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Objective:To investigate the correlations between perfused lung volumes, visual scores (using perfusion SPECT/CT) and right-heart catheter (RHC) hemodynamic parameters in patients with chronic thromboembolic pulmonary hypertension (CTEPH).Methods:A total of 51 consecutive CTEPH patients (17 males, 34 females, age (59±12) years) in the First Affiliated Hospital of Guangzhou Medical University between March 2015 and July 2019 were retrospectively analyzed. All patients underwent lung perfusion SPECT/CT imaging and RHC examinations. Perfused lung volumes were determined using threshold-based (15%-85%) segmentation. Visual semiquantitative scoring in each lung segment was performed using Begic method. RHC hemodynamic parameters including pulmonary artery systolic pressure (PASP), pulmonary arterial diastolic pressure (PADP), mean pulmonary artery pressure (mPAP), pulmonary arteriolar wedge pressure (PAWP), pulmonary vessel resistance (PVR), cardiac output (CO), cardiac index (CI) were recorded. Spearman correlation analysis was used to evaluate the correlations between perfused lung volumes, visual scores and hemodynamic parameters.Results:There were significant correlations between perfused lung volumes (30%-70% threshold) and mPAP ( rs values: from -0.414 to -0.302, all P<0.05). Among them, perfused lung volumes under the threshold of 40% and 45% were moderately correlated with mPAP ( rs values: -0.414, -0.412, both P<0.05). Perfused lung volume (40% threshold) was moderately negatively correlated with PASP, PADP ( rs values: -0.402, -0.440, both P<0.05), and slightly negatively correlated with PVR ( rs=-0.352, P<0.05). Visual scores were slightly positively correlated with the PADP ( rs=0.311, P<0.05), while there was no correlation between visual scores and other RHC hemodynamic parameters ( rs values: from -0.201 to 0.275, all P>0.05). Conclusion:Perfused lung volumes based on threshold-based segmentation in lung perfusion SPECT/CT imaging can accurately reflect hemodynamic status and may provide useful information for severity assessment of CTEPH.
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Objective To analyze the clinical characteristics, pathological types, treatment modalities of lymphoma patients with mediastinal bulky masses. Methods The clinical data of 17 lymphoma patients with mediastinal bulky masses from January 2010 to January 2017 in Beijing Friendship Hospital were analyzed retrospectively. Results All the patients were pathologically diagnosed, including 6 cases of primary mediastinal large B-cell lymphoma , 5 cases of lymphoblastic lymphoma , 3 cases of Hodgkin lymphoma and 3 cases of other non-Hodgkin lymphoma. Four cases with bulky disease were treated with combined chemoradiotherapy regimen, 7 cases were treated with combined chemotherapy, 5 cases were treated with combined chemotherapy regimens followed by autologous bone marrow transplantation and 1 case was treated with high dose chemotherapy followed by allogeneic stem cell transplantation. The effects were evaluated after the final treatments. Twelve patients achieved complete remission, 1 case achieved partial remission, 1 case achieved stable disease, 1 case achieved progressive disease and 3 cases died. Conclusions The prognosis of lymphoma patients with mediastinal bulky masses is related with histopathology and treatment response. Combined treatment modalities including chemoradiotherapy regimen and chemotherapy regimen followed by autologous or allogeneic stem cell transplantation are helpful to improve treatment effects.
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Objective To analyze the clinical characteristics of adult patients with hemophagocytic lymphohistiocytosis (HLH) receiving haploidentical donor hematopoietic stem cell transplantation (HID HSCT).Method We retrospectively reviewed 20 adult patients with HLH from August 2009 to August 2014.The clinical features and outcome were analyzed.Results Conditioning regimens consisted of total body irradiation/etoposide/cyclophosphamide (TBI/VP-16/CTX) and busulfan (Bu)/VP-16/CTX in HLH with anti-thymocyte globulin (ATG) 8 mg/kg.The stem cells were mobilized from donors' peripheral blood.Median time to white blood cell engraftment was 13 (9-27) days.Median time to platelet engraftment was 14 (10-28) days.Mixed chimerism after transplantation developed in 4 patients and no patient presented graft failure.Eight patients developed grade Ⅱ to Ⅲ acute graft-versus-host disease (GVHD),while as chronic GVHD occurred in 9 patients.Among 12 patients with EB virus (EBV) reactivation,2 patients developed post-transplant lymphoproliferative disorder (PTLD),7 were suspected as PTLD and 3 were considered as relapse of primary disease.With a median follow-up of 20 months (range:0.5-108 months) after transplantation,the estimated 2-year overall survival (OS) rate was (60.0 ± 11.0) ％ in all patients.During the follow-up,12 patients survived,8 died including 5 within 100 days after HSCT.Among 5 non-remission patients before HSCT,4 patients died within 100 days after HCT.Conclusions HID HSCT is an effective treatment for adult patients with HLH to achieve remission and long-term survival.High proportion of mixed chimerism has been seen at early stage after transplantation.EBV reactivation and early transplant-related mortality are common.
