Search

Introduction: AD is a progressive neurodegenerative disorder causing significant cognitive decline and impaired daily functioning. Current treatments offer only modest relief, and many amyloid-targeting therapies have failed, prompting exploration of alternative approaches such as PE with albumin replacement. Objectives: This scoping review systematically maps the literature on PE with albumin replacement in AD management, focusing on outcomes, methodologies, and reported benefits and risks. Methods: A comprehensive search in PubMed, supplemented by reference scanning and hand-searching, identified studies involving PE with albumin replacement in AD patients. Data charting and critical appraisal were conducted using standardized tools. Results: Seven primary studies from the AMBAR (Alzheimer Management by Albumin Replacement) trial met the inclusion criteria, consistently reporting improvements in cognitive function, positive neuroimaging results, and favorable neuropsychiatric outcomes. For instance, one study found a significant slowing of cognitive decline (p < 0.05) among patients receiving PE with albumin replacement. Another study showed better preservation of hippocampal volume and improved brain perfusion metrics in the treatment group (p < 0.05). The intervention was generally well-tolerated with manageable side effects. Conclusion: PE with albumin replacement is a promising therapeutic approach for AD, warranting further investigation to confirm its efficacy and safety across broader settings. [ABSTRACT FROM AUTHOR]

Summary: A young adult African American female presented with normocytic microangiopathic haemolytic anaemia, elevated lactate dehydrogenase and thrombocytopenia. The patient responded to therapeutic plasma exchanges (TPE) for presumed thrombotic microangiopathy caused by thrombotic thrombocytopenic purpura (TTP). After relapsing, the patient was found to have pancytopenia, megaloblastic bone marrow and low vitamin B12 consistent with pernicious anaemia, which improved with intramuscular B12 and discontinuation of TPE. B12‐deficient macrocytosis was not seen at presentation due to concomitant alpha‐thalassaemia. Initial clinical/laboratory improvement is attributed to B12 present in TPE plasma. B12 deficiency can mimic TTP. Vigilance is needed regarding atypical presentations of pernicious anaemia. [ABSTRACT FROM AUTHOR]

Sepsis and septic shock are frequent and severe clinical pictures in intensive care medicine that result from a dysregulated immune response to an infection and cause a high mortality rate. This article provides an overview of the various extracorporeal procedures used to treat sepsis. Various procedures are used to treat sepsis and septic shock. These include high-volume hemofiltration (HVHF), very high-volume hemofiltration (VHVHF), high cut-off filter (HCO), polymyxin B hemoperfusion and cytokine adsorption filters. The HVHF and VHVHF remove inflammatory mediators but show no significant benefit in terms of stabilization and survival in sepsis patients. The HCO filters effectively eliminate cytokines but so far there is no evidence of a survival benefit. Polymyxin B hemoperfusion shows promising results in initial studies in certain patient groups, while evidence for cytokine adsorption filters is limited. Combined plasma filtration and adsorption (CPFA) and therapeutic plasma exchange (TPE) have so far shown promising results in small studies. Although CPFA shows no survival benefit, TPE may have protective effects on the vascular glycocalyx. Extracorporeal procedures carry risks such as thrombosis and loss of proteins and clotting factors. The therapeutic benefit of these procedures in the treatment of sepsis remains unclear and further prospective randomized multicenter studies are needed to evaluate their efficacy and safety. There are currently no guideline recommendations for the routine use of these procedures in sepsis. [ABSTRACT FROM AUTHOR]

To report use trends of plasma exchange (PLEX) as well as sociodemographic and medical comorbidities associated with PLEX in the United States. Retrospective cross-sectional study. Adult patients (≥ 18 years) admitted for inpatient hospitalization with a primary diagnosis of optic neuritis (ON). Data from the National Inpatient Sample database was compiled to assess PLEX use rates between 2000 and 2020. The cohorts of patients receiving PLEX versus not receiving PLEX were analyzed between quarter 4 of 2015 through 2020 (International Classification of Diseases, Tenth Revision [ICD-10], only) for patient sociodemographic variables, medical diagnoses, insurance types, hospital characteristics, cause of disease, time to therapy, length of stay (LOS), and total charges incurred. Incidence of ON, incidence of PLEX, demographics, diagnoses associated with PLEX therapy, total charges, and LOS. From 2000 through 2020, 11 209 patients hospitalized with a primary diagnosis of ON were identified, with a significant majority managed at urban teaching hospitals. Use of PLEX increased steadily over 2 decades from 0.63% to 5.46%. Use was greatest in the western United States and least in the eastern United States. In the subset of ICD-10 cases, 3215 patients were identified. The median time to therapy of PLEX was 1 day after admission, and PLEX use was highest in patients with neuromyelitis optica spectrum disorder (NMOSD) (21.21%) and lowest in multiple sclerosis-associated ON (3.80%). Use of PLEX was associated with significantly longer LOS and higher total charges incurred. Medical comorbidities associated with PLEX included adverse reaction to glucocorticoids (adjusted odds ratio [aOR], 31.50), hemiplegia (aOR, 28.48), neuralgia (aOR, 4.81), optic atrophy (aOR, 3.74), paralytic strabismus (aOR, 2.36), and psoriasis (aOR, 1.76). Over the last 2 decades in the United States, PLEX therapy for ON has increased, with the highest use in the western United States and for patients with the diagnosis NMOSD ON. The author(s) have no proprietary or commercial interest in any materials discussed in this article. [ABSTRACT FROM AUTHOR]

