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.     

双重滤过血浆置换治疗难治性重症肌无力的研究

目的探讨多次双重滤过血浆置换治疗难治性重症肌无力的方法、临床疗效和应用价值。方法23例难治性重症肌无力患者全部采用双重滤过血浆置换法治疗4次,3～4d置换1次,置换液为20％白蛋白50ml加706代血浆1000ml。治疗前及每次置换完成后第3天抽空腹血查乙酰胆碱受体抗体（AchR—Ab）,并根据临床绝对评分法评分和相对评分法判断临床疗效。结果痊愈1例,基本痊愈2例,显效9例,好转8例,无效3例,总有效率为86．96％。结论多次双重滤过血浆置换能有效降低血液中较高的AchR—Ab水平,对治疗难治性重症肌无力具有重要的临床价值。     

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.     

Clearance of fibrinogen and von Willebrand factor in serial double-filtration plasmapheresis

Double filtration plasmapheresis (DFP) is a widely used and effective way to clear autoantibodies from plasma. It can, however, transiently alter the hemostatic system and cause a bleeding tendency in some patients. There is limited data on the consecutive effect of serial DFP on the hemostatic system, especially on fibrinogen and von Willebrand factor (vWF) levels. This study measured fibrinogen and vWF serially before and after each session of DFP in 8 patients who received one course of DFP treatment for 3 to 5 consecutive sessions on an alternate-day basis. In each session of DFP, the clearance rate of fibrinogen and vWF exceeded 63 and 45%, respectively. The final levels of fibrinogen and vWF after a full course of DFP were reduced to 14.3 and 51.2% of baseline level, respectively. No bleeding tendency was observed in any of the 34 DFP sessions. In conclusion, although an obvious decrease in fibrinogen level and the modest decrease in vWF were observed after an intensive course of DFP treatment, the low incidence of clinically important bleeding confirms the hemostasis-related safety of DFP.     

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。     

Double-filtration plasmapheresis in the treatment of leg ulcers in cryoglobulinemia

Hepatitis C virus (HCV) is the major cause of cryoglobulinemia. Skin lesions are frequent and can be cured from the removal of cryoglobulins by therapeutic apheresis. We describe a case of HCV-positive type I cryoglobulinemia with severe leg ulcers, not responsive to antiviral and immunosuppressive treatment. Thirty sessions of double filtration plasmapheresis were performed, over a period of 6 months, with no other associated treatment. Before and after each session an assessment of immunoglobulins, complement, cryocrit, and fibrinogen was made. HCV RNA levels were determined in serum cryoprecipitate, supernatant before and after each session, and in the collection bag. No differences in pre and postapheresis values were observed in the serum concentrations and the supernatant, whereas the postapheresis cryoprecipitate showed a significantly reduced viral load (P < 0.02) as compared with the preapheresis values. There was improvement in the condition of ulcers in the leg during apheresis and had completely regressed by the end of the cycle.     

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.     

Successful treatment of severe rhesus D‐incompatible pregnancy with repeated double‐filtration plasmapheresis

Fetal anemia is caused by Rhesus (RhD) sensitization as a result of RhD incompatibility during pregnancy. The severe form of this disease can cause hydrops fetalis leading to intrauterine death. We experienced a highly sensitized 39‐year‐old woman with B Rh‐negative blood. She had a history of three induced abortions and experienced perinatal death associated with hydrops fetalis. During the pregnancy prior to her most recent one, she was treated with double‐filtration plasmapheresis (DFPP), high dose γ‐globulin and intrauterine fetal blood transfusion (IUT). For her most recent pregnancy, we performed only weekly or fortnightly DFPP from 13 weeks until delivery. Anti‐D antibody titer was maintained between 32 and 256 without any signs of fetal anemia. IUT was not required at any stage of the pregnancy. No adverse events were observed. She successfully delivered a healthy male infant weighing 2,289 g by Cesarean section at 35 weeks. Repeated DFPP may be an effective and safe strategy to reduce antibody titers in highly sensitized women with RhD‐incompatible pregnancy, avoiding the need for IUT. J. Clin. Apheresis 30:305–307, 2015. © 2014 Wiley Periodicals, Inc.     

