肥大细胞白血病（mast cell leukemia，MCL）是系统性肥大细胞增生症（systemic mastocytosis，SM）中罕见的一种类型。2016年的WHO造血与淋巴组织肿瘤分类将SM分为五种亚型 [1]：惰性SM（indolent SM，ISM）、冒烟型SM（smoldering SM，SSM）、SM伴血液系统肿瘤（SM with associated hematologic neoplasm，SM-AHN）、侵袭性SM（aggressive SM，ASM）和MCL。MCL病情进展快，诊断困难，而合并嗜酸性粒细胞增多又增加了MCL诊断和治疗的难度。现报告我院新近诊断的一例MCL合并嗜酸性粒细胞增多患者并对相关文献进行复习，以提高对此罕见疾病诊疗的认识。

目的 分析浆母细胞淋巴瘤(plasmablastic lymphoma,PBL)患者的临床和病理特征、治疗及预后.方法 回顾性分析青岛大学附属医院2013年4月至2021年11月确诊并治疗的8例PBL患者的临床资料,并进行文献复习.结果 8例PBL患者均为人类免疫缺陷病毒(human immunodeficiency virus,HIV)阴性,其中男3例,女5例,中位年龄61(39～75)岁.1例为腹股沟淋巴结起病,其余7例为淋巴瘤结外器官受累起病,初诊Ann Arbor分期Ⅳ期5例,4例存在B症状(发热、盗汗、体重减轻).7例患者瘤细胞弥漫表达CD38,5例患者瘤细胞表达CD138,1例患者可见B细胞标志物CD20,EBER阳性1例.6例患者接受以CHOP方案(环磷酰胺+多柔比星+长春新碱+泼尼松)为一线化疗方案治疗,其中1例完全缓解并接受自体造血干细胞移植联合沙利度胺维持治疗,另1例完全缓解并接受原发病灶切除术,6例死亡.8例患者中位总生存时间7.5(0.8～96.1)个月.结论 HIV阴性PBL发病率低,但侵袭性高、预后不良,常规化疗反应性差;自体造血干细胞移植联合免疫调节剂维持治疗在一定程度上有助于延长患者生存期.

体外膜 肺 氧 合(extracorporeal membrane oxygenation,ECMO)是一种有效的心肺支持手段,它通过离心泵将患者的静脉血从体内引出,经过膜肺氧合后再泵入患者体内,从而保证机体重要器官的血供及氧合功能,为积极治疗原发病争取时间[1].然而应用ECMO治疗的患者病情危重且变化迅速,常为完善诊断或寻求最佳诊疗方式而需要进行转运.国外研究显示,近一半的危重患者在住院期间进行过外出检查或其他治疗,其中有超过2次转运经历的占一半,由此可见院间或院内转运是危重患者一项重要的护理工作[2].ECMO治疗期间患者在运转过程中受距离、时间和设备等多种条件制约,且转运前准备工作繁琐,如何安全、顺利地完成转运工作,成为风险管理中的重要环节.急性心肌梗死是常见的致命性胸痛疾病,具有病死率高、并发症多的特点.急诊行经皮冠状动脉介入(percutaneous coronary intervention,PCI)术能够在短时间内实现冠状动脉血运重建,改善心肌梗死患者生存质量,然而急性心肌梗死患者的病情发展迅速,常伴有心搏骤停或恶性心律失常,手术难度和危险系数较高.2019年8月28日青岛大学附属医院收治一例急性心肌梗死伴心搏骤停的患者,经过充分准备和多部门协作给予ECMO治疗后,患者病情平稳并经安全转运后行冠状动脉造影术及冠状动脉支架植入术,手术过程顺利,术中ECMO机器运转良好.患者术后第10天ECMO顺利撤机,术后第19天好转出院,现报告如下.

目的 通过对体外心肺复苏院内急救流程(以下简称"急救流程")的优化,缩短抢救时间,减少不良事件的发生.方法 2018年10月成立医疗失效模式与效应分析管理团队,应用医疗失效模式与效应分析法分析急救流程缺陷,通过文献检索、专家会议法、专家咨询法优化急救流程并于2019年1月应用于临床.临床应用急救流程2年后分析比较急救流程优化前后风险优先指数、各环节用时及不良事件发生情况.结果 急救流程优化后急救流程风险优先指数由(217.63±20.60)分降低到(102.73±39.95)分,各环节用时缩短,各类不良事件发生率均有所下降,差异均具有统计学意义(P<0.05).结论 应用医疗失效模式与效应分析法优化急救流程,可以有效改善急救延时,减少不良事件的发生.

