Objective To analyze the clinical features,diagnosis and treatment of thrombotic thrombocytopenic purpura (TTP) in patients with systemic lupus erythematosus (SLE).Methods Clinical manifestations,laboratory findings,diagnosis,treatment and prognosis of 14 SLE patients with TTP were retrospectively analyzed.Results Of the 14 patients diagnosed with SLE and TTP,4 were men and 10 were women.The median age at diagnosis was 23 (17-69) years old.In five patients,the onset of SLE preceded TTP,and in nine patients SLE and TTP occurred simultaneouslv.All the 14 patients had thrombocytopenia and hemolytic anemia,12 had fever,11 had neurologic abnormalities,and 11 had renal dysfunction.Eight patients presented with the classic pentad of symptoms.Six patients were given steroids (alone or in combination with intravenous immunoglobulin and cyclophosphamide),and eight patients were treated with steroids in combination with plasmapheresis,with response rates of 2/6 and 6/8,respectively.Six patients died,with overall mortality rate of 6/14.No patients relapsed during the follow-up period.Conclusions SLE and TTP share some similar clinical symptoms.As a result,repeated examinations of peripheral blood smears are very important for early diagnosis.The renal damage in patients of co-existing diseases is more serious than those with TTP alone or SLE alone.Early diagnosis and prompt treatment with plasma exchange and steroids may improve the prognosis in SLE patients with TTP.

Objective To evaluate the efficacy and safety of prophylactic cefiazidime on early bacterial infection in APBSCT recipients during neutropenia.Methods APBSCT recipients were prospectively randomly assigned to intravenous ceftazidime treatment group and control group (no prophylaxis of antibiotics).The treatment started from the first day until resolution of neutropenia or the appearance of early bacterial infection.Results From March 2010 to January 2013,70 APBSCT recipients were enrolled in the study with 36 in treatment and 34 in control group.Overall,29 (41.4％) patients developed early bacterial infection,among which,9(25.0％) in the treatment group and 20(58.8％) in the control group (P =0.004).The median infection free survival (IFS) was not reached in the treatment group and was 8 days in the control group (P =0.005).Despite whether patients received single high dose melphalan or other conditioning regimes,the early bacterial infection rate was lower in the treatment group than in the control group,and the median IFS was longer in the treatment group than that in the control group.The mean courses of antibiotic administration were (8.08 ± 2.03) days and (3.68 ± 3.56) days respectively in the treatment and control groups (P ＜ 0.001).However,the duration of empirical carbapenems were (1.67 ±3.03) days and (3.68 ±3.56) days respectively (P =0.013).There was no significant difference of antibiotics cost per patient between the two groups.Four patients in the treatment group had a transient elevated serum creatinine.Overall,no infection related mortality was observed in either group.Conclusions Prophylaxis of intravenous ceftazidime for APBSCT recipients is effective in preventing early bacterial infection with an acceptable toxicity and cost profile.However,it doesn't have effect on infection related mortality.Therefore,our results do not support the use of antibiotic prophylaxis for patients undergoing APBSCT.

A 17-year-old young man with a history of swollen leg and intermittent jaundice was presented to Peking Union Medical College Hospital with acute fever and mental disturbance.He developed deep venous thrombosis,acute myocardial infarction and plantar skin necrosis during the past four years,and was presented with an acute episode of fever,thrombocytopenia,acute kidney injury,acute myocardial infarction,mental disturbance,and obstructive jaundice.Laboratory tests showed schistocytes on peripheral blood smear.High titer of antiphospholipid antibodies was detected.Strikingly,the activity of a disintegrin and metalloprotease with a thrombospondin type 1 motif,member 13 (ADAMTS13)was significantly decreased without the production of inhibitors.Images indicated stenosis of the common bile duct,common hepatic duct,and cystic duct,which caused dilation of bile ducts and the gall bladder.Corticosteroids and anticoagulation therapy were effective at first,but the disease relapsedonce the corticosteroids tapered down.Plasma exchange was administrated for 17 times,which was effective temporarily during this episode.Methylprednisolone pulse therapy,intravenous immunoglobulin,rituximab,anticoagulation therapy,and bile drainage,were all tried but still could not control the disease.The patient's family agreed to withdraw treatment after he developed septic shock.

