血浆置换辅助治疗重症肌无力的临床疗效分析

目的评价血浆置换辅助治疗重症肌无力患者的临床效果。方法应用CS-3000PLUS全自动血细胞分离机对肌无力危象、为胸腺摘除术准备以及撤呼吸机困难患者进行血浆置换,以"706"代血浆和新鲜冰冻血浆作为置换液,每次置换血浆量为2 000 ml。结果 103名患者中每例进行PE 2～18次,平均(4.27±1.98)次,除1名肌无力危象患者因突发心肌梗塞死亡外,其余患者经过血浆置换单独或辅助治疗后均有显效或者好转,基本达到治疗目的。其中63名患者在93次血浆置换过程中出现不同程度副作用,包括口周麻木、四肢麻木、荨麻疹、寒战和发热等,经对症处理后均好转。结论血浆置换辅助治疗MG患者的肌无力危象、为胸腺摘除术准备以及撤呼吸机困难的1种有效治疗手段。     

双重滤过血浆置换与血浆置换治疗重症自身免疫性疾病的比较研究

目的比较双重滤过血浆置换(DFPP)和血浆置换(PE)治疗重症自身免疫性疾病(SAID)的差异。方法 27例SAID患者分为两组,分别采用DFPP和PE进行治疗,对疗效、并发症、费用、住院时间、实验室指标等进行比较。结果 PE组平均治疗2.2次、住院时间42.5d、治疗费用3.95万元,DFPP组平均治疗2.88次、住院时间41.94d、治疗费用4.99万元。PE组显效50%,好转30%,无效20%;DFPP组显效52.94%,好转35.29%,无效11.76%;两组疗效相当。PE组并发症发生率45.45%,DFPP组并发症发生率8.16%,DFPP组并发症明显少于PE组(P0.05)。PE后白细胞、血红蛋白、血小板、血钾、血肌酐、尿素氮、谷丙转氨酶、白蛋白、球蛋白、免疫球蛋白A(IgA)、IgG、IgM、C3没有差异(P均0.05),只有血钠略有上升(P0.05)。DFPP后白细胞、血红蛋白明显升高,白蛋白、球蛋白、IgA、IgG、IgM、C3明显降低(P均0.05),血小板、血钾、血钠、血肌酐、尿素氮、谷丙转氨酶变化不大(P均0.05)。结论 DFPP在治疗SAID中的作用与PE相当,并发症和风险较小,治疗更有保障。     

膜型双重血浆滤过治疗重症肌无力危象患者的护理

目的:探讨膜型双重血浆滤过(DFPP)治疗重症肌无力危象患者时的护理方法.方法:对16例重症肌无力危象患者进行DFPP治疗67次,护理措施包括做好治疗前的准备；治疗过程中做好血管通路护理,正确使用抗凝剂,严格无菌操作；对患者进行健康指导.结果:痊愈4例,好转7例,显效2例,无效1例,中途放弃治疗2例,总有效率81.25％.结论:DFPP治疗可以显著缓解重症肌无力危象患者的临床症状,规范专业的护理措施可保证患者的安全.     

膜型双重血浆滤过治疗重症肌无力的临床观察与护理

[目的]探讨膜型双重血浆滤过(DFPP)治疗重症肌无力(MG)病人的临床效果及护理.[方法]将39例病人随机分为观察组19例和对照组20例.分别采取DFPP治疗和综合治疗,观察两组病人乙酰胆碱受体抗体(AchR-Ab)下降程度、临床相对评分和绝对评分、开始缓解时间及住院时间等.[结果]两组病人AchR-Ab下降程度、临床相对评分和绝对评分、开始缓解时间及住院时间比较,差异有统计学意义(P＜0.05或P＜0.01).[结论]在综合治疗的基础上行DFPP治疗,可明显改善病人临床相对评分和绝对评分、缩短开始缓解时间及住院时间.     