Copyright of Zeitschrift für Rheumatologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction/Aims Methods Results Discussion Randomized controlled trials show that repeat intravenous immunoglobulin (IVIG) dosing and plasma exchange (PLEX) followed by IVIG (combination therapy) have no additional therapeutic benefit in Guillain‐Barre Syndrome (GBS) non‐responders. Furthermore, the delineation between GBS and Acute Onset CIDP (A‐CIDP) can be particularly challenging and carries therapeutic implications. We aimed to evaluate the presence of repeat IVIG, combination therapy, and diagnostic reclassification from GBS to CIDP.We performed a retrospective study of a large healthcare database for patients with GBS in the US from 2001 to 2018. We identified individuals initially diagnosed with GBS and later re‐classified as CIDP. Multivariable logistic regression models were developed to determine associations between patient factors and repeat IVIG dosing, combination therapy, and diagnostic re‐classification from GBS to CIDP.We identified 2325 patients with GBS. A total of 39.7% received repeat IVIG and 6.1% received combination therapy. The proportion of individuals initially diagnosed with GBS and then re‐classified as CIDP was 32.0%. Repeat IVIG, combination therapy, and diagnostic reclassification remained stable over time. Female sex (OR 0.79, 95% CI 0.65–0.96) and medium‐high net worth (OR 0.64, 95% CI 0.45–0.90) associated with repeat IVIG therapy, while Asian ethnicity associated with diagnostic re‐classification from GBS to CIDP (OR 1.77, 95% CI 1.09–2.86).Repeat IVIG dosing was quite common in GBS before newer trials suggesting harm in non‐responders, and IVIG/PLEX combination therapy continues to persist despite strong evidence against use in non‐responders. Further, nearly one in three patients initially diagnosed with GBS is subsequently diagnosed with CIDP, but the reasons are unclear. [ABSTRACT FROM AUTHOR]

Objective To analyze the clinical outcomes of a patient with thrombotic thrombocytopenic purpura(TTP) as the starting performance who was considered as systemic lupus erythematosus(SLE) under multiple abnormal autoantibodies, and summarize the treatment experience through the clinical outcomes of plasma exchange combined with high-does steroid pulse therapy. Methods A middle-aged female patient presented with initial symptoms of fever, bleeding and hemolytic thrombocytopenia. Laboratory tests showed decreased activity of thrombospondin type 1 motif no.13(ADAMT13) and abnormal autoantibodies. The diagnosis was considered SLE with TTP, and the patient underwent continuous plasma exchange combined with high-dose steroid pulse therapy. The patient's platelet recovery was dynamically observed. Results The patient responded well to plasma exchange combined with high-does steroid pulse therapy, with platelet count raising to normal in a short period of time and hormone gradually decreasing. There was no recurrence during the follow-up period. Conclusion TTP combined with lupus is not common, but when both occur simultaneously, plasma exchange combined with high-does steroid pulse therapy may effectively alleviate symptoms. This treatment approach can serve as a valuable reference for similar clinical cases.

Yanping Xiang, Renhua Li, Jia Cai, Qian Jiang Department of Infectious Diseases, The First Affiliated Hospital of Chongqing Medical University, Chongqing, People’s Republic of ChinaCorrespondence: Renhua Li, Department of Infectious Diseases, The First Affiliated Hospital of Chongqing Medical University, No. 1 Youyi Road, Yuanjiagang, Yuzhong District, Chongqing, 400016, People’s Republic of China, Email 854561075@qq.comBackground: This study aimed to investigate clinical efficacy, safety and short-term prognosis of plasma exchange (PE), plasma perfusion combining PE (PP+PE), dual-plasma molecular adsorption system combining PE (DPMAS+PE) in treating acute-on-chronic liver failure (ACLF).Methods: Two hundred and fourteen ACLF patients admitted to our hospital were included and divided into PE (n = 72), PP+PE (n = 75), DPMAS+PE group (n = 67). Laboratory indexes and MELD scores were collected, and clinical efficacy was compared. Patients’ adverse reactions during and 24-h after treatment were collected, and safety was compared. Survival status of patients was followed-up within 90 days, and prognosis was analyzed.Results: PE, PP+PE and DPMAS+PE significantly reduce TBiL, DBiL, ALT, AST, SA, PT, INR, PCT and CRP levels, and increase PA and PTA levels, compared with pre-treatments (P < 0.05). WBC and SCR levels in DPMAS+PE group decreased significantly post-treatment (P < 0.05). Na+ and Cl− levels in PE and PP+PE group decreased significantly post-treatment (P < 0.05). Total adverse reaction incidence in PE, PP+PE, DPMAS+PE group were 38.89%, 22.70%, 17.90%, respectively, with significant differences among three groups (P < 0.05). Ninety-day mortality rates of patients in PE, PP+PE, DPMAS+PE group were 41.67%, 34.67%, 20.90%, respectively, with significant differences among three groups (P < 0.05).Conclusion: PE, PP+PE and DPMAS+PE three artificial liver treatment modes can effectively improve liver, kidney and coagulation function of ACLF patients. DPMAS+PE demonstrated better ability to remove endotoxin and inflammatory mediators, showed advantages in reducing ACLF patient mortality within 90 days, and had the least impact on electrolyte post-treatment. Therefore, DPMAS+PE can be used as a better choice for clinical treatment.Keywords: artificial liver treatment mode, acute-on-chronic liver failure, plasma exchange, plasma perfusion, double plasma molecular adsorption system

Abstract Background Thrombotic thrombocytopenic purpura (TTP) is a rare hematological disorder. The occurrence of TTP subsequent to an emergent aortic valve replacement after a TAVR procedure is exceedingly uncommon with only a few reported cases worldwide. Case Presentation We report the case of a 70-year-old female patient diagnosed with aortic insufficiency. Following a transcatheter aortic valve replacement, she underwent emergency aortic valve replacement under cardiopulmonary bypass on the subsequent day due to heart valve displacement. The postoperative diagnosis revealed TTP and symptomatic treatment involving plasma exchange was administered. After demonstrating steady improvement, the patient was eventually discharged. Conclusion Aortic valve replacement after TAVR is a high-risk procedure and increases susceptibility for developing secondary TTP. The diagnosis and treatment of secondary TPP is considerably challenging, and early diagnosis with symptomatic treatment including plasma exchange can increase patient survival.

Autoimmune hepatitis (AIH) is a rare, immune-mediated inflammatory liver disorder, while its acute form has a spectrum of clinical manifestations including acute-icteric AIH, acute severe AIH (AS-AIH) and AS-AIH with acute liver failure (ALF) (1). A subset of patients with dermatomyositis (DM) presents with characteristic skin findings but without muscle involvement, termed clinically amyopathic dermatomyositis (CADM). This subgroup includes patients with amyopathic DM(AMD), who are devoid of all clinical and laboratory findings of muscle involvement (2). We report a case of a previously healthy 54-year-old woman who presented with acute icteric AIH and features of AMD, rapidly progressing to AS-AIH with ALF and the diagnostic and therapeutic challenges encountered while managing the patient.

