OBJECTIVE: To investigate the therapeutic efficacy of using decitabine as maintenance therapy for patients with relapsed MDS/AML and as prophylactic therapy for patients with high-risk AML after allogeneic hematopoietic stem cell transplantation (allo-HSCT). METHODS: Clinical data of 10 patients with MDS/AML from November 2016 to May 2018 were analyzed retrospectively. Among 10 patients there were 4 cases of AML, 2 cases of MDS, and 4 cases of AML transformed from MDS (t-AML). The 10 patients were devided into 2 groups: the relapsed group (n=8) and the prophylactic group (n=2). In relapsed group the decitabine was used as maintenance therapy after achieved complete remission (CR) with decitabine chemotherapy. In prophylactic group the decitabine was used as prophylactic therapy if the patients didn't appear the symptom of graft-versus- host-disease (GVHD) during 30 to 45 d after allo-HSCT. Eight patients received G-CSF-mobilized donor lymphocyte infusion (DLI). The dosage of decitabine for maintenance therapy and prophylactic therapy was 5 mg/m for 7 to 10 days every 4 to 6 weeks, as 1 cycle, amount to 3 to 7 cycles. The dosage was adjusted by the endurance of patients. RESULTS: Until Nov 30, 2018, 7 out of 10 patients survived. The average survival time was 15.5±1.9 months. 1-year OS rate was 64.0%. Six patients appeared aGVHD, and four patients appeared cGVHD. CONCLUSION The usage of decitabine combined with DLI in patients with relapsed MDS/AML and high-risk AML after allo-HSCT can prolong lives of patients, reduce relapsed rate, and provide the probability for long time survival.

OBJECTIVE: To investigate the clinical efficacy, related side-effectt and long-term survival condition of Philadelphia chromosome positive acute lymphoblastic leukemia (Ph ALL) patients treated with second generation TKI dasatinib and chemotherapy followed by allogeneic hematopoietic stem cell transplantation (allo-HSCT). METHODS: Clinical data of 19 newly diagnosed as Ph ALL patients treated by dasatinib, chemotherapy and allo-HSCT from January 2012 to September 2018 were collectd and analyzed. RESULTS: There were 10 males and 9 females with median age of 29 years old. 14 patients were BCR/ABL P190 positive while 5 with BCR/ABL P210 positive. Three patients had complex karyotype, and 3 cases were confirmed to have central nervous system leukemia. All the patients received treatment with the induction chemotherapy regimen of VDCLP and consolidation regimens such as HD-MTX and MAE. 11 patients (57.9%) received dasatinib during induction chemotherapy, 3 patients (15.8%) received dasatinib after remission and 5 patients (26.3%) received dasatinib to replace imatinib. Side-effect appeared in 3 patients including rash, edema and nausea. All the patients got morphological remission and 7 patients(63.6%) got MMR after 4 weeks of induction chemotheraphy. 17 patients (89.5%) got MMR and 15 patients(78.9%) got CMR before allo-HSCT. All the patients received related bone marrow and peripheral hematopoietic stem cell transplantation from related donors, the median time of WBC and platelet engraftment were 12 d and 14 d after transplantation, respectively. The incidence rate of aGVHD and cGVHD were 42.1% and 57.9% respectivety. 13 patients received therapy of dasatinib after HSCT but 7 patients discontinued because of severe headache, vomiting and serious effusions. All the patients were followed-up for the median time of 42 months, the 3-year and 5-year OS both were 94.4%, and 3-year and 5-year RFS of 81.9% and 71.6%, respectively. CONCLUSION First-line administration of dasatinib and chemotherapy followed by allo-HSCT for treatment of PhALL is effective and patients can well-tolerate, the patients long-tern survival maybe superior to that of the patients treated with first generation TKI.