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Objective To explore the clinical effect and toxicity of daunorubicin combined with cytarabine (DA regimen) and idarubicin combined with cytarabine (IA regimen) for the treatment of patients with acute myeloid leukemia (AML) as induction chemotherapy. Methods The clinical data of 84 newly diagnosed AML patients (except M3) treated with DA or IA regimen were analyzed retrospectively. DA regimen group included 32 patients (17 males and 15 females with median age of 46 years), while IA regimen group included 52 patients (29 males and 23 females with media age of 49 years). Efficacy index was complete remission (CR), total efficiency and adverse reactions after one course of chemotherapy rate. Results In DA regimen group,the CR rate was 65.6 %(21/32), and the total efficiency rate was 75.0 %(24/32), while in IA regimen group, the CR rate was 71.2 %(31/52), and the total efficiency rate was 80.8 %(42/52), respectively, but, the differences of media survival and 5-year survival rate were not statistically significant (16.8 months vs. 24.9 months, 26 % vs. 44 %, both P>0.05). The main side effect in the two groups included hematologic (bone marrow suppression) and non-hematologic adverse reactions, with no significant difference between the two groups (all P>0.05). Conclusion For newly diagnosed AML patients, remission rate and total efficiency of DA regimen are same as IA regimen after one course treatment, and adverse events between the two regimens do not differ significantly.
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Objective To investigate the efficacy of rituximab-containing regimen in Epstein-Barr virus associated hemophagocytic lymphohistiocytosis (EBV-HLH). Methods A retrospective analysis involving 6 EBV-HLH patients who had received treatment with rituximab-containing regimen was performed. The patients who were diagnosed with lymphoma or primary HLH subsequently were not included in the analysis. Results All patients were males. The median age was 27.5 years (range 20-61 years). Two patients received rituximab-containing regimen as primary therapy, and got partial remission (PR) within 2 weeks after the first course of rituximab, but relapsed within 4 weeks. Four patients received rituximab-containing regimen as salvage therapy, but none achieved remission. The 6 patients died due to HLH and complications, such as infection and hemorrhage. Laboratory data including white blood cell count, haemoglobin concentration, platelet count ferritin, alanine transaminase, aspartate transaminase,total bilirubin, fibrinogen and EBV-DNA did not show statistical significance (all P>0.05). Conclusion The efficacy of rituximab as a treatment for EBV-HLH is not as good as that in the previous study, and a prospective clinical trial of rituximab-based monotherapy is needed to answer the question.
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Objective To explore the clinical efficacy and safety of low-dose decitabine combined with cytarabine for myelodysplastic syndromes (MDS). Methods Clinical data of 15 patients with MDS who took the therapeutic regimen with decitabine combined with cytarabine were collected from January 2012 to January 2015. The clinical efficacy and adverse effects were assessed. Results Among the 15 patients, 4 cases were complete remission (CR), 5 cases were partial remission (PR) and 6 cases were stable disease (SD) and progressive disease (PD). The total effective rate was 60.0 % (9/15). Grade Ⅲ-Ⅳ bone marrow depression occurred in 11 cases with incidence rate of 73.3 % (11/15), and the total incidence rate of infection was 40.0 % (6/15), including lung infection of 26.7 % (4/15). All the infections were controlled after active supportive treatment and anti-infection therapy. No patient died of chemotherapy. Conclusions Low-dose decitabine combined with cytarabine can effectively treat MDS and delay the progress of disease. The patients can tolerate the adverse effects in chemotherapy with a low mortality rate.
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As originated from hematopoietic stem cell clonal diseases,bcr-abl-negative chronic myeloproliferative neoplasms (MPN) include polycythemia vera (PV),essential thrombocythemia (ET) and primary myelofibrosis (PMF).With the discovery of JAK2 mutations,many new mutations have been identified.With the in-depth study of gene mutation,the pathogenesis of MPN has been gradually uncovered.Novel drugs have been developed accordingly.
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Objective To investigate the clinical and pathological characteristics,treatment response and clinical follow-up for patients with mantle cell lymphoma (MCL).Methods Clinical data,treatment efficacy and outcomes of 25 cases of MCL enrolled from Beijing Friendship Hospital were retrospectively analyzed.Results Median age of onset was 65 years old.Male to female ratio was 3.4:1,15 cases (60 ％) had bone marrow invasion,Ann Arbor clinical stage Ⅲ were 6 cases (24 ％),stage Ⅳ were 17 cases (68 ％).10 cases (40 ％) of patients had symptoms B.5 cases (20 ％) patients had elevation of lactate dehydrogenase (LDH).17 cases (64 ％) had elevation of β2 microglobulin (β2-MG).11 eases (64.71％) had complete remission (CR) after rituximab combined chemotherapy in 17 patients,2 years overall survival (OS) rate was 69.6 ％,2 years progression-free suvival (PFS) rate was 45.1％,significantly higher than those in traditional chemotherapy group (P ＜ 0.005).Outcomes analyses showed that variants of plasma cells,bone marrow invasion,LDH elevation,Ki-67 level,sMIPI ＞ 5 scores and increased leukocyte number were negative prognostic factors.IPI score,splenomeagly,age,symptoms B and elevation of [β2-MG had no predicative value.Conclusion MCL has higher invasion and poor prognosis.Rituximab combined chemotherapy could significantly improve CR,PFS and OS rates.
