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The biosynthesis and maturation of proteins are primarily regulated by the endoplasmic reticulum in its physiological state. Thus, the disruption of physiological homeostasis initiates the buildup of unfolded and misfolded proteins in the endoplasmic reticulum, resulting in endoplasmic reticulum stress (ERS) and unfolded protein response (UPR). One of the important pathways by which UPR maintains intracellular homeostasis under ERS is activating protein kinase R-like endoplasmic reticulum kinase (PERK). The activation of the PERK pathway stimulates eukaryotic translation initiation factor 2 subunit-α (eIF2α) phosphorylation and the selective translation of active transcription factor 4 (ATF4), and PERK induces cell apoptosis by directly binding to the promoter of pro-apoptotic transcription factor C/EBP homologous protein (CHOP). This signaling pathway is also one of the important mechanisms by which UPR participates in the regulation of hematological malignancies and immune cells in a tumor microenvironment. This article provides an overview of advancements in research into the PERK-eIF2α-ATF4-CHOP signaling pathway in hematological malignancies and the potential therapeutic benefits of targeting this signaling pathway.
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Glucose transporter 1(GLUT1)overexpression in tumor cells is a potential target for drug therapy,but few studies have reported screening GLUT1 inhibitors from natural or synthetic compounds.With cur-rent analysis techniques,it is difficult to accurately monitor the GLUT1 inhibitory effect of drug molecules in real-time.We developed a cell membrane-based glucose sensor(CMGS)that integrated a hydrogel electrode with tumor cell membranes to monitor GLUT1 transmembrane transport and screen for GLUT1 inhibitors in traditional Chinese medicines(TCMs).CMGS is compatible with cell membranes of various origins,including different types of tumors and cell lines with GLUT1 expression knocked down by small interfering RNA or small molecules.Based on CMGS continuous monitoring technique,we inves-tigated the glucose transport kinetics of cell membranes with varying levels of GLUT1 expression.We used CMGS to determine the GLUT1-inhibitory effects of drug monomers with similar structures from Scutellaria baicalensis and catechins families.Results were consistent with those of the cellular glucose uptake test and molecular-docking simulation.CMGS could accurately screen drug molecules in TCMs that inhibit GLUT1,providing a new strategy for studying transmembrane protein-receptor interactions.
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AIM: To investigate the preventive and therapeutic effects of Hawthorn flavone on hyperlipidemia and atherosclerosis rats. METHODS: The atherosclerosis model was established by high fat diet plus vitamin D2. The blood lipid levels, heart index, atherosclerosis index (AI1, AI2) and coronary heart index were measured in each group. The histopathological changes of aorta were observed by oil red O staining, HE staining and Masson staining. ELISA experiments were used to detect IL-6, ICAM-1, MCP-1 and VCAM-1 protein level. RESULTS: Compared with normal group, total cholesterol (TC), triglyceride (TG), low density lipoprotein (LDL-C), heart index, atherosclerosis index (AI1, AI2) and coronary index in atherosclerosis model group were significantly increased (P<0.01), while high density lipoprotein cholesterol (HDL-C) was significantly decreased (P<0.01). The pathological score of aorta and the degree of fibrosis were significantly increased (P<0.01). Compared with model group, TC, TG, LDL-C, heart index, atherosclerosis index (AI1, AI2) and coronary heart index were significantly decreased (P<0.01), and high-density lipoprotein cholesterol (HDL-C) was significantly increased (P<0.01) in medium, high dose hawthorn flavonoids and atorvastatin groups. The pathological score of aorta significantly decreased and the degree of fibrosis significantly improved (P<0.01). The variation trend of blood lipid levels in hyperlipidemia rats is basically consistent with atherosclerotic rats. Meanwhile, compared with model group, the medium, high dose hawthorn flavonoids and atorvastatin groups could significantly inhibit the expression levels of IL-6, MCP-1, ICAM-1 and VCAM-1 adhesion molecules (P<0.01). CONCLUSION: The hawthorn flavone can inhibit the formation of aortic endothelial atherosclerotic plaque, reduce the degree of fibrosis and inflammation of atherosclerotic plaque in rats, and achieve the purpose of anti-atherosclerosis. Meanwhile, the hawthorn flavone has the effect of regulating blood lipid.