双重滤过血浆置换治疗难治性类风湿关节炎的疗效观察

目的 探讨双重滤过血浆置换(DFPP)治疗难治性类风湿关节炎(RA)的临床疗效.方法 经两或三种改善病情的抗风湿药(DMARDs)规范治疗3～6月以上、病情不能缓解的活动性RA患者82例,随机分为DFPP组42例和非DFPP组40例.均给予柳氮磺胺吡啶+甲氨堞呤治疗;DFPP组在此治疗的基础上予以DFPP治疗.随访12～24个月.DFPP治疗结束后次日及随访结束时进行评估,通过疾病活动指标及类风湿因子、功能评估(HAQ)及美国风湿病学会推荐RA病情改善ACR标准判断临床疗效.结果 ①DFPP组ACR20、ACRS0和ACR70分别为100%、92.9%和81.0%,明显高于非DFPP组(17.5%、0、0,P<0.001),并于随访1～24个月后保持不变.②DFPP组功能评估明显改善,非DFPP组无变化,两组相比差异有统计学意义(P<0.001).③DFPP组疾病活动指标、类风湿因子明显下降,变化显著高于非DFPP组(P<0.001).结论 DFPP可迅速控制难治性RA患者的病情,明显改善功能,提高生活质量,临床疗效显著.     

连续式血浆置换抢救重症肌无力危象1例

重症肌无力（Myasthnia Gravis，MG）是一种自身免疫性疾病，患者血清中抗乙酰胆碱受体抗体（AchRAb）明显升高；封闭或破坏神经肌肉接头突触后膜上的乙酰胆碱受体，影响信息传递，从而导致肌无力。部分病人病情发展迅速凶猛。若不及时采取积极抢救措施常常会危及生命。连续式血浆置换治疗术是将病人体内的血浆通过一定的方法分离出来，同时输入正常人血浆、血浆代用品或白蛋白，     

双重滤过血浆置换治疗高脂血症的护理

总结了25例高脂血症患者应用双重滤过血浆置换（DFPP）治疗的护理措施：包括在治疗过程中,加强病情观察、心理护理,掌握技术操作要点、积极预防并发症;在治疗后做好饮食指导和健康教育一认为双重滤过血浆置换治疗高脂血症安全、有效,精心的护理是治疗成功不可或缺的重要条件。     

血浆置换法治疗重症肌无力患者的护理

采用血浆置换法治疗重症肌无力，在缓解肌无力症状方面，效果良好。笔者报告了９例重症肌无力患者７２次血浆置换治疗过程中的操作方法、术后常见并发症的防治措施以及出院指导。     

血浆置换法治疗重症肌无力患者的护理

采用血浆置换法治疗重症肌无力，在缓解肌无力症状方面，效果良好。笔者报告了９例重症肌无力患者７２次血浆置换治疗过程中的操作方法、术后常见并发症的防治措施以及出院指导。     

Prethymectomy plasmapheresis in myasthenia gravis

Plasma exchange before thymectomy may decrease the time on mechanical ventilation (MV) and shorten the stay in the intensive care unit (ICU) for patients with myasthenia gravis (MG). This study evaluated the effects of prethymectomy plasmapheresis. A total of 29 myasthenic patients, 18 women and 11 men aged 20-73 years, were treated with double filtration plasmapheresis (DFP) for two to five consecutive sessions over a period between 2 and 21 days (mean 8.1 days) before transsternal thymectomy. Acetylcholine receptor antibody (AchRAb) titers, vital capacity (VC), maximal inspiratory pressure (Pimax), and MG score were measured before and after the course of DFP. Three outcome measures including duration of postoperative hospital stay, duration of ICU stay, and duration of MV were analyzed for correlation with clinical variables. The duration of MV ranged from 6 to 93 h, with a median of 21 h. The median ICU stay was one day and the median postoperative hospital stay was 10 days. A higher removal rate of AchRAb was associated with a shorter duration of ICU and postoperative hospital stay (P = 0.001 and 0.019, respectively). Postoperative hospital stay was strongly correlated with post-DFP Pimax (P = 0.010), and marginally correlated with pre-DFP VC (P = 0.047) and to a lesser extent with pre-DFP Pimax (P = 0.063). Univariate analysis using the log rank test revealed that removal rate of AchRAb <30% (P = 0.043) and pre-DFP Pimax <-60 cmH2O (P = 0.024) were significantly associated with prolonged ICU stay. Risk factors for prolonged postoperative stay included post-DFP Pimax <-60 cmH2O (P = 0.017), pre-DFP Pimax <-60 cmH2O (P = 0.031), and post-DFP VC < 1.0 L (P = 0.046). Our results confirmed the efficacy and safety of DFP in prethymectomy preparation for myasthenic patients.     