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Objective To investigate the effects of ubiquitin isopeptidase inhibitor Ⅰ on proliferation and apoptosis of human leukemic K562 cells.Methods Human leukemic K562 cells were treated with different concentration of ubiquitin isopeptidase inhibitor Ⅰ.Cell proliferation was monitored by MTT assay.Cell apoptosis was analyzed by flow cytometry after Annexin V/PI staining,and mRNA expression levels of bcl-2,bax and Caspase-3 were quantified by reverse transcription-polymerase chain reaction (RT-PCR).Results MTT assay showed that the proliferation of K562 cells was markedly inhibited by ubiquitin isopeptidase inhibitor Ⅰ in a time-and-dose dependent manner.The Annexin V positive rate of K562 cells after treatment with ubiquitin isopeptidase inhibitor Ⅰ was significantly increased by FCM analysis (P ＜ 0.05).The early apoptosis rate of K562 cells treated with 100 nmol/L ubiquitin isopeptidas inhibitor Ⅰ by 72 h,was obviously increased compared to control cells [(15.4±1.3) ％ vs (4.1±0.9) ％,P ＜ 0.01].The mRNA levels of Caspase-3 and bax were up-regulated and bcl-2 was down-regulated after treated with ubiquitin isopeptidase inhibitor Ⅰ,and the differences were statistically significant from control cells (P ＜ 0.05).Conclusion Ubiquitin isopeptidase inhibitor Ⅰ has inhibitory effect on proliferation and inductive effect on apoptosis of K562 leukemia cells,implying its possible application in treating leukemia.
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Objective To investigate the therapeutic outcomes of chemotherapy with infusion related HLA-mismatched hematopoietic stem cells for hematologic malignancies.Methods Therapy responses and hematopoietic recovery as well as complications of 9 patients were analyzed.Results In the 9 patients aged 29-67 years,four were acute myeloid leukemia,one was acute B lymphocytic leukemia,two were multiple myeloma,one was Hodgkin' s disease,one was diffuse large B cell lymphoma.The average of MNC was (3.12±1.29)×108/kg,CD34+ cells was (1.71±1.00) ×106/kg,and CD3+ cells was (2.13 ±0.99) ×108/kg.There was complete remission in four patients,partial remission in one,disease progression in four.Following up 2 to 14 months,four patients were in survival.No donor chimerism was detected and no graft-versus-host disease was observed in any patient.Conclusion Chemotherapy with infusion related HLA-mismatched hematopoietic stem cells for hematologic malignancies may provide a promising treatment method as a novel therapeutic strategy.
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Objective To explore the use of antithymocyte globulin in peripheral blood stem cell transplantation from HLA-identical sibling donors. Methods Fifty-seven adults with hematological malignancy were treated with a first myeloablative matched related donor peripheral blood stem cell transplantation with pretransplantation of ATG. All patients received modified Bu/cy conditioning regimen or Cy/TBI conditioning regimen. GVHD prophylaxis protocol included Rabbit ATG (thymoglobulin, genzyme),Cyclosporin A, methotrexate and mycophenolate mofetil. Results All patients underwent engraftment with neutrophils and platelets. Incidence of aGVHD was 36.8 %. Gades Ⅰ - Ⅱ was observed in 31.6 %. Grade Ⅲ was 5.2 %. Grade Ⅳ was not seen. Incidence of cGVHD was 33.3 %, including two extensive cGVHD cases.Infection had occurred in twenty-two patients during transplantation. Twenty-nine (50.9 %) patients occurred CMV reactivation. The 5-year estimated OS is 71.35 %. The 5-year estimated DFS is 68.61%. Conclusion Pretransplantation with low dose ATG given to recipients undergoing MRD PBCT may result in less aGVHD and cGVHD. No cases of posttransplantation lymphoproliferative disease was observed. Significant morbidity and mortality from opportunistic infection were not seen. Relapse remains a problem among high-risk patients.
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Using the semi-empirical MNDO/H method several systems simulating the reaction of tetrahetral intermediate formation in the active site of serine proteases have been studied. The role played by elements of the《catalytic triad》in increasing the reactivity of serine hydroxyl has been discussed.The formation of a strong hydrogen bond between His and Asp was shown to be important in lowering the activation energy in the reaction of Ser with substrate.The change in position of the proten located between Ser and His and between His and Asp was analysed.The influence of substrate distortion on the energy of intermediate formation has been considered.