Objective To investigate the incidence and risk factors of deep venous thrombosis in patients with open fracture of lower extremities.Methods A retrospective study was conducted to analyze the clinical data of 203 patients with open fracture of lower limbs admitted to Department of Orthopaedics and Trauma,Xi'an Jiangtong University Medical College Red Cross Hospital from January 2015 to November 2017.There were 75 males and 45 females,the average age was 47.3 years,and the age ranged from 18 to 63 years.Classification of open fracture sites of lower limbs:open fracture of femoral shaft in 59 cases;open fracture of tibia and fibula in 107 cases;open fracture of ankle in 37 cases.Gustilo classification of open fracture included 34 cases of type Ⅰ,71 cases of type Ⅱ and 98 cases of type Ⅲ.According to whether thrombosis occurs or not,the patients were divided into thrombosis group (n =63) and non-thrombosis group(n =140).The incidence of thrombosis was expressed as percentage (％).The age,fracture degree,injury type,Gustilo classification of open fracture,gender,body mass index (BMI),combined medical diseases,fracture site,preoperative and postoperative D-dimers were subjected to x2 test or Fisher's exact test.The test αt value was taken as 0.05 on both sides.P ＜ 0.05 suggested that the difference was statistically significant.Results The incidence of deep venous thrombosis in 63 cases of open fracture was 31.03％,of 63 DVT patients,5 were central thrombosis (7.93％),17 were mixed thrombosis (26.98％) and 41 were peripheral thrombosis (65.08％).The main type of thrombosis was peripheral thrombosis.There were significant differences in age,fracture degree,injury type and open fracture Gustilo type between the two groups of thrombus group and non thrombus group (P ＜ 0.05).Sex,BMI,internal medical diseases,fracture sites,and D-two polymer before and after operation were not statistically significant (P ＞0.05).Conclusions The incidence of DVT was higher in patients with open fracture of lower extremities,the main type of thrombosis was peripheral thrombosis.Age over 60 years old,high falling injury,2 or more fractures and comminuted fractures,open fractures Gustilo type Ⅲ was associated with DVT.

Objective To quantitatively evaluate the diagnostic value of blood oxygen leveldependent (BOLD) MRI in the diagnosis of different degrees of liver warm ischemia-reperfusion injury (WIRI) in rabbits and evaluate the intervention effect of liposomal prostaglandin E1 (Lipo-PGE1).Methods Seventy healthy adult New Zealand white rabbits were randomly divided into sham -operated group (A0),thermal ischemic groups (A1～A3) and intervention groups (A4～A6).All experimental rabbits were scanned by routine MR and BOLD MRI after 6-hour reperfusion.R2* images were calculated by two radiologists.The levels of alanine aminotransferase (ALT),asparate aminotransferase (AST) and lactate dehydrogenase (LDH) were examined.And liver pathological sectioning was performed.All data were processed by one-way,Spearman's correlation and receiver operating characteristic curve analyses.Results The intraclass correlation coefficient (ICC) was 0.805 of two measurements suggesting that the repeatability of the outcome was decent.R2* values among sham-operated,thermal ischemia and intervention groups were statistically significant (P＜0.05).R2 * values in sham-operated and ischemia groups were statistically significant (P＜0.05).As warm ischemia time elapsed,R2* value showed a rising trend.R2* values in sham-operated and intervention groups were statistically significant (P＜0.05).R2* values of sham-operated group at the same timepoint of thermal ischemia and intervention groups were statistically significant (P＜0.05).Under the same ischemic time,R2* values of intervention groups were smaller than those of thermal ischemia groups.With the prolongation of ischemia time,reduction of R2* values became more pronounced.However,it did not reach the level of A0 group.R2* values were significantly positively correlated with ALT,AST and LDH (r＞0.5,P＜0.05).ROC analysis indicated that R2* had an excellent diagnostic performance.Conclusions BOLD MRI may be applied for noninvasive assessment of liver ischemia-reperfusion injury in different degrees.Lipo-PGE1 alleviates ischemia -reperfusion injury and BOLD MRI can evaluate the relieving degree of Lipo-PGE1.