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Objective@#To analyze the correlation between plasma trough level of generic imatinib and its metabolism and clinical outcomes in Chinese patients with chronic myeloid leukemia in chronic phase (CML-CP) .@*Methods@#The 21 patients with CML-CP who enrolled in a clinical trial YMTN 1.0 from Oct 11th, 2012 to May 8th, 2013 and received generic imatinib were as study subjects. The correlation between steady plasma trough levels of imatinib and its metabolism with clinical response, age, weight and body surface area (BSA) were evaluated.@*Results@#①The mean steady plasma trough level of generic imatinib and its metabolism was (1 185.07±417.91) μg/L and (251.53±76.50) μg/L, respectively. ②Age, weight and BSA has no significant effects on plasma trough level of generic imatinib and its metabolism (P>0.05) . ③Patients with steady plasma trough level of generic imatinib more than 1 000 μg/L are possible to have higher major molecular response (MMR) /complete molecular response (CMR) rate than those below 1 000 μg/L (42% vs 0, P<0.05) .@*Conclusion@#Plasma trough levels of generic imatinib varied in CML patients. The steady plasma trough levels of generic imatinib is maybe related to molecular response in CML patients.
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Objective@#To evaluate the efficacy and safety of recombinant human thrombopoietin (rhTPO) treatment for primary immune thrombocytopenia (ITP) patients during the perioperative period.@*Methods@#Adult ITP patients who were refractory to first-line glucocorticoid therapy and underwent selective surgery were enrolled to be treated with rhTPO at the dosage of 1.5×104U/d subcutaneously during the perioperative period. rhTPO treatment would not be terminated until one of the following conditions occurred: ①Platelet counts met the requirement of surgery; ②Platelet counts were ≥100×109/L; ③Completed the 14 days of therapy. End points of the study were surgery rate, rhTPO therapy response rate, rescue therapy rate and adverse responses.@*Results@#42 patients were enrolled from Jan. 1, 2016 to Jun. 30, 2018. 14 were male and 28 were female. The median age was 60 (25-73) years old. There were no newly diagnosed patients. 5 patients were persistent and 37 were chronic. 27 patients completed selective surgery. The surgery rate was 64.3% (27/42) . Among them, 13 patients were under local anesthesia and 14 under general anesthesia. Of 42 cases receiving rhTPO therapy. 31 patients achieved responses, The overall response rate was of 73.8%. Among them, 24 patients achieved CR. The CR ratio was 77.4% (24/31) . 7 achieved response. The response ratio was 22.6% (7/31) . 11 patients did not respond to rhTPO therapy. The non-response rate was 26.2% (11/42) . The median time to reach CR was 7 (3-16) days. The median time to reach the peak of platelet counts were 10 (3-21) days. rhTPO was used for a median of 7 (3-14) days. The median platelet counts of patients undergoing surgery before rhTPO therapy, before surgery and at day 7 after surgery were 33 (20-89) ×109/L, 125 (78-245) ×109/L and 72 (30-250) ×109/L, respectively. The median peak of platelet counts was 149 (101-466) ×109/L. No infection, bleeding, thromboembolism and therapy-related adverse responses occurred in the patients.@*Conclusion@#rhTPO for ITP patients during the perioperative period is safe and effective.
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Objective@#Abstract: Objective: To investigate the expression and significance of miR-138 and programmed cell death protein 1 (PD-1) in the patients with hepatitis B virus (HBV)-related hepatocellular carcinoma (HCC). @*Methods@#A total of 30 patients with HBV-related HCC, 20 with HBV-related cirrhosis (LC) and 30 with chronic hepatitis B (CHB) were recruited from Jiaozuo People′s Hospital. The blood samples from all patients and the peritoneal effusion samples from HCC and LC patients were collected. The levels of miR-138 and soluble PD-1 (sPD-1) in blood and peritoneal effusion samples were detected by real-time PCR and ELISA, respectively. The expressions of PD-1 in T lymphocytes were measured with flow cytometry and western blot. The targeting effect of miR-138 on the 3′-non-coding region (3′-UTR) of PD-1 gene was verified by the dual-luciferase reporter gene system. @*Results@#The relative expression levels of miR-138 in the peritoneal effusion and plasma of HBV-related HCC patients were significantly lower than those in LC and CHB patients (P<0.05). The serum sPD-1 levels and the expression levels of PD-1 in CD3 + T lymphocytes of HBV-related HCC patients were significantly higher than those in LC and CHB patients (P<0.05). The relative expression levels of miR-138 were negatively correlated with serum sPD-1 levels and the expression levels of PD-1 in CD3 + T lymphocytes (P<0.05). The dual-luciferase reporter gene system and western blot results demonstrated that there was a targeting relationship between miR-138 and the 3′-UTR of PD-1 gene. After miR-138 was transfected, the expression level of PD-1 was significantly down-regulated. @*Conclusion@#miR-138 participates in the development and progression of HBV-related HCC probably by targeting PD-1.