Abstract Atypical haemolytic uremic syndrome (aHUS) is a rare disorder characterised by complement-mediated thrombotic microangiopathy (TMA). Despite clinical guidelines, the diagnosis and treatment of aHUS in its early stages remains challenging. This study examined the annual trends in aHUS clinical practices in Japan and explored factors influencing early diagnosis and treatment. Using data from the 2011–2020 Diagnosis Procedure Combination database, 3096 cases with the HUS disease code were identified, of which 217 were confirmed as aHUS and treated with eculizumab or plasma exchange. Early initiation, defined as starting eculizumab or plasma exchange within 7 days of admission, was the focus of the study. Our study revealed no significant changes over time in the number of aHUS diagnoses, cases treated with eculizumab, or early initiation cases. Early initiation cases underwent haemodialysis earlier and had ADAMTS13 activity measured earlier, shorter hospital stays, and lower hospitalisation costs than late initiation cases. In conclusion, we found no increase in the number of newly diagnosed aHUS cases or early treatment initiation over time. Early recognition of TMA and differentiation of the causative disease are crucial for identifying potential aHUS cases, which may lead to better patient prognoses.

Background: Therapeutic plasma exchange (TPE) is a highly effective rescue treatment for patients with acute exacerbation of neuroimmunological disease that removes circulating autoantibodies and inflammatory components from the bloodstream. The aims of this study are to explore the safety and the effectiveness of TPE in patients with autoimmune neurological disorders. Methods: We retrospectively evaluated the frequency of adverse events (AEs) and the effectiveness of TPE using the modified Ranking Scale (mRS) in patients with acute neurological flares who underwent TPE at the University Hospital of Palermo. Results: Of 59 patients, the majority underwent TPE due to multiple sclerosis (MS) relapse. In 23.7% of cases, TPE was performed before obtaining a definite diagnosis due to the severity of the clinical presentation. After TPE, the mRS score was globally reduced (p < 0.0001), and this effect was marked in patients with MS, Guillain–Barré syndrome, and myasthenia gravis crisis but not in those with paraneoplastic syndromes. Circulating pathogenetic antibodies, younger age, and the early use of TPE were factors strongly associated with TPE effectiveness. The overall safety profile of TPE was satisfactory with an AE frequency of 15%. Conclusions: These results highlight the early use of TPE in patients with circulating pathogenetic antibodies as well as its favorable safety profile.

Objective To compare the efficacy of double filtration plasmapheresis(DFPP), centrifugal therapeutic plasma exchange(cTPE) and centrifugation-filtration plasmapheresis(CFPP) in improving renal insufficiency after kidney transplantation, as well as the differences in inducing plasma exchange-related adverse reactions. Methods Clinical data from 46 patients who underwent plasma exchange after renal transplantation in our hospital were retrospectively collected, and patiens were divided into DFPP group(n=33), cTPE group(n=7) and CFPP group(n=6). Changes in peripheral blood creatinine, albumin, hemoglobin, platelets, fibrinogen levels and urine volume before and after TPE were compared and analyzed among the three groups. Results Among the DFPP group, cTPE group and CFPP group, the creatinine after TPE decreased by (31.40±25.38)%, (58.91±19.75)% and (39.44±28.64)%, respectively, with cTPE group significantly higher than the DFPP group(P0.05); the urine volume after TPE increased by (49.33±30.03)%, (54.62±39.32)% and (68.89±23.00)%, showing no significant differences(P>0.05); the hemoglobin after TPE decreased by (11.97±5.94)%, (20.17±5.75)% and (9.65±8.75)%, respectively, with the cTPE group significantly higher than the DFPP group and CFPP group(P0.05). The platelet count after TPE decreased by (37.88±18.39)%, (24.56±12.36)% and (21.40±12.51)%, respectively, with no significant differences between the three groups(P>0.05); the fibrinogen after TPE decreased by (0.57±0.20)%, (0.14±0.06)% and (0.26±0.22)%, respectively, with the DFPP group significantly higher than the cTPE group(P0.05); the albumin after TPE decreased by (11.41±5.97)%, (14.67±6.52)% and (25.18±5.10)%, respectively, with cTPE group and DFPP group significantly lower than the CFPP group(P0.05). Conclusion The effect of three plasma exchange methods varies on renal function, anemia and coagulation function of patients after kidney transplantation. It is necessary to consider the the patient’s disease characteristics and treatment needs, as well as the laboratory′s technical conditions and plasma supply when selecting TPE methods.

The use of posttransplant cyclophosphamide has revolutionized the field of haploidentical hematopoietic stem cell transplant. The impact has been especially enhanced in low- and middle-income countries. Unrelated donor pool in developing nations is often limited due to the lack of large unrelated donor registries. Donor-specific antibodies (DSAs) are preformed immunoglobulin G anti-human leukocyte antigen (HLA) antibodies against HLA antigens that are not shared with the donor. We present successful desensitization in a 13-year-old patient with class III β-thalassemia with very high MFI DSAs with flow crossmatch positivity, persisting after pretransplant immunosuppression.