目的 比较停跳冠状动脉旁路移植术(On-Pump CABG)与不停跳冠状动脉旁路移植术(Off-Pump CABG)对平均70岁以上患者随访1月内的临床疗效.方法 检索万方、维普、中国知网、PubMed、Web of Science、Science Direct和Cochrane Library等中英文数据库中关于Off-Pump CABG(试验组)与On-Pump CABG(对照组)临床随机对照试验,对两组患者的病死率及心肌梗死、脑卒中、肾功能衰竭4项指标进行Meta分析.结果 共有13项随机对照试验16966例患者纳入本次Meta分析.试验组患者病死率、心肌梗死、脑卒中和肾功能衰竭的发生率均明显低于对照组(P<0.05).病死率的OR值为0.76,95％CI=0.65～0.90;心肌梗塞发生率的OR值为0.77,95％CI=0.62～0.96;脑卒中的发生率的OR值为0.64,95％CI=0.45～0.89;肾功能衰竭的发生率的OR值为0.59,95％CI=0.47～0.73.结论 Off-Pump CABG对平均70岁以上患者随访1月内的疗效优于On-Pump CABG.

51岁女性患者，查体发现左心室肿物半个月。超声心动图示左心室占位性病变。2019年4月在全麻下行达芬奇机器人左心室肿物切除术。术后病理示左心室海绵状血管瘤。术后恢复良好。出院3个月复查超声心动图示肿瘤无复发。

目的:总结新冠肺炎患者的护理经验.方法:19例新冠肺炎患者的护理重点包括加强序贯氧疗、俯卧位通气等呼吸支持护理、中西医结合治疗护理、心理护理和早期康复锻炼,同时做好疫情防控工作.结果:患者病情均得到有效控制,出院13例、好转6例,未出现死亡病例.结论:在治疗及护理过程中,护理人员应发挥主观能动性,采取个体化、具体化的优质护理措施,及时更新疫情知识,熟练掌握相关理论,立足早期干预,科学、有效地做好治疗、护理及疫情防控工作.

多发性骨髓瘤(multiple myeloma,MM)是好发于中老年人的一种恶性克隆性浆细胞病,发病率占所有人类肿瘤的1％,占血液系统恶性肿瘤的13％,在过去的20年其中位生存期已从3年增长至6年[1].

达芬奇机器人技术自应用于心血管外科领域后,已经获得了长足的发展,中国心血管外科团队在其中起到了举足轻重的作用.达芬奇机器人技术在中国正逐步推广,并应用到各学科手术当中.本综述简要介绍了达芬奇机器人的诞生与组成,在心外科手术中的应用以及达芬奇手术的基本要点.

目的 比较达芬奇机器人辅助下二尖瓣手术与常规二尖瓣手术的临床效果,以明确达芬奇机器人技术的优点及不足.方法 2014年11月-2017年12月,我科共行达芬奇机器人辅助下二尖瓣手术82例(达芬奇组),同期行常规二尖瓣手术246例(常规组),将两组病人的临床治疗情况进行对比分析.结果 两组均无院内死亡.达芬奇组病人手术时间、体外循环时间、升主动脉阻断时间较常规组延长(P＜0.05);重症监护室治疗时间、拔除气管插管时间、术后住院天数较常规组缩短(P＜0.05);术中出血量、术后1个月射血分数与常规组比较差异无显著性(P＞0.05).结论 达芬奇机器人技术可安全、有效地应用于二尖瓣手术当中,并且能明显缩短病人重症监护室治疗时间、拔除气管插管时间和术后住院天数,但其手术、体外循环和升主动脉阻断时间较传统手术延长.