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Objective To observe the effec t and safety of posaconzole in prophylactic antifungal treatment of high-risk patients with hematological malignancies. Methods The effect of posaconzole on prevention of invasive fungal disease (IFD) during chemotherapy in 102 patients with hematological malignancies in Shanghai Ruijin Hospital from February 2014 to April 2017 were analyzed retrospectively. The correlation analysis was carried out by Spearman analysis. Results A total of 102 patients were included, and only 2 (1.96%) patients had IFD. 2 patients died because of development of hematological malignancies. 11 (10.8%) patients had to discontinuation of posaconzole because of sever adverse events. Two patients with IFD and 1 case of undiagnosed IFD had to discontinue posaconzole. The total rate of discontinuation was 13.7 % (14/102). There was no correlation between the time of using pothiazol and the time of hospitalization (rs= -0.02, P= 0.853) and the time of neutrophilic deficiency (rs= 0.167, P= 0.113), but the time of hospitalization was positively correlated with the time of neutrophilic deficiency (rs = 0.448, P=0.000). Conclusion Posaconzole could effectively and safely prevent IFD in patients with hematological malignancies.
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Objective@#To analyze the diagnosis and treatment of acute myeloid leukemia (AML) complicated with myeloid sarcoma (MS).@*Methods@#The clinical features, diagnosis and therapy of 3 AML who were treated with routine regimen induction and consolidation therapy in Shanghai Beizhan Hospital and showed MS during the marrow remission stage were retrospectively analyzed.@*Results@#Three female patients included 2 cases of CBFβ-MYH11 positive and 1 case of AML1-ETO positive. One patient occurred in the first complete remission(CR1) and 2 patients occurred in CR2. The involved sites included brain, vagina, and breast respectively. Three patients all received short efficacy after chemotherapy, including 1 patient in bone marrow remission stage, 1 patient died of after bone marrow relapse and 1 patient without follow-up.@*Conclusions@#For AML patients in remission with extramedullary lesions, pathological examination is warranted to confirm the diagnosis of the disease. Systemic chemotherapy is still the main treatment for MS. Central nervous system(CNS) leukemia treatment should be performed to the patients with CNS involved. Allogeneic hematopoietic stem cell transplantation may be the best option for the treatment of these patients.
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Objective@#To analyze the disease progression of acute leukemia (AL) transformed from myeloproliferative neoplasms (MPN) with Philadelphia chromosome-negative (Ph-), and to investigate its mechanism and clinical treatments.@*Methods@#The pre-transformation and post-transformation data of 8 AL patients with Ph- MPN from July 2013 to December 2017 in Ruijin Hospital Affiliated of Shanghai Jiao Tong University School of Medicine were retrospectively analyzed. The literature was also reviewed.@*Results@#All 8 cases transformed into acute myelogenous leukemia (AML). The median conversion time was 47.5 months (2-180 months), and the median survival time after transformation was 2 months (1-17 months). Three of 4 patients who undergone AML-related molecular biology after transformation had new mutant genes. One refractory patient achieved stable disease after oral treatment with ruxolitinib.@*Conclusions@#AML patients transformed from MPN have poor clinical outcomes and short survival time. Allogeneic hematopoietic stem cell transplantation is the only known potential curative treatment strategy and JAK2 inhibitor may be effective.
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Kandelia obovata Sheue, Liu & Yong, a mangrove species which distributed in tropical, subtropical coastal and estuarine intertidal, has important ecological functions in coastal ecosystems. Here, we reviewed several aspects of the recent research progress in molecular biological studies of K. obovata. We focused the phylogeography and genetic diversity of this species by several types of molecular markers, proteome analyses based on two-dimensional electrophoresis platform accomplished for this species, and functional genes isolated under non-biotic stress environment. Finally, based on the current research progress, we proposed some orientations for future molecular biological research on K. obovata.