Introduction. Myasthenia gravis (MG) or Erb-Goldflam syndrome is an autoimmune neuromuscular disease based on autoaggression of humoral immunity against elements of neuromuscular synapses and intracellular structures of muscle fibers (antibodies to acetylcholine receptors (AchR), muscle-specific tyrosine kinase (MuSK), protein 4 related to low-density lipoprotein receptors (LRP4), ryanodine receptors (RyR), titin, and skeletal muscle antigens). The exact causes of autoimmune processes in MG are unknown, but abnormalities of the thymus gland (hyperplasia and neoplasia), especially in patients with antibodies to AchR, as well as genetic predisposition are of indisputable importance [1]. Aim. To analyze the researches of pathogenesis, diagnosis and treatment for patients with severe MG from latest literature sources to optimize the treatment of this group of patients. To analyze the own experience of implementing a patient-oriented approach to the treatment and rehabilitation of MG. Materials and methods. Bibliosemantic, comparative and system analysis methods, and clinical and neurological examination of patients with MG. Results. The course of MG is variable and can be represented by episodic muscle weakness, stationary flow, slow or rapid progression [2]. Currently, the complex therapy of patients with MG has a clear tendency to move from symptomatic (acetylcholinesterase inhibitors, potassium preparations, potassium-sparing diuretics) treatment to pathogenetic, mainly etiopathogenetic concepts of therapy are being formed (immunosuppressive therapy, antigen-specific immunotherapy, monoclonal antibodies), and it is also being clarified effectiveness of early thymectomy [3]. Conclusions. Complex therapy of patients with MG is based on the use of a differential diagnostic algorithm for various pathogenetic variants of MG, which forms a mainly etiopathogenetic concept of therapy. Antigen-specific immunotherapy, aimed at restoring tolerance to the attacked autoantigen by targeting only the damaged part of the immune system, while leaving the rest intact, is considered more promising for the treatment of MG. MG treatment should be carried out in the conditions of a specialized neurological center, where a full examination and targeted pathogenetic therapy can be carried out.

Background: Therapeutic apheresis (TA) is already used to treat various diseases in the field of nephrology. The aim of this study was to evaluate the frequency and types of complications that occur during TA in children with kidney disease. Methods: Records of children (≤ 18 years) who underwent TA between 2007 and 2022 were retrospectively reviewed. Children with missing data and those with a diagnosis of nonnephrological disease were excluded. Results: A total of 1214 TA sessions, including 1147 therapeutic plasma exchange (TPE) sessions and 67 immunoadsorption (IA) sessions, were performed on the 108 patients enrolled in the study. Forty-seven percent of the patients were male, and the mean age was 12.22 ± 4.47 years. Posttransplant antibody-mediated rejection (64.8%) and hemolytic uremic syndrome (14.8%) were the most common diagnoses indicating TA. Overall, 17 different complications occurred in 58 sessions (4.8%), and 53 sessions (4.6%) were not completed because of these complications. The distribution of complications among the patients was as follows: 41.4% had technical complications, 25.9% had allergic complications, and 32.7% had others. The most common technical complication was insufficient flow (37.5%). The incidence of complications was greater in patients aged 3–6 years than in patients in the other age groups (p = 0.031). The primary disease, type of vascular access, and rate of fresh frozen plasma/albumin use were similar between patients with and without complications (p values of 0.359 and 0.125 and 0.118, respectively). Conclusions: Our study showed that complications occurred in only 4.8% of TA sessions. The most common complication was technical problems. [ABSTRACT FROM AUTHOR]

Background: Therapeutic plasma exchange (TPE) is a highly effective rescue treatment for patients with acute exacerbation of neuroimmunological disease that removes circulating autoantibodies and inflammatory components from the bloodstream. The aims of this study are to explore the safety and the effectiveness of TPE in patients with autoimmune neurological disorders. Methods: We retrospectively evaluated the frequency of adverse events (AEs) and the effectiveness of TPE using the modified Ranking Scale (mRS) in patients with acute neurological flares who underwent TPE at the University Hospital of Palermo. Results: Of 59 patients, the majority underwent TPE due to multiple sclerosis (MS) relapse. In 23.7% of cases, TPE was performed before obtaining a definite diagnosis due to the severity of the clinical presentation. After TPE, the mRS score was globally reduced (p < 0.0001), and this effect was marked in patients with MS, Guillain–Barré syndrome, and myasthenia gravis crisis but not in those with paraneoplastic syndromes. Circulating pathogenetic antibodies, younger age, and the early use of TPE were factors strongly associated with TPE effectiveness. The overall safety profile of TPE was satisfactory with an AE frequency of 15%. Conclusions: These results highlight the early use of TPE in patients with circulating pathogenetic antibodies as well as its favorable safety profile. [ABSTRACT FROM AUTHOR]

Background and purpose: The predictive value of serum neurofilament light chain (sNfL) and serum glial fibrillary acidic protein (sGFAP) for apheresis outcome in steroid‐refractory multiple sclerosis (MS) relapse has not yet been evaluated. Methods: We used pre‐ and postapheresis serum samples from 38 participants of the IAPEMS trial (clinicaltrials.gov: NCT02671682), which investigated the use of immunoadsorption versus plasma exchange for the treatment of steroid‐refractory MS attacks. Response to apheresis was classified based on improvement on (i) the Expanded Disability Status Scale (EDSS), (ii) the affected functional system scores (FSS) of the EDSS, or (iii) the visual acuity for patients with optic neuritis, 4 weeks postapheresis. sNFL and sGFAP were measured by single molecule arrays. Results: Preprocedural sGFAP levels could discriminate between responders and nonresponders, determined by FSS improvement (p = 0.017). In multivariate logistic regression analysis, younger age (odds ratio [OR] = 0.781, 95% confidence interval [CI] = 0.635–0.962, p = 0.020) and lower sGFAP levels (OR = 0.948, 95% CI = 0.903–0.995, p = 0.031) could predict response to apheresis in the overall cohort. We could observe a trend towards a favourable apheresis outcome with higher sNfL levels (OR = 1.413, 95% CI = 0.965–2.069, p = 0.076). Analysis of the sNfL‐to‐sGFAP ratio showed an OR of 1.924 (95% CI = 1.073–3.451, p = 0.028) for predicting apheresis response. The ratio showed a better predictive value than the individual parameters. Neither biomarker was affected by the number of steroid cycles preapheresis. Conclusions: Lower sGFAP levels, a higher sNfL‐to‐sGFAP ratio, and younger age are associated with a favourable apheresis outcome. [ABSTRACT FROM AUTHOR]