双重血浆置换治疗类风湿性关节炎活动期的作用观察

目的探讨双重血浆置换（DFPP）对类风湿性关节炎（RA）活动期患者类风湿因子（RF）、C反应蛋白（CRP）和血沉（ESR）的影响，评价DFPP对RA的作用。方法RA活动期患者在规范药物治疗的基础上予以DFPP治疗2～3次，比较单次DFPP前后血RF、CRP和ESR的变化，并观察疾病的活动性与置换出血浆颜色的关系。结果DFPP后RF、CRP和ESR下降率分别为22．55％、57．08％和50．48％，均明显低于置换前（P均〈0．001）。活动期RA患者置换出的血浆呈墨绿色、绿色或黄绿色；墨绿色混浊，黄绿色清亮；墨绿色者疾病活动指标（关节压痛数、关节压痛指数、关节肿胀数、关节肿胀指数、CRP、ESR），均明显高于绿色和黄绿色患者（P均〈0．001）。结论DFPP能明显降低RA活动期患者的RF、CRP、ESR；患者置换出的血浆呈墨绿色、绿色或黄绿色；颜色深浅与疾病活动性相关，疾病活动性越高，颜色越深且越混浊。     

A comparison of long-term post-thymectomy outcome of anti-AChR-positive,anti-AChR-negative and anti-MuSK-positive patients with non-thymomatous myasthenia gravis

Objective: A single-centre, non-randomized, non-controlled study was designed to compare the long-term post-thymectomy clinical outcome in anti-AChR-positive, anti-AChR-negative and anti-MuSK-positive patients with non-thymomatous myasthenia gravis (MG). Methods: A total of 331 consecutive patients with seropositive MG, 55 with seronegative MG and 10 with anti-MuSK-positive MG underwent extended transsternal thymectomy (T-3b according to Myasthenia Gravis Foundation of America). The primary endpoint was to assess differences in the rate of complete stable remission (CSR) in patients with and without anti-AChR and anti-MuSK antibodies. Results: The mean follow-up was 218.3 (SD 128.1) months in the seropositive MG group, 149.8 (SD 131.1) in the seronegative group and 169.9 (SD 116) in the anti-MuSK-positive group. In the seropositive MG group, the probability of obtaining CSR at 5 years post-thymectomy was 51.1% for the seropositive group compared with 40 for the seronegative group (p = 0.05) and 20 for the anti-MuSK-positive group (p = 0.03). Differences between the seronegative and anti-MuSK-positive groups were not observed. The estimated median follow-up to obtain a CSR was 17.8 months (95% confidence interval [CI] 15.7 – 19.8 months) in seropositive MG patients, 22.1 (95% CI 16.7 – 27.4 months) in seronegative MG patients and 20.6 (95% CI 13.3 – 27.9 months) in anti-MuSK-positive MG patients (long-rank test, p = 0.07). Conclusions: Long-term post- thymectomy clinical outcome was better in patients with conventional anti-AChR antibodies than in those with seronegative disease. In seronegative anti-MuSK-positive MG, thymectomy seems to be less effective than in anti-MuSK-negative MG but this study cannot answer the question of whether thymectomy should be undertaken in anti-MuSK-positive patients.     

Clinical evaluation of a staphylococcal protein A immunoadsorption system in the treatment of myasthenia gravis patients

BACKGROUND: The role of plasma exchange is well established in the management of myasthenia gravis, an autoimmune disorder characterized by muscle weakness and caused by circulating IgG antibodies with specificity against the acetylcholine receptor. Plasma antibody removal by conventional means, however, is nonselective and uses replacement fluids (chiefly, albumin solution) derived from human plasma. STUDY DESIGN AND METHODS: The Canadian Apheresis Group undertook a study at two Canadian apheresis centers to clinically evaluate a staphylococcal protein A immunoadsorption system (EXCORIM) in myasthenia gravis patients. RESULTS: The immunoadsorption system was safe and well tolerated. Ten of 12 patients had improvement in their neurologic status, as measured by a 20-point scoring system. The mean improvement in the weakness score was significant for the group (p = 0.0013). CONCLUSION: Patients with myasthenia gravis respond to treatment with plasma immunoadsorption. Further studies are required for a cost-benefit analysis.     

