Objective To study the clinical and laboratory features of the patients with immunorelated pancytopenia(IRP).Methods The risk factors,manifestations,blood cell counts,bone marrow phenotypes,autoantibodies and immunosuppressive therapy response of 72 patients with IRP were analyzed.Then they were followed up for(6 ~36)months,to see their long-term outcome and the prognostic factors.Results The infection,anaphylaxis and pregnancy were highly suspected to be the risk factors of IRP.Most of these patients were with pancytopenia72.2%(52/72);91.7%(66/72)of them were anemic with large or normal MCV;75.0%(54/72)of them were with leukopenia;18.1%(13/72)of them had fever.Thrombocytopenia was common,but serious bleeding rare.68.1% of these cases were with normal or decreased bone marrow cellularities and increased normoblasts.They were all found to have positive results of bone marrow mononuclear cell Coombs test,negative results of routine hemolysis tests and no evidence of malignant clonal hematopoiesis.42.9%(18/42)of these patients had C3 decreased,and 19.0%(8/42)C4 decreased.Immunosuppressive therapy was administered to 72 IRP patients.The response rate at 36 months was 87.5%(28/32).Conclusion IRP is a blood disease induced by acquired autoantibodies mediated bone marrow inhibition or destruction.It manifests pancytopenia with relative higher percentages of reticulocytes and neutrophils,and responds well to immunosuppressive therapy.

Objective To explore the clinical features of sev er e aplastic anemia (SAA) patients with chronic hepatitis B virus (HBV) infection.Methods 737 SAA cases newly diagnosed in the institute of Hemat ology and Blood Disease Hospital,CAMS in recent 13 years (1991 ~ 2003) were anal yzed.The ratio of SAA patients with chronic hepatitis B (21 cases,group Ⅰ) in them was investigated.A case-control study was undertaken to investigate the di fferences of clinical and laboratory features、therapeutic effectiveness and pro gnosis between SAA patients with chronic HBV infection \[the cases with chronic hepatitis B and those with antibodies to hepatitis virus B (23 cases,group Ⅱ )\] and SAA control group (42 cases,group Ⅲ ).Results ①The ratio of SAA patients with chronic hepatitis B in all SAA was 2.8%.②There was no significant difference of clinical features am ong them before treatment.③The recovery of PB counts、bone marrow hematopoiesis of group Ⅰ was slower than that of group Ⅱ and group Ⅲ after treatment.Th e percentage of CD8 + cells of group Ⅰor Ⅲwas significantly higher than that of group Ⅱ (P

Objective To analyze the incidence,severity and morphologic characteristics of anemia in patients with malignant tumors.Methods Five hundred and thirty hospitalized patients with a primary diagnosis of tumor were enrolled in this study at our hospital from Jan.2009 to Mar.2011.Their hemoglobin (Hb) levels,age,sex,and type of tumor were recorded.Anemia was diagnosed to be present when their Hb on admission was less than 110 g/L Results There were 111 in the 530 patients (20.94％) had anemia on admission.Anemia was seen in 35.3％ of patients with digestive system cancer,in 19.4％ with gynecologic cancers,in 18.3％ withlung cancers,in 6.8％ with urinogenital cancers and 1.8％ with breast cancers.The difference of incidence of anemia in different types of cancers was statistically significant ( x2=44.9785,P ＜ 0.01 ).Grade Ⅰ and Ⅱanemia accounted for 15.66％ ; and grade Ⅲ and Ⅳ accounted for 5.28％ of all.There was no significant difference of incidence of anemia between male and female [ 22.36％ ( 72/322 ) vs.18.75％ ( 39/208 ),x2 =1.0020,P ＞ 0.05 ].The more severe the anemia was,the smaller the erythrocyte mean corpuscular volume was.The rate of anemia was 2.08％ when patients were discharged,and 5.66％ were intervened.Conclusion The incidence of anemia was high in patients hospitalized with tumor,but only a small percent got enough medical care.

Objective To analyze the clinical features of pure red cell aplasia (PRCA),and to improve the recognition of its pathogenesis and treatment.Methods Among 35 PRCA patients from 1990-01 to 2003-04 in our hospital,17 patients(group Ⅰ) had immunologic abnormality,and the other 18 patients (group Ⅱ)were normal at every immunologic index.Removed the primary affection,all patients were given the combine treatment with drugs:androgen,immunosuppressive agent or/ and glucocorticoid.Results Nine patients in group Ⅰ appeared T subgroup ratio inversion in peripheral blood,eleven patients had higher level than normal with TNF?,and six patients' IL-2 level was higher.The response was 70.6%.Some patients shifted to normal in immunologic index.The response was 86.7% in group Ⅱ.But 11 cases relapsed of the 25 cured and remission patients.They responded again to the initial therapy.Conclusion Dysimmunity is the most important pathogenesis in PRCA patients.Most patients respond to immunosuppression therapy.The relapse patients also respond to initial therapy.