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Objective · To compare the efficacy and prognostic factors of HCAG regimen with traditional IA regimen in the treatment of newly diagnosed elderly acute myeloid leukemia (AML) patients. Methods · Forty-one patients with AML (aged 55-71 years) were randomly divided into two groups (Group HCAG and Group IA) between 2014 and 2016 for induction and consolidation therapy. Multivariate analysis was applied to identify prognostic factors for relapse-free survival (RFS). Results · A total of 29 patients (70.7%) achieved complete remission (CR). The estimated 2-year overall survival (OS) was 66.8% in Group HCAG and 75.4% in Group IA (P=0.913). The estimated 2-year RFS was 61.8% in Group HCAG and 49.1% in Group IA (P=0.411). Age remained as the unfavorable prognostic factor, leading to significant differences in OS and RFS. In addition, RFS was influenced by cytogenetic/molecular risk stratification. Conclusion · Although HCAG seemed not to particularly benefit the group, the dose reduction of anthracyclines may be applied in elderly patients with comparable short-time outcome. Furthermore, the introduction of homoharringtonine resulted in an improvement of treatment response for more than 20% compared with CAG regimen.
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One hundred fifty-three patients with hypoxia of both sexes,underwent hip arthroplasty from January 2012 to January 2014,aged 65-97 yr,weighing 41-80 kg,living in areas at altitude above 1800 m,of American Society of Anesthesiologists physical status Ⅱ-Ⅵ,were selected.The hemoglobin (Hb) at 3-4 days after operation was collected,and the patients were divided into 3 groups depending on whether or not postoperative anemia occurred:no anemia group (Hb> 110 g/L);mild anemia group (90 g/L≤ Hb≤ 110 g/L);moderate anemia group (70 g/L ≤ H b< 90 g/L).The 36-item Short-Form Health Survey score and Harris Hip Score at 28 days after operation were collected.There was no significant difference between the three groups in the postoperative 36-item Short-Form Health Survey score and Harris Hip Score (P>0.05).Postoperative mild and moderate anemia did not affect the short-term outcome after hip arthroplasty in elderly patients in high-altitude regions.
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Objective To evaluate the effect of class 10000 laminar flow ward on the incidence of healthcare-asso-ciated infection (HAI)in patients with initial occurrence of acute leukemia during induction chemotherapy period. Methods Patients with initial occurrence of acute leukemia admitted to a hematological department of a hospital be-tween October 2013 and June 2014 were investigated retrospectively,patients in class 10000 laminar flow ward was as trial group,in general ward was as control group. All patients received standard induction chemotherapy and the same nursing measures,the incidence of HAI between two groups of patients,and ward air cleanliness were com-pared.Results A total of 79 patients with initial acute leukemia were received (trial group,n= 39;control group, n= 40). The average air cleanliness value in rooms and corridors of laminar flow wards were both significantly dif-ferent with general ward (3.57×106/m3 vs 149.36×106/m3 ,t= 45.80,P<0.001;24.46×106/m3 vs 15854.38 ×106/m3 ,t= 108.70,P<0.001). Incidence of HAI between trial group and control group was significantly differ-ent (23.08% [9/39]vs 45.00% [18/40],χ2= 4.219,P= 0.040). The main infection site in trial group was gastro-intestinal tract (n= 5 ),in control group was lower respiratory tract (n= 8 ). The duration of fever,duration and cost of antimicrobial use in trial group were (6.20±2.10)d,(9.35±2.12)d,and (27113.79±1559.03)yuan re-spectively,in control group were (10.20±2.90)d,(14.15±3.14)d,and (58566.29±2217.54)yuan respectively, difference in duration of fever and cost of antimicrobial use between two groups were all significant(t= 1.021, 1377.45,both P<0.05).Conclusion Laminar flow ward can reduce the incidence of HAI in patients with initial occurrence of acute leukemia,and decrease cost of antimicrobial use.
