Clinical study of DEP regimen as a salvage therapy for refractory macrophage activation syndrome / 中华风湿病学杂志

Objective:To investigate the efficacy of liposomal doxombicin combined with etoposide and high dose methylprednisolone (DEP) as a salvage therapy for refractory macrophage activation syndrome (MAS).Methods:Totally 38 patients with refractory MAS were enrolled in this study from January 2016 to January 2022 in Beijing Friendship Hospital, including clinical characteristics and laboratory test results before and after DEP treatment, were retrospectively collected. The efficacy was evaluated every 2 weeks according to the United States Midwest Cooperative HLH Group. Relevant samples were statistically analyzed using non-parametric tests.Results:Of 38 refractory MAS patients, 8 males and 30 females were included into this study.The median age was 30(15-69) years old. The underlying disease were adult onset Still's disease in 29 cases, Systemic lupus erythematosus in 6 cases, Rheumatoid arthritis in 1 case and Undifferentiated Connective-Tissue disease in 2. The overall response rate was 95% (36/38), including 9 patients (24%) achieved complete remission and 27 patients (71%) achieved partial remission after 2 weeks of treatment. The overall response rate was 97% (34/35), including 16 (46%) complete remission and 18 (51%) partial remission after 4 weeks of treatment(due to lack of data in some patients). The overall response rate was 97% (34/35), including 17 (49%) complete remission and 17 (49%) partial remission after 6 weeks of treatment. Patients who achieved partial remission or complete remission were actively treated for the underlying diseases after induction, and their conditions were in persistent remission.Conclusion:The DEP regimen may be an effective salvage therapy for the treatment of refractory MAS.

Predictive value of N-terminal-pro-B-type brain natriaretic peptide and B-type brain natriaretic peptide in premature infants with symptomatic patent ductus arteriosus / 中国医师进修杂志

Objective:To explore the predictive value of N-terminal-pro-B-type brain natriaretic peptide (NT-pro BNP) and B-type brain natriaretic peptide (BNP) of symptomatic patent ductus arteriosus (sPDA) in premature infants.Methods:Sixty premature infants were recruited. The gestational age was 26-32 weeks and the birth weight was less than 1 500 g. According to the echocardiography measured on the 3rd day, the infants were divided into PDA group (40 cases) and control group (20 cases). PDA group was divided into sPDA group (20 cases) and asPDA group (20 cases). The sPDA group was divided into treatment group (10 cases) and non-treatment group (10 cases). The asPDA group was further divided into self-closed group (10 cases) and non- self-closed group (10 cases). NT-pro BNP and BNP were examined on the 3rd and 7th day.Results:The level of NT-pro BNP among sPDA group, asPDA group and control group had significant difference on the 3rd and 7th day ( P<0.05). On the 7th day, the level of NT-pro BNP between treatment group and non-treatment group had significant difference [4 082(111 - 7 566) ng/L vs. 9 643 (2 362 - 25 000) ng/L, P<0.05] between self-closed group and non- self-closed group [603 (27 - 1 698) ng/L vs. 4 595 (896 - 9 873) ng/L, P<0.05]. The level of NT-pro BNP on the 3rd day was positively correlated with of ductus arteriosus diameter, ratio of left atrium diameter and aortic root diameter, ratio of ductus arteriosus diameter and left pulmonary artery diameter ( r=0.358 - 0.455, P<0.05). The area under the curve drawn by NT-pro BNP on the 3rd day was 0.783. Conclusions:The level of NT-pro BNP on the 3rd day can be used as a predictor of sPDA.