Clinical data of a 33-year-old rheumatoid arthritis patient accompanied with pregnancy and lactation-associated osteoporosis(PLO) was retrospectively reviewed.The patient had two pregnancies within three years,and bone pain occurred during lactation of the second pregnancy after 2 months of delivery.Dual-energy X-ray (DEXA) showed decreased bone density,while thoracolumbar spine and pelvic X-ray film suggested osteoporosis.Her rheumatoid arthritis was stable and previous treatment did not include glucocorticoids.Other causes of metabolic bone diseases were excluded and the diagnosis of PLO was confirmed.The breastfeeding was stopped;calcium,active vitamin D,alendronate sodium were administrated,and the treatment was effective.Domestic and international literature from January 2000 to January 2019 was searched and no cases of PLO accompanied with rheumatoid arthritis were reported.There were only 100 cases of PLO retrieved in the literature,including 19 cases of PLO in China reported by four articles.It is suggested that PLO should be considered in RA patients presenting with arthragia during the lactation,in addition to relapse of disease.Awareness of PLO should be increased in physicians,especially rheumatologists,and anti-osteoporosis therapy is effective in patients with PLO.

Objective To establish a test of autoantibody-panel for the diagnosis of autoimmune cerebellitis (AC) and determine the prevalence of AC in patients with cerebellar ataxia of unknown etiology.Methods Autoantibody screening tests with indirect immunofluorescence were performed in serum and cerebrospinal fluid (CSF) samples of 400 previously'idiopathic'Chinese patients with cerebral ataxia (inpatients and outpatients in Peking Union Medical College Hospital or referred from hospitals of Beijing Encephalitis Group from 2016 to 2018).Immunotherapy was given to autoantibody positive patients and the effectiveness of immunotherapy was assessed.Detailed AC autoantibodies panel included anti-glutamate decarboxylase 65 (GAD65) antibody,anti-Tr (delta notch-like epidermal growth factor-related receptor (DNER)) antibody,anti-zinc finger protein 4 (ZIC4) antibody,anti-inositol 1,4,5-trisphosphate receptor 1 (ITPR1) antibody,anti-homer protein homolog 3 (Homer 3) antibody,anti-neurochondrin (NCDN) antibody,anti-carbonic anhydrase-related protein (CARP) antibody and anti-Purkinje cell antibody 2 (PCA2) antibody.Results Eight out of 400 (2％) ataxia patients were positive for this AC panel tests,of whom two were positive for anti-GAD65 antibody,two for anti-Tr antibody,one for anti-PCA2 antibody,one for anti-Homer 3 antibody and two were positive for serum anti-NCDN antibody.Autoantibodies against ZIC4,ITPR1 and CARP were not detected in this cohort.Two of the eight ataxia patients also presented with limbic encephalitis,and only one anti-GAD antibody patient was screened with underlying small cell lung carcinoma (SCLC).All the eight patients received immunotherapy and four experienced partial response.Conclusions Autoimmune cerebellitis is the cause of acquired cerebellar ataxia.Tests of autoantibodies associated with AC have diagnostic value for paraneoplastic and non-paraneoplastic cerebellar ataxia.Immunotherapy may yield partial response in patients with AC.