目的 观察多发性骨髓瘤(MM)患者血小板与淋巴细胞比值(PLR)变化,并探讨其与患者临床病理特征和预后的关系.方法 选择MM患者180例(MM组)、体检健康者90例(对照组),采集静脉血,采用XN-9000血液分析仪及其配套试剂计数血小板和淋巴细胞,计算PLR.利用受试者工作特征(ROC)曲线确定PLR诊断MM的cut off值,以其cut off值为界值将MM患者分为低PLR者和高PLR者,比较二者年龄、性别、血红蛋白、β2微球蛋白、乳酸脱氢酶、国际分期系统(ISS)分期、免疫分型等临床病理参数和生存期.结果 MM组PLR明显低于对照组(P<0.05).ROC曲线结果显示,PLR诊断MM的cut off值为117.92.180例MM患者中,低PLR者108例、高PLR者72例.与高PLR者比较,低PLR者血清白蛋白明显降低(P<0.05),二者其余临床病理参数比较P均>0.05.低PLR者中位生存期为16个月,高PLR者为24个月,二者比较P<0.05.结论 MM患者PLR明显降低,低PLR者预后更差.PLR可初步评估MM患者预后.

Objective To analyze blood pressure variability(BPV) in patients with essential hypertension from the local community health service center.Methods A total of 130 patients with essential hypertension who had visited the local community health service center(Yan'an Rd,Shibei District,Qingdao) were selected as study samples from December 2016 to June 2017.Each patient was followed up in the community health service center once a month with more than three times of follow-up in total.Systolic blood pressure coefficient of variation(CV) was selected to represent BPV and was calculated during follow-up.Comparison was conducted among patients with different mean systolic blood pressure,comorbidities,medication intake and between patients with and without complications.Results There was no statistical significance in systolic blood pressure CV among patients with different comorbidities and between patients with and without complications(P>0.05).Statistical significance could be found in systolic blood pressure CV when comparing patients with different mean systolic blood pressure and medication intake(P<0.05).Lower systolic blood pressure CV could be found in patients with mean systolic pressure<160 mm Hg comparing with those with mean systolic blood pressure ≥ 160 mm Hg(P<0.05).Patients who took medicine regularly showed lower systolic blood pressure CV than those who did not take medicine regularly(P<0.05).Conclusion Mean systolic blood pressure and medication intake have an impact towards long term BPV.Medical workers in community health service centers shall not only pay attention to the mean blood pressure of patients with essential hypertension,but also pay attention to their BPV.

Objective:To investigate the mutaome profile of the 15 common mutated genes and related clinical significance in patients with myelodysplastic syndromes (MDS).Methods:Ninety-seven patients primarily diagnosed as MDS and MDS progressed to acute myeloid leukemia (MDS/AML) were enrolled.Mutated genes were analyzed in bone marrow samples by Sanger sequencing,including the RNA splicing factor genes U2AF1,SF3B1 and SRSF2,epigenetic regulation genes ASXL1,DNMT3A,IDH1,IDH2 and TET2,signal transduction pathway genes JAK2,NRAS,KRAS,PTPN11,as well as other genes TP53,RUNX1 and SETBP1.Results:Mutations were detected in 67.98％ of the patients,23.71％ carried mutations of multiple genes,and a total of 18 combinations of different mutations were detected.U2AF1 was the most commonly mutated gene,followed by ASXL1 and TP53.ASXL1 and RUNX1 mutations are often concomitant with other gene mutations,while SF3B1 mutations usually occurred alone.RNA splicing factor genes were mutually exclusive,and they are more likely accompanied by mutation of epigenetic regulation genes.The genes mutation frequency in primarily diagnosed MDS/AML patients was significantly higher than that in MDS patients (85.71％ vs 60.52％,P=0.038).The age in mutation positive group was higher than that of mutation negative group,the frequency of gene mutation in adult group was higher than that of children and adolescent group,the differences were all statistically significant (P=0.003).Conclusion:There were certain rules in the mutaome profile of MDS and MDS/AML patients,which was related to gene function and age of patients.