Introduction: Apheresis practices in the United States (US) have not been comprehensively characterized to date. This study aimed to address this gap by evaluating apheresis therapy through a national survey. Methods: A multi‐institutional survey was conducted between April and July 2023. The survey, comprising 54 questions, focused on institutional demographics, procedures, equipment, staffing, training, and impacts of the Coronavirus Disease 2019 (COVID‐19) pandemic. Responses from 22 institutions, primarily academic medical centers, were analyzed. Results: Therapeutic plasma exchange (TPE) was the most common procedure, followed by hematopoietic progenitor cell collection (HPC‐A) and red blood cell exchange (RCE). CAR‐T cell collections were widespread, with some institutions supporting over 30 protocols concurrently. Most sites used the Spectra Optia Apheresis System, were managed by a transfusion medicine service, and employed internal apheresis providers. Insufficient staffing levels, exacerbated by the COVID‐19 pandemic, were common and most often addressed using overtime. Discussion: The survey highlighted the ubiquity of TPE, expanding cellular collections and staffing challenges. The role of apheresis in supporting cellular therapy, particularly in newly developing cell and gene therapies and clinical trials, was evident. Staffing issues during the pandemic emphasized the need for innovative recruitment strategies. Conclusion: This nationwide survey provides the most comprehensive analysis to date of apheresis practices in large US academic centers. [ABSTRACT FROM AUTHOR]

This meta‐analysis aims to evaluate the effectiveness of the double plasma molecular adsorption system (DPMAS) in combination with plasma exchange (PE) compared to plasma exchange alone in the treatment of Acute‐on‐Chronic liver failure (LF) caused by hepatitis B. Until August 31, 2023, a comprehensive search of databases including Embase, Chinese Medical Journal Full‐text Database, China Biomedical Literature Database, Wan Fang Medical Network, PubMed, and the Cochrane Library was carried out using keywords like "liver failure," "acute‐on‐chronic liver failure," "PE," "DPMAS," and related terms. The quality of the included studies was evaluated using QUADS (quality assessment of diagnostic accuracy studies). Software Revman 5.3 was used to examine the data, while Stata 15.1 was used to run Egger's test. Following thorough screening, 452 patients who received PE alone and 429 patients who received DPMAS in addition to PE were included. Every study that was included was of a high caliber. When comparing the DPMAS plus PE group to the PE alone group, the total bilirubin reduction was considerably higher (mean difference [MD] = −49.09, 95% confidence interval [CI]: −54.84 to −43.35, p <.00001). Prothrombin activity (PTA; MD = −1.53, 95% CI: −3.29 to −0.22, p =.09), albumin (ALB; MD = −0.58, 95% CI: −1.57 to 0.41, p =.25), prothrombin time (PT; MD = −0.07, 95% CI: −1.47 to 1.34, p =.92), and platelet count (PLT; MD = −0.08, 95% CI: −1.33 to 1.66, p =.90) did not differ significantly. The improvement in international standardized ratio (INR) was significantly greater in the PE group (MD = 0.07, 95% CI (0.03, 0.10), p =.0001). When combined with DPMAS, PE has been shown to be more effective in lowering total bilirubin levels. PE can also lower INR in individuals who have hepatitis B‐related ACLF. This therapeutic strategy also lessens the need for plasma transfusions. [ABSTRACT FROM AUTHOR]

Introduction: Serpiginous choroiditis presents with large yellow‐white exudative lesions that occur near the optic nerve papillae, that progresses slowly with repeated relapses and cures. Although infection and autoimmunity have been implicated, the cause is unknown. Methods: A man was diagnosed with serpiginous choroiditis on clinical and other examinations. He started treatment with oral corticosteroids, cyclophosphamide, adalimumab, azathioprine, rituximab, and mycophenolate mofetil. Only the steroids and cyclophosphamide had a therapeutic effect. Plasma exchange was initiated, and the lesions quickly resolved. Results: Disease control has been maintained by plasma exchange and cyclophosphamide during flare‐ups in the fall and winter, suggesting that plasma exchange is effective in the treatment of serpiginous choroiditis. Conclusion: The reproducible response with each recurrence suggests a strong association between the disease and autoimmunity. Furthermore, that some, as yet unknown, autoantibodies are involved in the pathogenesis of serpiginous choroiditis. [ABSTRACT FROM AUTHOR]

This article discusses two cases of patients with severe forms of lupus and lupus-related complications. In the first case, a teenage female with neuropsychiatric lupus and lupus nephritis experienced bacterial pneumonia and cytomegalovirus antigenemia while being treated with rituximab. Plasma exchange (PE) was used before rituximab to manage the patient's condition, and the combination of PE and rituximab effectively controlled the lupus activity. The second case involves a patient with neuropsychiatric systemic lupus erythematosus (NPSLE) who was treated with apheresis therapy, specifically plasma exchange, to remove autoantibodies and cytokines. The PE treatment successfully bridged to rituximab therapy, although it was delayed due to infections. The document also mentions a potential link between systemic lupus erythematosus and HPV vaccination, but further research is needed. Overall, apheresis therapy, specifically plasma exchange, was found to be effective in these refractory cases of lupus. [Extracted from the article]

Background: Plasma exchanges (PEX) and immunosuppression are the cornerstone of management of anti-factor H (FH) antibody-associated atypical hemolytic uremic syndrome (aHUS), particularly if access to eculizumab is limited. The duration of therapy with PEX for anti-FH aHUS is empirical. Methods: We compared the efficacy of abbreviated PEX protocol (10–12 sessions) in a prospective cohort of patients diagnosed with anti-FH aHUS (2020–2022), to standard PEX protocol (20–22 sessions) in a historical cohort (2016–2019; n = 65). Efficacy was defined as 70% decline in anti-FH titers or fall to ≤ 1300 AU/ml at 4 weeks. Patients in both cohorts received similar immunosuppression with oral prednisolone, IV cyclophosphamide (5 doses) and mycophenolate mofetil. Outcomes included efficacy, rates of hematological remission and adverse kidney outcomes at 1, 3 and 6 months. Results: Of 23 patients, 8.2 ± 2.1 years old enrolled prospectively, two were excluded for significant protocol deviation. PEX was abbreviated in 18/21 (86%) patients to 11.5 ± 3.3 sessions. Abbreviation failed for lack of hematological remission by day 14 (n = 2) and persistent neurological manifestations (n = 1). All patients in whom PEX was abbreviated achieved > 70% reduction in anti-FH titers at day 28. The percentage fall in anti-FH titers was similar for the abbreviated vs. standard PEX protocols at 1, 3 and 6 months. At last follow-up, at median 50 months and 25 months for standard and abbreviated cohorts, the estimated GFR was similar at 104.8 ± 29.1 vs. 93.7 ± 53.4, respectively (P = 0.42). Conclusion: Abbreviation of the duration of PEX is feasible and efficacious in reducing anti-FH titers. Short-term outcomes were comparable in patients managed by abbreviated and standard PEX protocols. [ABSTRACT FROM AUTHOR]