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Objective To evaluate the efficacy of PAD regimen (bortezomib, doxorubicin, dexamethasone) and VAD-like T regimen (vincristine, doxorubicin/doxorubicin derivatives, dexamethasone combined with thalidomide) in the treatment of patients with newly diagnosed multiple myeloma (MM). Methods The efficacy of 54 patients with MM who received VAD like-T regimen and 72 patients with MM who were treated with PAD regimen, including complete remission (CR) rate, very good partial remission (VGPR) rate, overall response rate (ORR), overall survival (OS), progression-free survival (PFS) and adverse events, were retrospectively analyzed. Results The CR rate of PAD group was higher than that of VAD-like T group [31.5 % (23/72) vs. 9.3 % (5/54), χ2=0.30, P=0.002]. The VGPR rate and ORR of PAD group were not statistically higher than those of VAD-like T group [16.7 % (12/72) vs. 16.6 % (9/54), P=0.180; 82.2 %(65/72) vs. 81.5 % (44/54), P=0.190, respectively]. Median PFS of PAD group was significant longer than that of VAD-like T group [(38.2±2.2) months vs. (28.0±7.6) months, P=0.017]. The 3- and 5-year OS rates of PAD group were higher than those of VAD-like T group, but there were no significant differences between two groups (P>0.05). In terms of the adverse events, the incidence of peripheral neuropathy in PAD group was significantly higher than that of VAD-like T group [31.5 % (23/72) vs. 14.5 % (8/54), P=0.03]. Conclusions Compared with PAD protocol, the CR and median PFS of VAD-like T regimen are poor, however, VGPR,ORR, PFS and 5-year OS are similar between the two groups, and VAD-like T regimen is safer with low incidence of peripheral neuropathy. VAD-like T regimen as the first-line treatment is effective and well-tolerated, especially for newly diagnosed MM patients not suitable for transplantation and bortezomib.
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Objective To investigate the clinical,safety and cost effect of porcine pulmonary surfactant (import)and bovine pulmonary surfactant(domestic)with full dose and low dosage of early treatment for late preterm children with respiratory distress syndrome,for the clinical selection treatment programs provide a reference.Methods Late preterm children with respiratory distress syndrome were divided into full dose group A of 24 cases,low dose group A of 25 cases,full dose group B of 26 cases,low dose group B of 24 cases.The first dose of full dose group A was 200mg/kg,the first dose of low dosage group A was 100mg/kg,again administered doses was 100mg/kg,the first dose of full dose group B was 70mg/kg,the first dose of low dosage group B was 50mg/kg,again administered dose was 50mg/kg.Results The differences of PaO2 ,PaCO2 ,FiO2 ,OI between two low dosage groups and full dose group A were significantly different.Treated 6h,the differences of PaCO2 ,FiO2 between low dosage group B and full dose group A were significantly different(q =3.273,3.236,all P <0.05).A group of low volume with a sufficient amount PaO2 6 h B group differences were statistically significant(q =3.823,P <0.05),12h of PaO2 ,PaCO2 ,FiO2 ,OI with enough B group difference was statistically significant(q =3.293,3.923,3.423,3.434,all P <0.05),24h of FiO2 , OI group was significantly lower than the full amount of B(q =3.234,3.356,all P <0.05).Low dose group B 6h, 12h,24h of PaO2 was significantly lower than enough group B,6h,12h of PaCO2 were significantly higher than enough group B(q =3.892,3.922,3.583,all P <0.05),12h,24h of FiO2 ,OI group were significantly higher than a suffi cient amount B(12h of q =3.892,3.762,24h of q =3.819,3.678,P <0.05),24h of OI was significantly higher than the low amount of a group(q =3.784,P <0.05).Chest evaluation 12h,24h grade difference between groups was sta-tistically significant(Z =3.982,5.323,all P <0.05).Two low -volume set of ventilator -associated pneumonia rates were 16.00%,16.67%,significantly higher than the amount of the two -legged group(low dose group A χ2 =4.988, 5.023,low dose group B χ2 =5.102,5.234,all P <0.05).Conclusion Porcine pulmonary surfactant(import)and bovine pulmonary surfactant(domestic)full dose early treatment of late preterm children with respiratory distress syn-drome could significantly relieve pulmonary symptoms and signs,effects are better than low dosage,and has high secu-rity,low dosage treatment does not reduce the cost of treatment,bovine pulmonary surfactant therapy administered in an amount sufficient lower cost,select the appropriate treatment plan based on your specific needs.