Objective To comparatively analyze effect of different cooling temperature on postoperative results of patients with Stanford type A aortic disaection underwent surgical treatment.Methods Clinical data of 65 patients with Stanford type A aortic dissection perforred aortic root shaping,ascending aorta and total aortic arch replacement combined with stented elephant trunk technique from April 2007 to March 2012 were retrospectively analyzed.According to the cooling temperature,the patients were divided into two groups:group A and group B.The cooling temperature was 20 ℃ to 24 ℃ in group A and 25 ℃ to 2.8 ℃ in group B.Extracorporeal circulation time,circulatory arrest periods,volume of postoperative drainage,ventilation time and cerebral complications incidence after operation were analyzed contrastively.Results There was significant difference between two groups about extracorporeal circulation time,circulatory arrest periods,volume of postoperative drainage,ventilationtime and transient neurological dysfunction(TND) incidence after operation.There was no significant difference between two groups in postoperative pemunent neurological dysfunction (PND),use of continuous renal replacement therapy (CRRT) and mortality.Conclusion It is safe to appropriately increase the standard of cooling temperature through operating skillfully under the circumstance of deep hypothermic circulatory arrest.It,to some extent,shortens operating time and decreases injuries on patients,time of recovery,volume of blood transfusion and complications.

Objective To summarize the clinical experience and significance of 101 cases of thoracic endovascular aortic repair(TEVAR).Methods From October 2008 to August 2013,101 patients received endovascular repair.Among the cases,81 patients were male,and 20 were female.Before operation,all patients underwent thoracoabdominal aortic CTA examination,there were 5 cases of traumatic aortic diseases,10 cases of severe aortic penetrating ulcer,86 cases of Stanford type B aortic dissection.All patients follow up with CTA 1 weeks,3 months,half a year,and annually postoperatively.Postoperative complications,efficacy,morphology and its vascular stent were observed.Results All cases received TEVAR,there were 90 cases of simple repair of aortic lumen (89.11％),11 cases of brachiocephalic vessels bypass (10.89％),2 cases of death (1.98％),1 case of type Ⅰ endoleak(0.99％),3 cases of the application of lumbar cistern drainage(2.97％),1 case of severe stress ulcer(0.99％),1 patient died after surgery with paraplegia,acute renal failure,stroke,lower limb artery embolization,dissection rupture.Conclusion Thoracic endovascular aortic repair has obvious advantage,risks and complications incidence of preoperative period is low,the technology is worth popularizing widely,but needed to pay attention to strictly control the indications of operation and ensure the brain blood supply.

Objective Analysis of myocardial microvascular perfusion in patients with chronic total coronary occlusion (CTO) who underwent a coronary artery bypass graft (CABG) use real-time myocardial contrast echocardiography (RTMCE),to provide an effective method of detecting viable myocardium and a reference for the choice of CABG indications.Methods Twenty-seven patients with CTO underwent RTMCE 1 week before CABG,they underwent follow-up echocardiography and coronary artery 256-slice multislice computed tomography aagiography 1 year after CABG.Myocardial viability was defined as a postoperative ultrasound wall motion significantly improved ≥ 1 point.Semi-quantitative analysis of contrast images,myocardial viability was defined as myocardial perfusion score ≤ 2 points.Viable myocardium by quantitative assessment of myocardial blood flow (MBF) was determined by analyses of receiver-operating characteristic (ROC) curves.Results Patients with LVEF increased significantly after CABG (P ＜ 0.01),Of 259 segments with wall motion abnormality,149 (58％) showed wall motion significantly improved ≥ 1 point after CABG,considered viable myocardium,110 (42％) were not observed in wall motion improved,considered to be non-viable.The viable myocardial segments were significantly greater than non-viable myocardial segments in A,β,A × β value (P ＜ 0.01).Compared with the semi-quantitative analysis,quantitative analysis of MBF increased the sensitivity and accuracy of RTMCE for detecting viable myocardium (P ＜ 0.05).Conclusion RTMCE could accurately assess myocardial viability and provide a strong reference for clinical decision making and judging prognosis.