Thyroid storm is an endocrine emergency, and treatment must ensure primary goals, including reducing the production and release of thyroid hormones, mitigating the effects of thyroid hormones, increasing the elimination of thyroid hormones, treating systemic disturbances, and managing triggering factors. However, in a few cases where thyroid storm does not respond to initial treatment, therapeutic plasma exchange (TPE) should be considered. A 50-year-old male patient was admitted to the University Medical Center Ho Chi Minh City due to hypotonia and sensory disturbances gradually spreading from the lower extremities to the entire body. The patient was diagnosed with Guillain-Barré syndrome (GBS) and newly discovered hyperthyroidism. During the treatment course, the patient developed hospital-acquired pneumonia, acting as a trigger factor for a thyroid storm. Despite aggressive treatment for thyroid storm, the patient's condition worsened, leading to the decision to perform TPE. The replacement fluid was a combination of fresh frozen plasma (FFP) and albumin 5%. Subsequently, the patient returned to a euthyroid state and was discharged. Combining FFP and albumin 5% in TPE advantages FFP's high thyroid hormones-binding capacity and albumin's cost-effectiveness, safety, and efficiency. This reduces the drawbacks associated with high volumes of FFP and offers a balanced and effective approach to managing thyroid storms. Moreover, the concurrent presence of GBS and thyroid storm is extremely rare. Through this case, we aim to discuss the role of TPE in the treatment of thyroid storms and the effectiveness of the combination of FFP and albumin 5% as the replacement fluid. [ABSTRACT FROM AUTHOR]

Background: Mirror syndrome is a rare disease characterized by "triple edema", while Hemolytic Uremic Syndrome (PHUS) is a serious disease that occurs within a short period of time after the end of pregnancy, with a low prevalence and poor prognosis, and it is even rarer for both to occur in the same patient. Methods: We report a case of mirror syndrome combined with PHUS and analyze the clinical data to improve the understanding of the disease. Results: The patient presented clinically with "triple edema" and was diagnosed with mirror image syndrome. After cesarean section, the patient developed cardiac insufficiency, renal insufficiency, hemolysis, and other symptoms and was diagnosed as PHUS. After active treatment, the maternal prognosis was good. Conclusions: Mirror syndrome and PHUS are both clinically rare diseases with poor long-term prognosis if not diagnosed and treated in a timely manner; therefore, awareness of the diseases, early and accurate diagnosis and timely and correct treatment should be improved. [ABSTRACT FROM AUTHOR]

Background: Mean corpuscular hemoglobin concentration (MCHC) is one of the parameters detected by blood cell analyzers, often used together with mean corpuscular volume (MCV) and mean corpuscular hemoglobin content (MCH) as diagnostic indicators for anemia classification. It has important clinical value in early detection of the cause of anemia and the underlying etiology of anemia. Therefore, the accuracy of MCHC results is of great significance for the diagnosis and treatment of diseases. Methods: We reported two cases of false elevation of MCHC. Considering the possibility of cold agglutination and lipid blood interference detection, we used 37℃ water bath and plasma exchange to correct for interference on the sample. Results: After correcting the interference, MCHC returned to normal, consistent with the patient's disease status. Therefore, the two cases of abnormal elevation of MCHC are considered to be pseudo elevation caused by interference. Conclusions: For specimens with abnormally elevated MCHC levels, experimenters should first analyze possible interfering factors and choose effective methods to correct different interferences, providing accurate testing reports for doctors and patients. [ABSTRACT FROM AUTHOR]

Neutralizing capacity measurement (NCM) of soluble ABH substances (SAS) in plasma was assessed to guide the selection of the appropriate ABO group of fresh-frozen plasma (FFP) for plasma exchange (PE) in blood group O recipients with ABO-incompatible transplantations. Neutralizing capacity was assessed by measuring anti-A and/or anti-B titers in samples comprising one unit of O FFP and 10 O EDTA plasma samples and subtracting the binary logarithm of the titer in each group with a saline dilution. Ten EDTA plasma samples with Lewis b (Leb) antigen positivity and 10 sets of pooled FFP from each blood group were used as diluents. In O FFP, the NCM values (mean±SD) were 3.4±0.52 (2.6±0.52) and 2.6±0.52 (1.5±0.3) in B and AB for IgM (total antibody) anti-B (both P <0.001), and in the 10 O EDTA plasma samples, they were 3.9±0.88 (3.1±0.88) and 3.2±0.79 (2.4±0.97) for IgM (P =0.0013) and total anti-B (P =0.025), respectively. In vitro analysis revealed that B FFP is more effective than AB FFP in reducing IgM and total anti-B antibody titers in O recipients, regardless of Leb antigen positivity. [ABSTRACT FROM AUTHOR]