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Objective: To optimize the water decocting extraction procedures for Ganyangkang capsules by orthogonal design. Methods:Using the extraction yield and emodin content as the indices, the optimal extraction conditions for Ganyankang capsules were studied by L9(34) orthogonal test. Results:The influencing order of the factors was B>A>C (A:extracting time, B:extracting du-ration, C:added water volume) . The optimal preparation conditions were A3 B3 C2 , i. e. adding 10-fold water, decocting extracting 3 times with 2 hours each time. Conclusion:The optimum water decocting extraction procedures is rational and feasible. It can be used in the real preparation.
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Objective To investigate the effects of escitalopram (ESC)on the cognitive function,the expression of brain-derived neurotrophic factor (BDNF)in the hippocampus,the dendritic length and arborization and dendritic spines density of chronic cerebral ischemic rats.Methods Rats were randomly divided into sham-operation group,model group (permanent occlusion of bilateral common carotid arteries,2VO)and experimental group (treated with escitalopram at the dosage of 30 mg/kg·d).Rats were selected as study objects at week 1,2,4 and 8 after administration in each group.Their cognitive function was evaluated by the Morris water maze,the expression of BDNF protein was measured by Western blot,and dendritic morphology was studied by Golgi staining. Results In the Morris water maze test,the escape latency obviously extended in model group and experimental group compared with that in sham-operation group (P<0 .0 5 ),while the escape latency was shorter in experimental group than in model group (P<0.05).Compared with those in sham-operation group,the dendritic length and arborization and the density of dendritic spines in hippocampal CA1 significantly decreased in model group and experimental group (P<0 .0 5 ),while they increased significantly in experimental group compared with model group (P<0.05)by Golgi staining.Compared with sham-operation group,the expression of BDNF in the hippocampus of experimental group and model group significantly decreased (P<0 .0 5 ),but it increased significantly in experiment group compared with model group (P<0.05)by Western blot.Conclusion Escitalopram could significantly delay the progression of cognitive impairment induced by chronic cerebral ischemia in rats.The improvement of learning and memory may be related to the increased expression of BDNF.
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<p><b>OBJECTIVE</b>To explore the prognostic significance of Ph-positive and/or BCR-ABL positive acute lymphoblastic leukemia (Ph⁺ ALL).</p><p><b>METHODS</b>A retrospective analysis of 72 patients with Ph⁺ ALL to probe prognostic factors including sex, age, high white cell counts at diagnosis, additional chromosome abnormality, BCR-ABL transcripts type, imatinib based therapy, allo-HSCT and complete remission (CR) after one-course induction on the outcomes of Ph⁺ALL patients.</p><p><b>RESULTS</b>Of 72 patients with median age 40.5 (13-68) years, 38 patients received imatinib plus chemotherapy. With median follow-up of 11 (0.2-96) months, total CR rate in patients receiving imatinib plus chemotherapy was higher than of patients receiving chemotherapy only (97.4% vs 62.3%, P=0.019). High white blood counts at diagnosis or additional chromosome abnormality had no effects on CR rate. 2-year overall survival (OS) and disease free survival (DFS) in imatinib plus chemotherapy group were (28.9±7.4) % and (25±7.4) %, respectively, which were higher than those in chemotherapy group (P<0.001). OS rate in HSCT group was significantly higher than that in non-HSCT group[ (61.1±11.5) % vs (5.6±3.1) %, P<0.001]. Multivariate prognostic analysis for OS showed that imatinib-based therapy [RR=0.413 (95% CI 0.237-0.721), P=0.002], allo-HSCT [RR=0.175 (95% CI 0.075-0.389), P=0.000] and CR after one-course induction [RR=0.429 (95% CI 0.245-0.750), P=0.003] were of importance for survival.</p><p><b>CONCLUSION</b>allo-HSCT was an optimal choice for Ph⁺ALL patients. Imatinib-based therapy could increase CR rate, maintain CR duration and decrease relapse, resulting in more chance of HSCT. Imatinib improved the outcomes of Ph⁺ALL patients who were not eligible for HSCT.</p>