ObjectiveTo observe the effect of estrogenic-like effects ofWujia-Shenghua capsule and effective medication site.MethodsThrough D-101 macroporous resin column methods,Wujia-Shenghua capsule with 60% ethanol extraction was separated for water elution part, 20% ethanol elution part, 40% ethanol elution part, 60% ethanol elution part. Then each elution part was respectively mixed into concentration of 10mg/ml,1mg/ml, 0.1mg/ml, 0.01 mg/ml and acted on the MCF-7 cell to have, MTT test and rate of PR calculation.Results Compared with the blank control group(100%), when the concentration was 0.1 mg/ml, water elution part, 20% ethanol elution part and 60% ethanol elution part(98.10%, 101.06%, 106.04%)had no effect on the proliferation of MCF-7 cells, there was not statistically significant(P>0.05), while the 40% ethanol elution part(108.22%)can promote the proliferation of MCF-7 cells,there was a statistically significant(P<0.05). When the concentration was 1 mg/ml, 20%, 40% and 60% ethanol elution part(111.72%, 122.48%, 115.35%)can distinctly promote the proliferation of MCF-7 cells, there was a significant difference between four groups(P<0.01).ConclusionThe 40% ethanol elution part ofWujia-shenghua capsule has the strongest estrogen activity on plant.

Objective To investigate the in vitro effect of interferon-γ (IFN-γ) and ATRA on the morphological transition, proliferation of and apoptosis in a human cutaneous squamous cell carcinoma cell line SCL-1. Methods Cultured SCL-1 cells were divided into 6 groups to be treated with ATRA of 1 μmol/L, various concentrations ( 100, 500, 1000 U/ml) of IFN-γ, the combination of ATRA of 1 μmol/L and IFN-y of 1000 U/ml,respectively, or to remain untreated. MTT assay and flow cytometry were performed to evaluate the cell proliferation and apoptosis. The morphological features of apoptotic cells were observed by a transmission electron microscope (TEM) and inverted phase contrast microscope after 1% propidium iodide staining. Results IFN-γ could inhibit the proliferation of SCL-1 cells in a dose-dependent manner, and the most pronounced inhibitory effect was observed at a dose of 1000 U/ml . ATRA and IFN-γ induced an apoptosis in SCL-1 cells, and the early apoptosis rate was 4.84%, 11.96% and 18.71% in SCL-1 cells after treated with ATRA of 1 μmol/L, IFN-γ of 1000 U/ml and their combination, respectively. A series of morphological changes characteristic of apoptosis,such as bipolar changes, were observed in SCL-1 cells treated with ATRA and IFN-γ, with the presence of many early apoptotic cells, which showed a trend towards benign differentiation. Conclusions Within a certain concentration range, IFN-γcan promote the differentiation, but inhibit the proliferation of SCL-1 cells in a dose-dependent manner, and ATRA could enhance the effects of IFN-γ.

Objective Through investigating the clinicians’ scientific research level and weakness,we can carry out targeted clinical research training systematically,and then strengthen their utilization of clinical resources and data,and finally promote the transformation of scientific research resuits.Methods Filling and submitting the online questionnaires through WeChat,a popular chatting tool in China,physicians from 13 affiliated hospitals of a university in Shanghai have joined this research.Results 507 valid questionnaires have been received online:In terms of clinical research capacity,there seemed to be more barriers in the following issues:comprehension of the types and applications to design a clinical trial,formulation of the details including PICO elements(Patient-Intervention-Comparison-Outcome),methodological application including the category and principle of randomization,the category and principle of blind method and how to control the confounding elements and probable bias,and statistical problems in scientific research including discrimination of the different definition between FAS(Full Analysis Set) based on the principle of ITT(Intention-To-Treat) and PPS(Per-Protocol Set),how to write a standardized SAP(Statistical Analysis Plan) and how to calculate the sample size of a trial),and various management of clinical trials including data management,follow-up management,adverse event management and so on,and writing a protocol and a CRF (Case Report Form) in a standard and professional way.As for the needs for training,the top three topics were how to design a clinical research in a standardized style,how to practice the methodology and how to utilize the statistical skills into clinical trials.Conclusions Standardized design and writing of clinical trial protocols according to the CONSORT (Consolidated Standards of Reporting Trials) and SPIRIT (Standard Protocol Items:Recommendations for Interventional Trials),and the application of epidemiological and statistical methods are still short boards that most clinicians need to improve urgently,also are what they most concerned about at the same time.So it is necessary for physicians to receive systematic clinical research training to enhance their scientific research capacity.

