study. The efficacy of pd-F Ⅸ was evaluated by objective performance criteria. The doses of pd-FⅨ were calculated according to the bleeding symptom and disease severity. The infusion efficiency of pd-FⅨ and improvement of bleeding symptoms were measured at 30 minutes and (24±4) h after the first infusion, respectively. Adverse events were recorded. Viral infection and FⅨ inhibitor were detected 90 d after the first infusion.@*Results@#All 36 subjects with hemophilia B were enrolled in the study. The median age of these patients was 31 years old and the median injection doses were 4 (1-17) times. The hemostatic effect of 27/36 (75.00%) and 9/36 (25.00%) acute bleeding events were rated as "excellent" and "better" , respectively. The recovery rate was 111.92% (65.55%-194.28%) at 30 minutes after infusion of FⅨ. There was no adverse event related to FⅨ. No reactivation of HBV, HCV or HIV and FⅨ inhibitor was detected at 90-104 d after the first FⅨ infusion.@*Conclusion@#This domestically made human plasma derived FⅨ concentrate is safe and effective in the treatment of acute bleeding in patients with hemophilia B.@*Clinical trial registration@#China food and Durg Administration, 2016L08027.
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Primary immune thrombocytopenia (ITP) in pregnancy is a special type of ITP,its impact on the mother and fetus cannot be ignored.The correct diagnosis and effective treatment of ITP in pregnancy are the focus of the clinical practice and medical research.This article reviews the progress on the management of the primary immune thrombocytopenia in pregnancy.
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Objective To examine the influencing factors related to clinical efficacy and outcomes of adult primary immune thrombocytopenia (ITP).Methods The clinical data of 161 cases of ITP admitted in the Second Hospital of Shanxi Medical University from June 2013 to March 2017 were collected.The influencing factors related to clinical efficacy and prognosis of adult ITP patients were analyzed.Results There were 60 males and 101 females with a M/F ratio of 0.59∶1 and a median age of 45 years (18-84 years).There were 109 newly diagnosed ITP cases,14 persistent ITP cases and 38 chronic ITP cases in this series.Seventy nine patients received intravenous immunoglobulin g (IVIg) treatment and 82 patients received high dose-dexamethasone treatment.There were no significant differences in clinical efficacy [91.13％(72/79) vs.87.80％(72/82),x2=0.181,P=0.914] and relapse rate [36.11％(26/72) vs.30.55％(22/72),x2=0.189,P=0.910] between IVIg and high dose-dexamethosone groups.Multivariate regression analysis showed that bleeding score ≥2 was the independent risk factor for the lower clinical efficacy (RR=1.415,95％CI:1.008-1.986,P＜0.05).Patients were followed up for a median of 9.0 months (0.5-55.0 months),48 patients relapsed with a relapse rate of 33.33％ and a median relapse time of 1.8 months (0.5-24.0 months).Conclusions IVIg and high dose-dexamethasone have the similar clinical efficacy and relapse rate for treatment of adult ITP.The patients with the bleeding score ≥2 are more likely to get lower remission rate.