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Adolescent , Adulte , Sujet âgé , Femelle , Humains , Mâle , Adulte d'âge moyen , Jeune adulte , Protocoles de polychimiothérapie antinéoplasique , Utilisations thérapeutiques , Benzamides , Utilisations thérapeutiques , Survie sans rechute , Protéines de fusion bcr-abl , Transplantation de cellules souches hématopoïétiques , Mésilate d'imatinib , Chromosome Philadelphie , Pipérazines , Utilisations thérapeutiques , Leucémie-lymphome lymphoblastique à précurseurs B et T , Diagnostic , Génétique , Thérapeutique , Pronostic , Inhibiteurs de protéines kinases , Utilisations thérapeutiques , Pyrimidines , Utilisations thérapeutiques , Études rétrospectivesRésumé
<p><b>OBJECTIVE</b>To investigate the clinical and hematological features and prognostic factors of primary myelofibrosis (PMF) patients in Shanghai and surrounding area of Shanghai, China.</p><p><b>METHODS</b>The clinical manifestations, laboratory parameters and survival were retrospectively analyzed in 75 PMF patients diagnosed from Jan, 1996 to Dec, 2013 in our hospital and were compared with those of Chinese subjects from Tianjin and foreign Caucasian patients, respectively. Comparison of categorical variables was performed by χ² test. Survival was estimated using the Kaplan- Meier method. Log- rank test was used to compare survival date. A Cox model was used for multivariate analyses.</p><p><b>RESULTS</b>The median age of the 75 patients was 56(19-81) years old. There were 51(68%) patients with HGB less than 100 g/L. The median value of HGB was 83 g/L. Similar with those from Tianjin, the patients in our study were significantly younger with higher proportion of severe anemia and lower platelet counts when compared with foreign Caucasian patients. Using IPSS and dynamic international prognostic scoring system (DIPSS) model, the survival curves of intermediate- 1, intermediate- 2 and high risk groups were significantly different. In univariate analyses, variables significantly correlated with poor prognosis were systemic symptoms, HGB<100 g/L, HGB<80 g/L, PLT ≤ 100 × 10⁹/L, WBC<10 × 10⁹/L and blood blasts ≥ 0.01. Multivariate analysis identified IPSS and HGB < 80 g/L as independent risk factors for survival.</p><p><b>CONCLUSION</b>Chinese PMF patients may have characteristics of younger age at onset with more severe anemia. However, IPSS and DIPSS model are still suitable for Chinese patients to predict survival. To increase the weight of anemia severity may provide a better prognostic stratification for Chinese patients with PMF.</p>
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Adulte , Sujet âgé , Sujet âgé de 80 ans ou plus , Humains , Adulte d'âge moyen , Jeune adulte , Chine , Myélofibrose primitive , Diagnostic , Pronostic , Études rétrospectives , Facteurs de risqueRésumé
Objective To investigate the clinical characteristics and survival analysis of T-cell lymphoma with hemophagocytic syndrome (HPS).Methods The clinical features,laboratory tests and survival of 30 patients of T-cell lymphoma with HPS were analyzed comparing with 50 random patients of T-cell lymphoma without HPS (control group),and the data were obtained from department of hematology,Ruijin Hospital,Shanghai Jiaotong University School of Medicine from January 2006 to December 2011.Results 30 cases of T-cell lymphoma with HPS manifested for high fever [100.00 % (30/30)],splenomegaly [96.67 % (29/30)],more than two-line reduction in blood cells [93.33 % (28/30)],bone marrow with bloodthirsty phenomenon [86.67 % (26/30)],LDH increased [100.00 % (30/30)],hypertriglyceridemia [46.67 % (14/30)],low fibrin hyperlipidemia [60.00 % (18/30)],ferritin increased [93.33 % (28/30)],liver damaged [86.67 % (26/30)] from patient group,which were higher than those of control group,the differences were statistically significant (x2 =23.11,22.50,36.05,64.20,21.82,5.31,16.54,26.82,46.43,all P < 0.05).But the incidence of superficial lymph nodes enlargement was not high,only 33.33 %,which made the diagnosis of primary disease difficult.Combined chemotherapy of CHOP or etoposide plus dexamethasone could make the symptoms and laboratory indicators improved,but the median survival time was only 20 days.Compared with the control group,there were significant differences on the survival curves (x2 =35.05,P < 0.0001).Conclusion In T-cell lymphoma with HPS,the clinical manifestations are complex,usually with multi-organ dysfunction,aggressive course,and poor prognosis.The pathogenesis and treatment of HPS should be further studied.