Objective To analyze the clinical manifestations and the criteria for the diagnosis of POEMS syndrome.Methods The clinical characteristics of 36 cases of POEMS syndrome were retrospectively reviewed and compared with the cases reported in literature.Results In addition to the typical characteristics of polyneuropathy(100%),organomegaly(92%),endocrinopathy(86%),monoclonal plasmaproliferative disorder(100%) and skin changes(86%),the patients of POEMS syndrome also have other important features including extravascular volume overload(97%),papilledema(57%) and bone lesions(25%).Furthermore,25% of POEMS syndrome patients have co-existent Castleman disease.Conclusion To make the diagnosis of POEMS syndrome,both major and minor criteria are required.The former includes polyneuropathy and monoclonal plasmaproliferative disorder and the latter includes osteosclerotic bone lesions,Castleman disease,papilledema,organomegaly,edema or serous cavity effusion,endocrinopathy and skin changes.

Objective To observe the serum levels of endothelial microparticles (EMP) and tissue factor-bearing microparticles (TF+MP) in patients with acute leukemia before and after daunorubicin-based chemotherapy. Methods From July 2012 to February 2013, 15 patients with newly diagnosed acute leukemia in Peking Union Medical College Hospital received DA (daunorubicin + cytarabine) regimen or VDCLP (vincristine + daunorubicin + cyclophosphamide + L-asparaginase + prednisone) regimen chemotherapy. There were 8 males and 7 females, and the median age of patients was 44 years old. Eleven patients were acute myeloid leukemia (M01 case, M11 case, M29 cases), and 4 were acute lymphocytic leukemia. The peripheral blood samples were taken before induction chemotherapy and after 3 days of daunorubicin. Levels of EMP and TF+MP were assessed using flow cytometry. Results The serum EMP and TF+MP levels were significantly higher after 3-day daunorubicin infusions than those before induction chemotherapy (28.94/μl vs. 10.74/μl, P= 0.001; 64.24/μl vs. 43.80/μl, P= 0.02). Conclusion Daunorubicin-based chemotherapy may cause increased numbers of EMP and TF+MP in patients with acute leukemia.

Objective: To improve the understanding of the rare clinical presentation and management of purpura fulminans (PF) in patients with paroxysmal nocturnal haemoglobinuria (PNH). Methods: A case of PF occurring in PNH is reported, while the related literature review is conducted. Results: A 49-year-old male patient suffered from one-week history of fever, greenish-brown colour urine, multiple well demarcated and painful purpura of the head and neck. He had been reported to have two thromboembolic events during the 22-year course of PNH. Skin biopsy displayed classic PF features. Laboratory testing showed a high PNH clone, intravascular hemolysis and coagulation system changes. After sufficient anticoagulation and short course of glucocorticoid therapy, the clinical conditions were improved correspondingly. During a follow-up period of 6 month, there was no recurrence of thrombosis. Conclusion PF should be considered in PNH patients with unexplained, quickly developed painful purpura. Extensive work-up should be performed to find out other potential thrombophilic risk factors after diagnosis of PF. Early diagnosis, adequate anticoagulation therapy and control hemolysis were essential to PF treatment occurring in PNH. The survival of patients and the qualities of life can be improved. The PNH clone detection is needed to evaluate the status of procoagulation and predict the risk of recurrent thrombosis.

Objective: To investigate clinic-pathological characteristics, diagnosis, treatment and prognosis of intravascular large B cell lymphoma (IVLBCL) in China. Methods: Clinical and pathological records were analyzed from 12 IVLBCL patients diagnosed between Jan 2010 to Jun 2016. Kaplan-Meier method was used to estimate overall survival (OS), and univariate analysis was performed to identify prognostic factors. Results: A series of 12 patients with IVLBCL (median age, 53.8 years; range, 32-76 years; 6 males and 6 females) was reviewed. Fever was the most common symptom (10/12), respiratory symptoms (cough, pleural effusion, dyspnea, 50%) and hemophagocytic lymphohistiocytosis (50%) were frequently observed, and only 12 patients had neurological symptom. All patients had elevated lactic dehydrogenase and serum ferritin. International Prognostic Index score was high in 75% of total patients. All patients had extra-nodal involved, pulmonary (6/12) and bone marrow (4/12) were frequently involved. Large lymphoid cells within vessel lumina or sinuses were observed in all patients. These cells were large, with scant cytoplasm, vesicular nuclei, and one or more nucleoli, and the structures of vessels and sinus were reserved. CD20 and CD79a were positive in all cases. 11patients received rituximab combined CHOP regimen chemotherapies, overall response rate (ORR) was 90.1%, and complete response rate was 66.7%. Median survival time and median progression time were not reached after a median follow-up of 20 months. Univariate analysis revealed that no clinical characters were associated with OS. Conclusion As a rare variant of DLBCL, IVLBCL presented with pulmonary involved frequently, and trans-bronchial lung biopsy had good positive rates. Rituximab contained chemotherapy was the backbone for IVLBCL.