Pancytopenia with severe thrombocytopenia in a patient treated with twice‐weekly LDL‐apheresis by polyacrylate adsorption from whole blood

Pancytopenia with severe thrombocytopenia occurred in a patient treated with low‐density lipoprotein (LDL)‐apheresis by polyacrylate adsorption from whole blood, after treatment frequency had been increased from once to twice a week. Cell counts recovered with discontinuation of LDL‐apheresis, but thrombocytopenia recurred after resumption of twice‐weekly treatments. Thrombocyte counts remained stable following the replacement of polyacrylate adsorption from whole blood by double‐filtration plasmapheresis. The complications' close coincidence with twice‐weekly polyacrylate adsorption from whole blood suggests a causal relationship, although by a still unknown mechanism. Monitoring of thrombocytes should be advised in patients treated with LDL‐apheresis by polyacrylate adsorption from whole blood. J. Clin. Apheresis 2010. © 2010 Wiley‐Liss, Inc.     

Lung function in patients with prior poliomyelitis

Summary. Lung volumes and spirometric flows, maximal respiratory pressures, lung diffusion capacity and blood gases were evaluated in 20 patients with prior poliomyelitis and compared with data from an age- and sex-matched control group. In the polio patients, static lung volumes and pulmonary flow capacity were significantly reduced to approximately 65% of the values of the control group. However, expiratory and inspiratory flows were normal in relation to lung volumes, while maximal respiratory pressures were significantly reduced in the polio patients. Static lung volumes and forced flows were correlated with the maximal respiratory pressures both in the polio patients and in the controls. However, when the muscle force in the polio patients decreased below a certain level, it became more important as a determinant of lung-flow capacity. The polio patients had a significantly increased Pco₂ but a normal oxygen saturation and Pco₂ was inversely correlated with the maximal respiratory pressures in the patients but not in the controls. The lung diffusion capacity was reduced in the polio patients compared to the controls, but less markedly than vital and total lung capacity. The findings indicate a restrictive respiratory dysfunction and alveolar hypoventilation most probably due to weakness of the respiratory muscles.     

重症肌无力患者白细胞介素6水平的测定

目的　探讨白细胞介素 6 (IL 6 )在重症肌无力 (MG)发病机制中的作用。方法　采用双抗体夹心ELISA法对 18例MG患者用糖皮质激素 (GC)治疗前、治疗 3个月后和 2 0例正常对照血清IL 6、乙酰胆碱受体抗体 (AchRab)水平进行检测。结果　MG患者组血清IL 6水平显著高于对照组 (P <0 .0 1) ,MG患者组血清IL 6水平在用GC治疗 3个月后显著降低 (P <0 .0 1) ,其血清IL 6与血清AchRab水平呈正相关 (r =0 .6 89,P <0 .0 1)。结论　IL 6与MG发病密切相关 ,IL 6参与了MG的免疫病理过程 ;检测血清IL 6水平对MG临床有重要价值 ;GC可抑制IL 6合成及Ach Rab的产生。     

聚甲基丙烯酸甲酯骨水泥充填治疗骨损伤后的肺功能变化

背景：目前聚甲基丙烯酸甲酯骨水泥充填在治疗骨折以及人工关节置换骨损伤中的应用已很普遍，对于其引起的肺功能变化，尤其是老年骨损伤患者，应积极防治。目的：观察骨损伤应用聚甲基丙烯酸甲酯骨水泥充填治疗后患者的肺功能变化。方法：选取2010年5月至2011年6月大庆油田总医院骨科收治的骨折以及人工关节置换骨损伤患者24例，应用聚甲基丙烯酸甲酯骨水泥充填治疗，比较治疗后1d以及1周的肺活量、用力肺活量、最大通气量变化。结果与结论：所有应用聚甲基丙烯酸甲酯骨水泥治疗骨折以及人工关节置换的骨损伤患者，在治疗后1d的肺活量（2．45±0．56）L、用力肺活量（2．20±0．39）L、最大通气量（50．9±23．9）L，均明显低于治疗后1周。说明应用聚甲基丙烯酸甲酯骨水泥可以在一定程度上影响患者的肺功能，这种改变可能与骨水泥的使用导致肺组织的微血管栓塞有关，需要加强监测以及通过在治疗前后应用药物来预防和治疗肺栓塞。