Objective    To evaluate the quality of warfarin anticoagulant therapy in patients with stable stage after mechanical valve replacement surgery, to observe the effect of compound salvia miltiorrhiza tablet on the anticoagulant effect of warfarin in patients after mechanical valve replacement, and to understand the impact of genetic polymorphisms of VKORC1, CYP2C9 and CYP4F2 on warfarin resistance in patients with mechanical valve replacement in the stable period. Methods    From July 2011 to February 2014, 1 831 patients who had ≥ 6 months after mechanical valve  replacement surgery were enrolled at the outpatient follow-up. The basic clinical data were recorded. Anticoagulant therapy uses a target international normalized ratio（INR, 1.60–2.20) and a weekly warfarin dose adjustment strategy. Forty-six patients who needed compound salvia miltiorrhiza tablet were screened and the INR values. Before and after taking tablets were recorded and compared. The patients were divided into three groups according to the percentile of warfarin dosage including a warfarin sensitive patients group, a control patients group, and a warfarin resistance patients group. And 101 of them were selected. TIANGEN blood DNA Kit blood genomic DNA extraction kit was used to extract samples and polymerase chain restriction fragment length polymorphism (PCR-RELP) was used to determine the genotypes of patients. The detected gene loci included CYP4F2: rs2108622C>T locus; VKORC1:1639G>A locus; VKORC1:1173C>T locus; CYP2C9*2: rs1799853C>T locus; CYP2C9*3:1061A>C locus. Results    The time in therapeutic range (TTR) and fraction of time in therapeutic range (FTTR) in the target INR range of the patients included in the study period was 27.2% and 49.4%, respectively, and the TTR and FTTR in the acceptable INR range was 34.25% and 63.36%, respectively. Before and after the addition of compound salvia miltiorrhiza tablets, the INR value was 1.55±0.03 and 1.69±0.30, respectively, and the difference was statistically different (P<0.05). A total of 101 patients with genetic testing, in which the C/T composition of the VKORC1: 1173C>T locus increased in the warfarin sensitivity, contrast and warfarin resistance patients, while the ratio of allelic loci of C/T in CYP2C9*3: 1061A>C loci decreased in turn. There was no difference in the CYP4F2 gene, VKORC1639 gene, and CYP2C9*2 locus. The IWPC model predicts that warfarin dose is only consistent with the actual warfarin dose in warfarin sensitive patients. Conclusion    Relatively low TTR and FTTR are acceptable in patients with stable stage after mechanical valve replacement. It is beneficial to the patients with compound salvia miltiorrhiza tablets in terms of some appropriate patients. VKORC1: 1173C>T site and CYP2C9*3: 1061A>C site mutation is the main pharmacological gene factor of warfarin dose sensitivity and warfarin resistance in stable period after mechanical valve replacement. The IWPC dose prediction model is only consistent with the actual dose of warfarin sensitive patients.

Objective To explore the characteristics and value for predicting prognosis of cytokines in septic children with or without neutropenia.Methods Totally 138 septic children were divided into the neutropenia and non-neutropenia groups according to absolute neutropenic count.Septic children were divided into the shock and non-shock groups according to circulation function and organ perfusion.The levels of C-reactive protein,procalcitonin,cytokines,PRISM-Ⅲ and clinical outcomes were analyzed between the relative groups.Results (1) Totally 138 septic children were recruited,64 with neutropenia and 74 without neutropenia.The level of PRISM-Ⅲ of the neutropenia group was significantly higher than that of the non-neutropenia group (P=0.048).Mortality showed no significant difference between the two groups,but hospital stay in the neutropenia group was longer than that in the non-neutropenia group.The levels of C-reactive protein,IL-6,and IL-10 ihe neutropenia group were significantly higher than those of the non-neutropenia group (P=0.001;P=0.001;P=0.032).The level of TNF-α in the neutropenia group was significantly lower than that of the non-neutropenia group (P=0.032).(2)Among the 64 septic children with neutropenia,23 were combined with shock.The PRISM-Ⅲ level of the shock group was significantly higher than that of the non-shock group (P=0.001).The mortality of the shock group (43.5％,10/23) was significantly higher than the non-shock group (2.4％,1/41) (P=0.001).C-reactive protein,procalcitonin,IL-6,IL-10 and TNF-α in the shock group elevated obviously than those in the non-shock group (P=0.001;P=0.001;P=0.001;P=0.005;P=0.019).The area under receiver operating characteristic curve was 0.8 for IL-6 (cut-offvalue 315.38 pg/mL),0.8 for IL-10 (cutoff value 45.18 pg/mL),and 0.85 for TNF-α (cut-off value 1.95 pg/mL).(3) Among the 74 septic children without neutropenia,19 were combined with shock The PRISM-Ⅲ level of the shock group was significantly higher than that of the non-shock group (P=0.022).There was no significant difference of mortality between the two groups (P=0.3).IL-10 level in the shock group elevated obviously than that in the non-shock group (P=0.015).(4) Among the 42 children with sepsis shock,23 were combined with neutropenia.The PRISM-Ⅲ level of the neutropenia group was significantly higher than that of the non-neutropenia group (P=0.005).There was no significant difference of mortality between the two groups (P=0.29).The levels of C-reactive protein,procalcitonin,IL-6 and IL-10 in the neutropenia group were significantly higher than those in the non-neutropenia group (P=0.001;P=0.001;P=0.001;P=0.035).There was no difference of TNF-α level between the two groups.(5) Among the 96 children without sepsis shock,41 were combined with neutropenia.No difference of PRISM-Ⅲ level was observed between the neutropenia and nonneutropenia groups.The mortality of the neutropenia group was significantly lower than that in the non-neutropenia group (2.4％ vs 20％,P=0.02).The levels of C-reactive protein and IL-6 in the neutropenia group were significantly higher than those in the non-neutropenia group (P=0.005;P=0.033).The TNF-α level was significantly lower than that in the non-neutropenia group (P=0.007).Conclusions Compared to children without neutropenia,septic children combined with neutropenia have longer hospital stay,and septic shock children combined with neutropenia have higher mortality,and levels of IL-6,IL-10 and TNF-α were also significantly increased.The levels of IL-6,IL-10 and TNF-α can help to predict prognosis of children with sepsis.