Fremanezumab plus plasmapheresis in a patient with chronic migraine and myasthenia gravis: Case report of an effective treatment

A patient with chronic migraine and generalized myasthenia gravis was concurrently treated with fremanezumab and with therapeutic plasmapheresis (PEX). Fremanezumab was dosed right after a PEX session, or in the midpoint between sessions, and the efficacy of both treatments was maintained. This case broadens the drug's clinical applications and it helps in choosing the appropriate medical regimen in patients requiring both treatments.     

Antisynthetase syndrome with refractory lung involvement and myositis successfully treated with double filtration plasmapheresis

Antisynthetase syndrome (ASS) is characterized by inflammatory muscle disease, pulmonary and joint involvement, and antisynthetase autoantibodies, with anti‐Jo‐1 antibody being the most common. Despite the use of immunosuppressive drugs, the prognosis of lung involvement seems poor. Herein, we report a case of refractory ASS, which maintained long‐term remission by double filtration plasmapheresis (DFPP) combined with immunosuppressive therapy. For a 65‐year‐old woman, who was diagnosed with ASS, immunosuppressive therapy was initiated and plasmapheresis (PP) was performed five times due to acute interstitial pulmonary disease and inflammatory myopathy. She remained in remission for eight months following PP. Increase in interstitial involvement was identified by lung tomography when the patient presented again with complaint of progressive increase in dyspnea and muscle pain. Although the immunosuppressive therapy was increased for the patient with elevated creatine phosphokinase (CPK) (2776 IU/mL), a rapid decrease in diffusion capacity of the lung for carbon monoxide (DLCO) was observed and the patient underwent PP. After four sessions of therapy, insufficient clinical and laboratory response was obtained (control CPK 1797 IU/mL) and because of that issue DFPP using a 2A filter was performed to the patient. There was a marked improvement in complaints of the patient, DLCO, and laboratory findings (control CPK 508 IU/mL) after three sessions of DFPP. The patient, who continued the immunosuppressive therapy after DFPP procedure, is being followed for 12 months in remission. Although our experience is limited with only one patient, DFPP seems promising as a treatment option for ASS with severe lung involvement. J. Clin. Apheresis, 28:422–425, 2013. © 2013 Wiley Periodicals, Inc.     

The efficacy of double filtration plasmapheresis in the treatment of homozygous familial hypercholesterolemia: A single-center experience

Introduction

Different modalities of low-density lipoprotein (LDL) apheresis are used to treat patients with familial hypercholesterolemia (FH). The aim of this study was to describe the efficacy of the Double Filtration Plasmapheresis (DFPP) method for FH cases in our hands and to compare with other LDL apheresis techniques.

二重滤过血浆置换治疗难治性高脂血症

目的　了解单次二重滤过血浆置换治疗非家族性、难治性高脂血症的短期疗效。方法　 2 0例非家族性高脂血症患者接受二重滤过血浆置换疗法 ,其中 14例患者治疗前血浆总胆固醇 (TC)大于6 .8mmol/L或甘油三脂 (TG)大于 2mmol/L ,低密度脂蛋白 (LDL)水平均高于 3.1mmol/L。取治疗前和治疗结束时的血样 ,分别测量血浆TC、TG、LDL、Apo(A)、Apo(B)、Lp(a)、HDL和纤维蛋白原水平。　结果11例患者具有完整的记录 ,其平均年龄 (4 5 .2 5± 8.33) (2 6～ 5 9)岁 ;平均病程为 6 .7年。单次治疗总置换量为 (4 183± 6 6 7) (30 0 0～ 5 0 0 0 )ml,治疗前后TC、TG、LDL、Apo(A)、Apo(B)、Lp(a)、HDL和纤维蛋白原水平分别为 (6 .86± 1.17)mmol/Lvs (3.4 2± 0 .6 3)mmol/L、(3.92± 2 .13)mmol/Lvs (3.5 7± 1.82 )mmol/L、(4 .0 3± 0 .86 )mmol/Lvs (1.13± 0 .6 6 )mmol/L、(1.34± 0 .37)g/Lvs (0 .85± 0 .30 )g/L、(1.34± 0 .5 7)g/Lvs (0 .70± 0 .34)g/L、(176 .14± 75 .5 3)mg/Lvs (98.4 3± 4 4 .0 8)mg/L、(1.0 3± 0 .2 8)mmol/Lvs (0 .6 8±0 .2 2 )mmol/L、(2 6 3.6± 86 .4 9)mg/dlvs (15 4 .88± 4 4 .12 )mg/dl。TC平均下降 5 1% ;LDL下降约 72 % ;Apo(a)和Apo(b)分别下降 32 %和 4 4 %左右。Lp(a     