Study on magnetic resonance monitoring index of fetal gallbladder during lategestation / 中国医师进修杂志

Objective To explore the normal reference range of fetal gallbladder in 28 to 35 weeks of pregnancy under magnetic resonance imaging characteristics, observe and explore the growth and development of fetal gallbladder, and provide a new detection method for evaluating fetal growth and development for prenatal examination. Methods One hundred single pregnant women who had underwent fetal abdominal magnetic resonance imaging from June 2017 to May 2018 in Dalian Municipal Women and Children′s Medical Center were selected. The pregnant women were divided into 2 groups according to the gestational weeks: group A (pregnancy 28 to 31 weeks, 64 cases) and group B (pregnancy 32 to 35 weeks, 36 cases). The length, width and transverse diameter of fetal gallbladder were measured by magnetic resonance, and the gallbladder volume was calculated. Results In group A, magnetic resonance result showed that 1 case had no gallbladder and 1 case had double gallbladder, but the fetal gallbladders of these 2 cases were normal after birth. The display rate of fetal gallbladder in group A was 98.4% (63/64), and that in group B was 100.0% (36/36); the abnormal rate in group A was 3.1% (2/64), and that in group B was 0. The length, width, transverse diameter and volume of gallbladder in group B were significantly higher than those in group A: (16.5 ± 6.0) mm vs. (13.2 ± 4.2) mm, (6.5 ± 3.5) mm vs. (5.2 ± 1.1) mm, (6.8 ± 2.6) mm vs. (6.4 ± 3.4) mm and (373.3 ± 284.1) mm3 vs. (258.5 ± 232.4) mm3, and there were statistical differences (P＜0.05). Conclusions Fetal magnetic resonance during lategestation has the new means of prenatal evaluation of fetal growth and development, which has clinical application value.

The application of prenatal MRI in congenital abdominal wall defect / 中国医师进修杂志

Objective To investigate the MRI findings and diagnostic value of congenital abdominal wall defect.Methods Eighteen fetuses with congenital abdominal wall defect were analyzed retrospectively, and the MRI features were analyzed. Results In 18 cases of congenital defect of abdominal wall, fetal MRI showed 6 cases had gastroschisis and 12 cases had omphalocele. They were diagnosed accurately by MRI. Conclusions Congenital abdominal wall defect has characteristic MRI findings.MRI is of great significance in prenatal diagnosis.

Application of MRI in abnormal urinary system of fetus / 中国医师进修杂志

Objective To investigate the clinical value of MRI in the diagnosis of fetal urinary system diseases.Methods The MRI images of 20 cases of genitourinary system diseases were analyzed retrospectively. Results In 20 cases, MRI showed 10 cases with urinary tract dilatation, 3 cases with ectopic kidney,1 case with renal cystic disease, and 6 cases with renal agenesis and/or renal dysplasia. Among them, MRI showed 4 cases with less/no amniotic fluid volume and 3 cases with pulmonary hypoplasia. Conclusions Prenatal MRI is an important examination method to evaluate fetal urinary system diseases.

The clinical characteristics of adult hemophagocytic lymphohistiocytosis treated with haploidentical donor hematopoietic stem cell transplantation / 中华内科杂志

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.

Inducing therapy of cytarabine combined with daunorubicin or idarubicin for the newly diagnosed acute myeloid leukemia：comparison of clinical efficacy / 白血病·淋巴瘤

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.

Clinical observation of low-dose decitabine combined with cytarabine in the treatment of myelodysplastic syndromes / 白血病·淋巴瘤

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.

Clinical study of 6 patients with Epstein-Barr virus associated hemophagocytic lymphohistiocytosis treated with rituximab-containing regimen / 白血病·淋巴瘤

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.

Treatment outcomes and prognostic analysis of 61 Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the outcomes, survival status, and the prognostic factors of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) patients.</p><p><b>METHODS</b>A retrospective study was carried out to analyze the clinical data of 61 EBV-HLH cases, from January 2008 to July 2014. Prognostic factors were analyzed through COX model (single factor and multiple factors).</p><p><b>RESULTS</b>A total of 246 patients with HLH were diagnosed, among which 102 cases were with EBV infection (including 61 EBV-HLH, 36 lymphoma associated HLH, 5 primary HLH. Among the 61 cases, 40 were male and 21 were female, with a ration of 1.9:1. The median age was 28 years (range, 12-78). 1, 3, 6 and 12-month overall survival rates of 61 EBV-HLH were 65.6%, 47.5%, 32.4%, and 25.0%, respectively. The median follow-up time was 3 (0.5-28) months. 12 patients didn't use etoposide within 4 weeks after diagnosis, while HLH-94 protocol was used in 33 patients and HLH-2004 protocol was used in 16 patients. Response rates of theses three groups were 33.3%, 51.5%, and 43.8%, respectively (P=0.401). There was statistically difference between the group without etoposide and the HLH-94/ HLH-2004 group in the overall survival rate (P=0.033). Serum albumin level (P=0.033) and whether EBV could became negative (P=0.010) were independent predictors for EBV-HLH.</p><p><b>CONCLUSION</b>EBV-HLH patients have severe clinical feature and poor prognosis. Early application of immune chemotherapy based on etoposide can improve survival. Serum albumin level and whether EBV can become negative are independent prognostic factors for survival.</p>