目的:探讨声辐射力脉冲成像(ARFI)技术与尿β2-微球蛋白(β2-MG)检测联合诊断高尿酸肾病(HUAN)的临床价值.方法:回顾性分析和比较192例原发性高尿酸血症患者(PHUA组)、162例HUAN患者(HUAN组)和360例健康体检者(对照组)的血尿酸(UA)、血尿素氮(BUN)、血肌酐(Cr)及尿β2-MG水平、二维超声检测结果及ARFI技术检测的肾皮质、髓质和肾窦SWV值,分析HUAN患者的SWV值与临床分期的相关性,其肾实质、肾窦SWV值与尿β2-MG水平的相关性.结果:162例HUAN患者中,肾实质回声增强76例(46.91％),肾窦增宽89例(54.94％),肾皮质髓质结石49例(30.25％);192例PHUA患者和360例健康体检者肾皮质和髓质回声均无明显异常,肾窦结石分别为3例(0.83％)和5例(2.60％).HUAN组肾皮质、髓质和肾窦SWV值分别为3.32± 0.45 m/s、3.02±0.51m/s和1.58±0.45m/s;PHUA组分别为2.92±0.64m/s、2.51±0.51m/s和1.41±0.35 m/s;对照组分别为2.73±0.58 m/s、2.26± 0.43m/s和1.21± 0.21 m/s;三组SWV值均为肾皮质＞肾髓质＞肾窦(P＜0.05).HUAN组尿β2-MG水平明显高于PHUA组和对照组(P＜0.05).HUAN组尿β2-MG水平与肾实质SWV值呈线性正相关(r=0.442,P＜0.001),而与肾窦呈零相关性(r=0).Ⅰ、Ⅱ、Ⅲ期HUAN患者肾皮质SWV值比较无明显差异(P＞0.05),Ⅱ-Ⅴ期肾髓质、肾窦SWV值均高于Ⅰ期(P＜0.05);Ⅳ、Ⅴ期肾皮质SwV值高于Ⅲ期(P＜0.001).结论:HUAN患者在BUN和Cr升高之前,ARFI技术的SWV值和尿β2-MG水平均显著升高.ARFI技术联合尿β2-MG检测可作为评估肾实质组织弹性硬度及诊断早期HUAN的重要方法.

Objective To explore the clinical features and the influence of various prognostic factors on survival of patients with multiple myeloma (MM) and the survival rates of major therapeutic options were evaluated.Methods A retrospective analysis were performed on the clinical characteristics, laboratory examination and therapeutic options of 214 cases of MM patients. Nine clinical and laboratory parameters were analyzed by univariate and multivariate process. Survival analysis about major therapeutic options were performed.Results The median age of onset was 59.7 years. The most common clinical manifestations include bone pain (77.1%), anemia (70%), renal impairment (24.8%). Univariate analysis suggested thatage, percentage of plasma cell in bone marrow, hemoglobin, β2-microglobulin (β2-MG), Scr, stageⅢ were prognostic factors for overall survival (OS). Multivariate analysis suggested that age, percentage of plasma cell in bone marrow, and β2-MG were all independent prognostic factors for OS. Compared with VAD-like regimen, the patients on a BD regimen showed a superior median OS, 56 months and 39 months, respectively, survival curve was significant (P=0.028).Conclusion Clinical manifestations of MM are complicated and difficult to diagnosis. Age, highβ2-MG, high proportion of MM cells were highly related to poor prognosis of MM patients. Compared with traditional VAD regimen, application of new drug bortezomib in patients significantly extend the survival rates of patients.

目的 观察国产重组人血小板生成素(rhTPO)治疗原发免疫性血小板减少症(ITP)的临床疗效和安全性.方法 原发免疫性血小板减少症(ITP)患者皮下注射rhTPO 15 000 u/d,每天一次,疗程14 d,血小板计数大于100×109/L停药,或给药至血小板计数比给药前增高≥50×109/L,动态观察患者血常规,定期监测肝、肾功及凝血功能.结果 38例ITP患者接受rhTPO治疗后,完全反应(CR)18例,有效(R)15例,无效(NR)5例.治疗前血小板平均值为(4.97 ±2.15)×109/L,治疗后血小板计数平均值为(84.13 ±42.13)×109/L,与治疗前相比差异有统计学意义(t=11.709,P＜0.01).所有患者按照2011年中国专家共识分为两组,第一组为新诊断ITP组,第二组为持续性及慢性ITP.第一组患者21例,治疗前血小板计数平均值为(5.48 ±2.42)×109/L,治疗后血小板计数平均值为(95.76±42.57)×109/L,完全反应(CR) 14例,有效(R)4例,无效(NR)3例,总有效率85.7％,治疗有效患者血小板计数升高至30×109/L用时中位数为5(3 ～13)d,第二组患者17例,治疗前血小板计数平均值为(4.35±1.61)×109/L,治疗后血小板计数平均值为(69.76 ±37.96)×109/L,完全反应(CR)4例,有效(R)11例,无效(NR)2例,总有效率88.2％,治疗有效患者血小板计数升高至30×109/L用时中位数为3(3 ～14)d,两组有效率对比无明显差异(P＞0.05),多数患者对药物耐受良好.结论 国产rhTPO治疗新诊断和持续性及慢性的原发免疫性血小板减少症患者均具有良好的临床疗效,安全性好.