双重血浆滤过治疗脂蛋白肾病的护理

目的探讨双重血浆滤过(DFPP)治疗脂蛋白肾病(LPG)的临床护理要点。方法选择LPG患者12例行DFPP治疗,共治疗53例次,并对患者实施以保证治疗安全、提高治疗效果为目的整体护理。结果所有患者均顺利完成治疗,经治疗后免疫学指标均明显下降,无1例发生低血压、导管感染、溶血、过敏等并发症。结论 DFPP治疗LPG效果显著,优质的专科护理技术是保证治疗安全有效完成的关键。     

蛋白A免疫吸附治疗重症肌无力的临床观察

目的探讨蛋白A免疫吸附治疗重症肌无力（MG）的临床疗效。方法采用病例对照方法,将24例MG患者非随机分为2组。治疗组12例,对照组12例,治疗组在传统治疗方法的基础上,给予蛋白A免疫吸附治疗,每周3次,共4周。对照组不进行蛋白A吸附治疗。治疗前和治疗后抽取清晨空腹血,测IgG、IgA、IgM、补体C3、C4和采用酶联免疫吸附法测乙酰胆碱受体抗剂（AChR-Ab）,并根据疗效标准临床相对评分法进行评分。结果治疗组总有效率为91.67%（11/12）,对照组总有效率为75%（9/12）,差异有统计学意义（P〈0.05）。治疗组免疫球蛋白IgG、IgA、IgM和AchR-Ab下降程度较对照组明显降低（P〈0.01）。结论蛋白A免疫吸附能有效降低MG血液中AchR-Ab和免疫球蛋白水平,比单纯药物治疗疗效好,是治疗MG的有效方法。     

Rh (D) alloimmunization treated by double filtration plasmapheresis

Here in this report a 31?year old pregnant woman with positive serum antiglobulin test against anti-D antierythrocyte antibodies who was treated succesfully with double filtration plasmapheresis (DFPP) is presented. The DFPP was started in the early stage of pregnancy together with intravenous immunoglobulin therapy and the antierythrocyte antibody titer of the patient was successfully maintained in a stable level below 1:64 dilution. She delivered successfully on the 30th week of gestation. The favorable outcome of this patient implies that DFPP is an effective and safe treatment modality in pregnant women with red cell alloimmunization.     

Long-term treatment of myasthenia gravis with immunoadsorption

Acute treatment of myasthenic crisis with immunoadsorption (IA) or plasma exchange is well established. The efficiency of chronic apheresis therapy in myasthenia gravis (MG), however, and its efficacy in reducing concomitant potentially harmful immunosuppressive therapy, is unknown. We treated 13 patients with therapy-resistant MG or severe steroid or azathioprine therapy-related side effects, or both, with long-term IA [median, 38 (range: 16-59) months]. IA was performed every second day until partial remission was achieved (modified Osserman score <2). Subsequently, oral immunosuppressive therapy was reduced and the frequency of IA adapted to the clinical symptoms. After initiation of IA the mean (SEM) Osserman score decreased from 3.23 +/- 0.12 to 1.23 +/- 0.08 within 1 month (P < 0.01). Mean azathioprine dose was reduced concomitantly from 89 +/- 9.4 mg/day to 56 +/- 11 mg/day (P < 0.05), and mean prednisolone dose from 41 +/- 7.6 mg/day to 22 +/- 8.5 mg/day (P < 0.05). After 36 months the number of IA-sessions/month had been reduced from 4.81 +/- 0.24 to 2.64 +/- 0.4 (P < 0.05), the mean azathioprine dose to 25 +/- 17 mg/day and the mean prednisolone dose to 9 +/- 3.6 mg/day. Six out of thirteen patients were weaned from IA after a median of 33 (range, 16-50) months and a decrease of the Osserman score to 0.33 +/- 0.33. In these patients MG remained stable during a follow-up period of 28 (range, 16-38) months. We conclude that long-term IA enables the reduction of oral immunosuppressants in patients with contraindications or resistance to steroid and azathioprine therapy. Furthermore, almost 50% of the patients can be weaned from IA with then substantial lower need of further immunosuppressive therapy.