OBJECTIVE: To investigate the clinical manifestations pathologic features, treatment options and prognosis of patients with bone lymphoma. METHODS: The clinical characteristics, pathologic features, treatment and prognosis of 34 BL patients diagnosed by histopathologic method or/and PET-CT and treated in first hospital of peking university from January 2004 to April 2018 were analyzed retrospectively. RESULTS: The median age of 34 BL patients was 56 years old, the male and female ratio was 1.43∶1 (24 /10). Among 34 patients, the patients with primary bone lymphoma(PBL) were 8 cases, the patients with secondary bone lymphoma(SBL) was 26 cases, the PBL and SBL ratio was 0.31∶1. Bone lymphoma lacks typical systemic symptoms, and its onset began mostly from bone pain and pathologic bone fracture. The most frequent pathological type of bone lymphoma in our study was diffuse large B-cell lymphoma (DLBCL), accounting for 55.88%. At present, the conventional treatment for bone lymphoma includes chemotherapy, or chemotherapy combined with radiotherapy and surgery, as well as hematopoietic stem cell transplantation. The average and median OS time of BL patients were 349 years and 3 years respectively, meanwhile the OS rate for three years and two years were 56.25% and 78.16%, respectively. Factors that affect survival of BL patients were PBL and SBL classification, pathological type, blood LDH level, and treatment methods. CONCLUSION Bone lymphoma is usually concealed onset，an adequate and adequate combination therapy can improve the survival rate and transplantation therapy plays an important role. Primary bone lymphoma is rare, the prognosis of patients with primary bone lymphoma is good, whereas the prognosis of patients with secondary bone lymphoma is poor.
Bone Neoplasms ; Female ; Hematopoietic Stem Cell Transplantation ; Humans ; Lymphoma, Large B-Cell, Diffuse ; Male ; Middle Aged ; Positron Emission Tomography Computed Tomography ; Prognosis ; Retrospective Studies

OBJECTIVE: To investigate the factors in first-time adrenocorticotropic hormone (ACTH) therapy and their influence on spasm control time in infants with infantile spasms. METHODS: A total of 72 infants with infantile spasms who were admitted from January 2008 to October 2013 were enrolled. Their clinical data were collected, and the exposure factors for infantile spasms were selected. A Cox proportional-hazards regression model analysis was performed for these factors to analyze their influence on spasm control time. RESULTS: Clarification of the etiology (known or unexplained etiology), frequency of spasms before treatment, and presence or absence of combination therapy (ACTH used alone or in combination with magnesium sulfate) had a significant influence on spasm control time in infants with infantile spasms. The infants with a known etiology had a significantly shorter spasm control time than those with unexplained etiology, and the infants with a low frequency of spasms before treatment and receiving ACTH combined with magnesium sulfate early had a significantly longer spasm control time than their counterparts (P<0.05). CONCLUSIONS For infants with infantile spasms at initial diagnosis, etiology should be clarified, which may helpful for evaluating prognosis. A combination of ACTH and magnesium sulfate should be given as soon as possible, which may improve their prognosis.
Adrenocorticotropic Hormone ; therapeutic use ; Anticonvulsants ; Humans ; Infant ; Proportional Hazards Models ; Spasm ; Spasms, Infantile ; drug therapy