Objective To explore the clinical significance of intracranial arterial pulsatility index(PI) in patients with type 2 diabetes mellitus combined with acute lacunar infarction.Methods The middle cerebral artery(MCA) of 48 patients with type 2 diabetes mellitus combined with acute lacunar infarction(DM-L group),65 patients with type 2 diabetes mellitus without cerebral infarction(DM-N group) and 59 normal controls(normal control group) were examinated by Transcranial Doppler(TCD).The speed of blood flow and PI were record.The results were compared.Results Eighteen patients who failed with temporal window detection,mean glow velocity significantly increased and showed arteriostenosis were eliminated.DM-L group was in 7 cases,DM-N group in 5 cases and normal control group in 6 cases.PI of bilateral side in DM-L group and DM-N group were significantly higher than those in normal control group(all P < 0.001).PI of bilateral side in DM-L group was significantly higher than those in DM-N group(all P < 0.05).Conclusions The elevated PI of intracranial arteries may reflect the degree of microangiopathy in type 2 diabetes mellitus patient.PI may be a useful predictor of type 2 diabetes mellitus patients combined with acute lacunar infarction.

Objective:To assess the efficacy and adverse reaction of decitabine 4-day program treatment for myelodysplastic syndrome(MDS).Method:Twelve patients with MDS were treated with decitabine 4-day program(25to 30mg·m-2·d-1×4days)and the clinical effect and side reaction were evaluated after every course.Result:Five cases(41.67%)had complete remission,3cases(25.00%)had partial remission and the overall response rate was 66.67%.The median progress free survival was 212(13to 328)d and the median overall survival was 255(13to 963)d.The main adverse reactions were bone marrow suppression,infection and gastrointestinal symptoms.Conclusion:Compared with 3-day and 5-day programs,decitabine 4-day program can effectively and securely cure MDS patients.Moreover,it can avoid medicine waste and reduce cost.

Objective To compare the efficacy of bortezomib combined both dexamethasone and thalidomide(BDT) with vindesine+epirubicin+ dexamethasone+thalidomide(VADT) in patients with multiple myeloma(MM).Methods BDT the-rapy was used for 67 MM patients,and VADT for 37,a treatment of four courses was carried out in both groups.Changes of β2-microglobulin,immunoglobulin,and myeloma cells,before and after treatment,between the two groups were observed and compared.Results The levels of β2-microglobulin,immunoglobulin,and myeloma cells in both BDT and VADT after chemotherapy were lower than that before the treatment,the differences were statistically significant(t=2.837-7.562,P0.05).After four courses of therapy,in BDT group: complete remission(CR) 13.0%,nearly complete remission(nCR) 20.0%,partial response(PR) 53.0%,and minimal remission(MR) 6.7%,total effective rate being 93.3%;in VADT group: that were CR accounting for 3.0 %,nCR for 10.8%,PR for 40.5%,and MR for 16.2%,with a total effective rate of 70.3%.The differences between BDT and VADT were statistically significant(Hc=51.67,P0.05).Conclusion BDT is superior to VADT in the treatment of MM in terms of fast action and improvement of patients' prognosis.

目的:探讨地西他滨4d疗程方案治疗老年急性白血病的疗效及安全性。方法:应用地西他滨30mg/m2 4d疗程方案治疗4例老年急性髓系白血病,并评价其疗效和不良反应。结果:2例患者应用地西他滨4疗程后达完全缓解,1例在3疗程后获部分缓解,1例1疗程后因多器官功能衰竭死亡。4例患者治疗过程中均出现较明显的粒细胞和血小板减少。结论:地西他滨是治疗老年急性髓系白血病的有效选择,疗效较好,不良反应可以耐受。4d方案在疗效和耐受性上与3d和5d方案相比,无明显差别,应用方便。