Copyright of Lijecnicki Vjesnik is the property of Croatian Medical Association and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Appropriate treatment reduces the severity and duration of relapses in demyelinating diseases of Central Nervous System (CNS). If high-dose corticosteroids treatment fails, therapeutic plasma exchange (TPE) is considered as a rescue treatment. Objectives: This study aimed to investigate early clinical response and complications of TPE and prognostic factors in CNS demyelinating relapses. Design: This prospective observational study was designed in a tertiary center during one year. Methods: All adult patients diagnosed corticosteroid-resistant Multiple Sclerosis (MS), NeuroMyelitis Optica Spectrum Disorder (NMOSD), idiotypic Transverse Myelitis or Clinical Isolated Syndrome relapses, were eligible. Clinical response is defined based on Expanded Disability Status Scale (EDSS) at discharge. Clinical and laboratory complications recorded. Results: Seventy-two patients were analyzed which 58.3% patients were female. MS was diagnosed for 61.1% of cases. Thirty-five patients (48.6%) responded and the mean differences of EDSS significantly decreased 0.60 score (CI95%:0.44-.77). Electrolyte imbalances and thrombocytopenia occurred in 80.6% and 55.6% of cases respectively and 40.3% of patients had systemic reactions. However, 26.4% patients experienced moderate to severe complications. In patients with moderate to severe disability, responders were younger (MD: 8.42 years, CI95%: 1.67-15.17) and had lower EDSS score at admission (median:6, IQR: 5.5-6 against 7.5 IQR: 6.5-8). The risk of failure was higher in active progressive MS patients compared with RRMS patients (OR: 6.06, CI 95%:1.37-26.76). Patients with thrombocytopenia were hospitalized more than others (MD: 1.5 days, CI 95%: 0-3). Females were more prone to hypokalemia and systemic reactions (OR: 3.11, CI 95%:1.17-8.24 and OR: 6.67, CI 95%:2.14-20.81 respectively). Conclusion: The most common indication of TPE was corticosteroid-resistant severe MS relapses. About half of the patients presented an early clinical response. Lower disability, younger age and RRMS diagnosis are prognostic factors of better response. One out of four patients experienced moderate to severe complications, mainly electrolyte imbalances and systemic reactions. Appropriate interventions against these complications should be considered during TPE, especially in females. [ABSTRACT FROM AUTHOR]

This research aims to investigate the effects of plasma from 12‐month‐old intermittently fasting rats (IFpls) and untreated rats (Npls) on the liver biomolecules and histological changes in 24‐month‐old male Sprague–Dawley rats. Fasting rats underwent an 18‐h daily fasting period and a 6‐h feeding window for 35 days. The plasma was administered bi‐daily, and blood samples were examined for specific liver biomolecules. Fourier transform infrared (FTIR) spectroscopy and linear discriminant analysis (LDA) was used to identify molecular profiles. Liver sections were stained for histopathological evaluation, and the expression levels of Notch signalling pathway components were assessed. Distinct molecular profiles were identified across liver biomolecules, lipids, proteins and nucleic acids with high accuracy. Notably, IFpls was found to protect against hepatic instability, microvesicular steatosis and liver fibrosis by decreasing lymphatic infiltration density and Notch pathway expression levels. Both treatments reduced protein oxidation and carbonylation, with Npls showing a pronounced decrease in protein oxidation. Furthermore, Npls increased protein conformation and glycogen/phosphate content, while IFpls increased glucose/protein content. Both IFpls and Npls induce substantial and unique alterations in liver biomolecules. IFpls offers a protective effect on various liver conditions, while Npls exhibits promising results in reducing protein oxidation and altering biomolecule content. These findings offer valuable insights for future research and potential therapeutic approaches. [ABSTRACT FROM AUTHOR]

Background & Objective: The existing practice in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is to initiate therapy with steroids, intravenous immune globulin (IVIg), or plasma exchange (PLEX) followed by period of immunosuppression. The objective of this study is to assess disability outcomes at 6 months after starting therapy. Methods: Patients who were diagnosed as having CIDP from the Army Hospital of Research and Referral, Delhi; who were initiated and maintained on therapy by treating neurologists with a six month follow up were included in this study. They were retrospectively divided into three groups based on initial therapy received. The primary outcome was comparison of the Inflammatory Neuropathy Cause and Treatment (INCAT) group overall disability sum score (INCAT-ODSS) at 6 months. Secondary outcomes were difference in score at 1 and 3 months, proportion with at least 20% response at 3 and 6 months ("responders") and proportion who needed "rescue" therapy during the 6 months. Results: Sixty patients (26 retrospective, 34 prospective) were included in this study. They were treated with IVIg (33), steroid (19) and PLEX (8). Baseline INCAT-ODSS score (±SD) was 7.2(2.2), 7.2(1.5) and 7.5(1.9) respectively. All received some form of oral immune suppression during follow up. Twenty one (35%) needed additional rescue therapy. There was an overall significant reduction in the mean INCAT-ODSS disability score from 7.2 to 3.1 (Mean difference 4.2; CI 3.6-4.8); p<0.01). Nearly 88% of patients (51/58) showed at least 20% improvement from baseline. Two were lost to follow up (1 IVIg, 1 steroid). There was no difference in the ODSS at 6 months [2.9(2.4), 3.5(2.7) and 2.7(1.3)] respectively. No difference in ODSS at 1 and 3 months. Proportion of responders at 6 months and proportion who needed rescue therapy were also similar. Conclusion: Irrespective of initial therapy and maintenance oral immunosuppression used, the overall disability reduction in treatment with IVIg, steroid or PLEX is significant; however the three modalities are comparable in terms of disability reduction at 6 months. At least a third may need additional rescue therapy. [ABSTRACT FROM AUTHOR]

A 15-year-old female presented with headaches and bilateral vision loss. Fundoscopic examination revealed bilateral optic nerve oedema as well as peripheral retinal haemorrhages. Magnetic resonance imaging of the brain showed findings consistent with bilateral optic neuritis. The patient was started on high dose intravenous corticosteroids but her vision failed to improve. The presence of retinal haemorrhages raised concern that a vasculitis was underlying her symptoms, prompting an extensive work-up, which was unrevealing. Plasmapheresis was initiated and the patient's vision eventually improved to 20/20 in both eyes. Ultimately, she was found to be positive for myelin oligodendrocyte glycoprotein (MOG) antibodies, consistent with a diagnosis of MOG-associated optic neuritis. The patient's course was typical for MOG-associated optic neuritis but her peripheral retinal haemorrhages were atypical, which created diagnostic uncertainty. It is important to be aware of the possibility of retinal findings in this disease. We also review potential causes for retinal haemorrhages in optic neuritis. [ABSTRACT FROM AUTHOR]