Two-dimensional Reconstruction and Three-dimensional Visualization of MRI Images Based on Compressed Sensing / 中国医学影像学杂志

Compressed sensing (CS) is a novel theoretical framework for information acquisition and processing. Taking advantages of the sparsity or compressibility of the signals inherent in the real world, compressed sensing can collect compressed data at the sampling rate much lower than that needed in Shannon's theorem based on random measurement matrix. This technique is used in medical imaging to accelerate MRI's scanning speed, reduce radiation dosage and alleviate patients' suffering. The whole process of the proposed algorithm was as follows: firstly, the wavelet transform was applied to achieve sparse representation of medical images and reserve certain parts with maximal coefficients; secondly, the reconstruction based on CS theory were achieved according to the improved optimized orthogonal matching pursuit (OOMP) algorithm;finally, maximum intensity projection algorithm was used to achieve three-dimensional volume reconstruction. The experimental results demonstrated that our proposed two-dimensional reconstruction method was accurate and effective, which was verified qualitatively by the local detail magnification of images and quantitatively by peak signal-to-noise ratio and sectional comparison. Therefore, the three-dimensional reconstruction can be rather helpful in clinic diagnosis and treatment.

Clinical characteristics of 192 adult hemophagocytic lymphohistiocytosis / 中华血液学杂志

<p><b>OBJECTIVE</b>To analyze the clinical manifestations, laboratory data, therapy, and prognosis in patients with hemophagocytic lymphohistiocytosis (HLH).</p><p><b>METHODS</b>A retrospective study was carried out in 192 adult patients with HLH between 2003 and 2013.</p><p><b>RESULTS</b>Of the 192 cases, 70 cases were secondary to cancer and 64 cases secondary to infection. According to HLH-2004 criteria, the coincidence rate of indices were: fever (98.96%), high level of serum ferritin (94.27%), increased level of soluble interleukin- 2 receptor(sCD25) (94.79%), decreased or absent activity of NK cells (94.27%), cytopenias (80.73%), splenomegaly (80.21%), emophagocytosis in bone marrow, spleen or lymph nodes (74.48%), hypofibrinogenemia (50.52%), hypertriglyceridemia (37.50%). In addition, 94.27% of patients were presented with liver dysfunction, 96.35% with infections, and 75.52% with coagulopathy. Incidences of central nervous system symptoms and rash were 19.27% and 20.31%, respectively. Among cancer, infection and rheumatic group, there were statistically differences on white blood cells (WBC), platelet (PLT), sCD25, alanine aminotransferase, aspartate aminotransferase, total bilirubin and globulin(GLO) (P<0.05). The differences of WBC, PLT, albumin (ALB), GLO, brain natriuretic peptide, creatinine, urea nitrogen between survival group and death group had statistical significance.</p><p><b>CONCLUSION</b>The secondary HLH occurs from various underlined diseases. Cancer, especially T- cell lymphoma, is the main cause, Secondly, it is EB virus infection. The diagnostic sensitive indicators are Persistent fever, higher level of serum ferritin, low or absent NK-cell activity, and increased sCD25 were the most valuable parameters for diagnosis. Cytopenias were not common in early phase of HLH secondary to rheumatic diseases. WBC, PLT, ALB, GLO could be used as the preliminary parameters for diagnosis. Cardiac insufficiency, renal insufficiency and coagulation dysfunction play important roles in prognosis.</p>