Objective To analyze retrospectively the therapeutic effect and safety of minimally invasive transthoracic device closure of perimembranous ventricular septal defect (PVSD).Methods One hundred and twenty-nine patients with PVSD from January 2011 to December 2011 in our hospital were selected and a 3 to 5 cm incision was made in the inferior sternum,which performed minimally invasive transthoracic device closure of ventricular septal defect (VSD).Right ventricular free wall was punctured and guided by transthoracic echocardiography (TEE),and then the occluder was released after a delivery sheath passed through the VSD.Location of occluder,effect on heart valve,fastness and residual shunt were evaluated by TEE instantly during operation.All patients were followed up intimately after operation,echocardiogram and electrocardiogram were rechecked regularly.Results One hundred and fourteen cases were successfully occluded.Fifteen cases were performed VSD repair operation with cardiopulmonary bypass.The amounts of concentric occluder and eccentric occluder were 96 and 20,respectively.The patients performed minimally invasive transthoracic device closure of VSD had no severe complications after operation.Conclusion Although the short-term therapeutic effect of minimally invasive transthoracic device closure of PVSD is definitely and has favourable prospect of application,the long-term follow-up remains to be studied.

Objective To investigate the strategies of nutritional therapy for severe acute pancreatitis (SAP) patients.Methods Two hundred and eight patients with SAP were randomly divided into early enteral nutrition (EEN) group,late enteral nutrition (LEN) group and total enteral nutrition (TPN) group.EEN group received enteral nutrition through nasojejunal tube feeding within 72 hours in the course of disease,LEN group received the same treatment after 6 d in the course of disease,TPN group received total enteral nutrition.The index of nutrition,incidence of complication were recorded,efficacy and safety were analyzed.Results EEN group included 67 cases,while 72 cases in LEN group and 69 cases in TPN group.The incidence rate of malnutrition after 14 d of treatment in EEN group and LEN group was significantly lower than that in TPN group [59.7％ (40/67),58.3％ (42/72) vs.84.1％ (58/69),P ＜0.05],but the difference between EEN group and LEN group was not statistically significant (P ＞ 0.05).There was no statistically significant difference in the incidence of hyperlipidemia and hyperglycemia among three groups (P ＞ 0.05).The expression level of albumin,prealbumin and retinol-binding protein after 14 d of treatment in three groups compared with those before treatment and the differences were statistically significant (P ＜ 0.05).The expression level of albumin,prealbumin and retinol-binding protein after 14 d of treatment in EEN group and LEN group compared with those in TPN group and the differences were statistically significant (P＜ 0.05),there was no statistically significant difference between EEN group and LEN group (P＞ 0.05).The incidence rate of total infections,abdominal infections,bloodstream infections,secondary superinfections in TPN group were significantly lower than those in EEN group and LEN group [62.3％(43/69) vs.25.4％ (17/67),19.4％(14/72) ;21.7％(15/69) vs.4.5％(3/67),6.9％ (5/72);39.1％(27/69) vs.14.9％(10/67),11.1％(8/72) ;33.3％ (23/69) vs.9.0％ (6/67),9.7％(7/72),P ＜ 0.05],there was no statistically significant difference in the incidence of peripancreatic infections,lung infections among three groups (P ＞ 0.05).There was no statistically significant difference in the incidence of total infections,different infections between EEN group and LEN group.There was no statistically significant difference in the incidence of noninfections among three groups (P ＞ 0.05).The rate of recovery in EEN group and LEN group was significanby higher than that in TPN group [91.0％ (61/67),94.4％ (68/72) vs.81.2％ (56/69),P ＜ 0.05],but the difference between EEN group and LEN group was not statistically significant (P ＞ 0.05).Conclusion Early enteral nutrition therapy for SAP patients is safe and effective,and could significantly improve the prognosis of patients.

Objective To analyze the clinical,imaging and pathological features of cortical vein thrombosis (CoVT). Methods Ten patients with CoVT (4 patients with CoVT alone and 6 patients with CoVT and venous sinus thrombosis)treated at Navy General Hospital from January 2006 to Jun 2013 were collected retrospectively.Its etiology,imaging,and pathological features of brain tissue in 3 patients were analyzed. Results Of the 10 patients with CoVT,3 were female and 7 were male.Their mean age was 31 ± 15 years old.(1)Brain CT scan and MRI showed hemorrhagic cerebral infarction,and contrast-enhanced MRI showed scattered heterogeneous enhancement within lesions. DSA could find CoVT at different parts.(2)3 patients underwent brain biopsy because they were initially diagnosed as brain tumor. Pathological examination showed glial cell,endothelial proliferation and phagocytic reaction.Scattered spotty bleeding was observed,and significant expansion of small veins,A few of them had blood stasis and thrombosis. Conclusion CoVT is one kind of intracranial venous thrombosis. It is more common occurred in young and middle aged adults,and most of them were venous sinus thrombosis.It is caused by retrograde thrombosis and spread to cortical veins.CoVT is easily to be misdiagnosed as brain tumor.Combination of clinical and imaging findings is needed for accurate diagnosis.