Clinical study of DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the efficacy of liposomal doxorubicin together with etoposide and high dose methylprednisolone (DEP) as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis (HLH).</p><p><b>METHODS</b>Total 41 patients with refractory HLH were enrolled in this study. The efficacy of treatment with DEP regimen after 2 and 4 weeks were evaluated according to the United States Midwest Cooperative HLH Group.</p><p><b>RESULTS</b>Of 41 refractory HLH patients, 28 were males and 13 females. The median age was 31(18-62) years old. The overall response rate (ORR) was 78.1%(32/41), including 12 patients (29.3%) achieved complete remission (CR) and 20 (48.8%) achieved partial remission (PR). The underlying disease of HLH were identified in 33 patients, including 1 case of primary HLH (CR), 20 cases of lymphoma associated HLH and 12 cases of EBV associated HLH. There were still 8 cases with unknown underlying disease. The 9 patients who had no response to DEP died within 2 to 4 weeks after salvage therapy. Twenty of the 32 patients who achieved PR or CR survived to undergo subsequent chemotherapy, allogenic hematopoietic stem cell transplantation (allo-HSCT) or splenectomy.</p><p><b>CONCLUSION</b>The single-arm study suggested that DEP regimen appeared to be an effective salvage protocol for adult patients with refractory HLH.</p>

Clinical characteristics and prognostic analysis of 25 patients with mantle cell lymphoma / 白血病·淋巴瘤

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.

Expression of serum thrombopoietin in patients with hemophagocytic syndrome / 白血病·淋巴瘤

Objective To investigate the expression of serum thrombopoietin (TPO) in patients with hemophagocytic syndrome (HPS) and to evaluate its clinical significance. Methods The clinical data of HPS patients from Beijing Friendship Hospital,Capital Medical University during the period from September 2008 to July 2010 were collected. They were divided into infection-associated HPS,tumor-associated HPS and rheumatological disease associated HPS according to different causes. The serum concentration of TPO in HPS patients and 26 healthy controls was measured with enzyme-linked immunosorbent assay (ELISA). Then the correlations between TPO and laboratory indicators was analyzed. Results The level of serum TPO in 26 cases of HPS was significantly lower than that of control group (P =0.001) and the variance was not statistically significant in different causes of HPS groups using ANOVA (P =0.183). The TPO levels in HPS patients showed positive correlations with serum creatinine,after we detected their TPO levels and platelets,red blood cells,hemoglobin,serum creatinine,urea nitrogen,triglyceride,fibrinogen,ferritin,NK cell activity and sCD25 levels on the same day. Conclusion TPO together with various cytokines may affect the formation of blood cells in HPS patients,and its reduction may be associated with impaired liver function. The underlying mechanism remains to be further explored.

Myeloablative regimens including pretransplant antithymocyte globulin used in peripheral blood stem cell transplantation from HLA-identical sibling donors / 白血病·淋巴瘤

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.

Analysis of complications for the treatment of uterine fibroids with bilateral uterine artery superselective embolization / 介入放射学杂志

Objective To discuss the complications of the bilateral uterine artery superselective embolization for uterine fibroids. Methods Fourty one cases(mean 42 years old) with uterine fibroids were approached by right femoral artery puncture; including thirty six cases of single hysteromyoma(33 cases with diameter 10 cm), and 5 cases of multi hysteromyoma. Bilateral uterine artery super selective 5F catheterization embolization were done along uterine descending artery(neck of uterus to vagina)with PVA particles. Rdsults Embolization succeeded with 100% and effective ratio was 92.3%. Sixteen of these cases showed post embolization syndrome, with something outflowed from vagina and 10 cases showed changes of emmenia but with no serious complications in all 41 cases. Conclusion Using bilateral uterine artery superselective embolization for uterine fibroids with PVP grains can decrease the incidence of complications. (J Intervent Radiol, 2006, 15: 280-282)