Background: Plasma exchange (PLEX) therapy is indicated for several disorders. The 5% albumin is often used as a sole replacement fluid during most PLEX. However, each 1.0 plasma volume exchange depletes coagulation factors by ~65%. Although most coagulation factors recover to hemostatic levels within 24 h post‐PLEX, fibrinogen requires 48–72 h to recover. Fibrinogen is the key coagulation protein for hemostasis. Therefore, fibrinogen is often monitored during the acute course of PLEX, and plasma is supplemented to prevent bleeding if fibrinogen is <100 mg/dL. Study Design and Methods: We conducted a prospective, single‐center, observational study to evaluate bleeding risk in adults who received an acute course of PLEX with a fibrinogen level of 80–100 mg/dL without plasma supplementation during the procedure or before central venous catheter removal. The study group was compared to patients with plasma fibrinogen >100 mg/dL. Results: Among the 275 patients who received 1406 PLEXes, 62 patients (23%) who underwent 323 PLEXes met the inclusion criteria, and only 2 (3%) patients had bleeding while on oral anticoagulants. In contrast, out of 275 patients, 143 (52%) with fibrinogen levels >100 mg/dL received 751 PLEX treatments, and bleeding occurred in 2 (1%) while on low‐molecular‐weight heparin. Conclusions: Our findings suggest that a pre‐procedure fibrinogen threshold of 80–100 mg/dL without plasma supplementation does not increase bleeding risk unless patients were on anticoagulation. See editorial on page 959–962, in this issue [ABSTRACT FROM AUTHOR]

Introduction: Plasma exchange (PE) is considered a Category II option for the treatment of acute attacks and relapse cases of neuromyelitis optica spectrum disorder (NMOSD). However, neurologists are also considering intravenous immunoglobulins (IVIg) as an add‐on therapy for this disorder. Aims: The aim of this study is to evaluate the efficacy of PE in acute attacks of NMOSD when compared with IVIg, in terms of improvement in the Expanded disability status scale (EDSS) and activities of daily living (ADL) scale score and levels of anti‐Aquaporin P4 (AQP4) antibody in seropositive patients. Methods: We enrolled 43 NMOSD patients in two groups: Group 1 (n = 29) received steroids and PE, and Group 2 (n = 14) received steroids with IVIg. The baseline EDSS and ADL scores were recorded and compared with scores at the end of therapy, 4 weeks, and 3 months after. Also, anti‐AQP4 antibody was measured at baseline and post‐therapy in seropositive patients of both groups. Results: We observed a significant difference in EDSS (p = 0.00) and ADL score (p = 0.00) at day 10 and 3 months in both groups. However, no significant difference in EDSS, as well as ADL score from baseline (p = 0.83; p = 0.25) to 3 months (p = 0.85; p = 0.19), was observed when delta change of score at 3 months was compared across the two groups (p = 0.39; p = 0.52). We observed improved visual acuity in both groups with mild improvement in findings of magnetic resonance imaging at 3 months. We observed a significant decline in AQP4 antibody concentration (at day 10) in group 1 seropositive patients (p = 0.013) with improved EDSS (p = 0.027) and ADL scores (p = 0.026) of these patients. Conclusions: PE should be considered as a choice of an add‐on therapy in anti‐AQP4 antibody‐positive NMOSD patients compared with IVIg as it is more effective in reducing antibody concentrations. [ABSTRACT FROM AUTHOR]

Background: Severe fever with thrombocytopenia syndrome (SFTS) is a zoonotic infectious disease caused by the severe fever with thrombocytopenia syndrome virus (SFTSV). Endemic in East Asia, SFTS is characterized by an exceptionally high mortality rate. Presently, there is no established treatment for SFTS, particularly for patients in critical condition. In this study, we collected and analyzed laboratory and clinical data from 92 critically ill patients with SFTS treated at Weihai Municipal Hospital between 2019 and 2022. We hope that our study will provide some hints for the treatment of critically ill patients with SFTS. Methods: A total of 92 critically ill patients with SFTS were included in this study. Of these patients, 45 received treatment with therapeutic plasma exchange (TPE) and ribavirin (referred to as the TPE group), while the remaining patients received only ribavirin (referred to as the non‐TPE group). Clinical and laboratory parameters were analyzed retrospectively. Results: The results showed significant improvements in multiple laboratory parameters following treatment with TPE and ribavirin, including white blood cell and neutrophil count, lactate dehydrogenase, creatine kinase isoenzyme‐MB, prothrombin time, activated partial thromboplastin time, D‐Dimer, serum sodium and copies of virus genomes. The combination of TPE with ribavirin demonstrated a significant reduction in mortality rates, with a mortality rate of 20.0% in the TPE group compared to 40.4% in the non‐TPE group (P = 0.033). Conclusions: Our findings suggest that critically ill patients with SFTS who received TPE and ribavirin experienced improvements in both clinical and laboratory parameters. These results indicate that TPE combined with ribavirin may represent a promising novel therapeutic approach for managing critically ill patients with SFTS. However, comparative studies of large sample size or randomized clinical trials are warranted to confirm the effectiveness of this combination therapy in the treatment of severe SFTS cases. [ABSTRACT FROM AUTHOR]

Background: Acute liver failure (ALF) following yellow phosphorous (YP) ingestion is similar to acetaminophen‐induced ALF and it has become a public concern in our region. This study assessed low volume therapeutic plasma exchange (LV‐TPE) efficacy in improving the transplant free survival in YP poisoning. Methods: Adult patients with toxicology reports of YP and ALF requiring critical care were included in the study. LV‐TPE was planned for three consecutive days and three more if required. Performed 1.3 to 1.5 plasma volume replacing with 0.9% normal saline, 5% human albumin solution, and fresh frozen plasma based on ASFA 2019 criteria. MELD score, laboratory parameters, LV‐TPE details were captured. The study end point was clinical outcome of the patients. Results: Among 36 patients, 19 underwent LV‐TPE and 17 opted out of LV‐TPE and they were included as a control arm. The MELD score was 32.64 ± 8.05 and 37.83 ± 9.37 in both groups. There were 13 survivors in LV‐TPE group leading to a 68.42% reduction in mortality. The coagulation and biochemical parameters showed a significant percentage change after LV‐TPE. Refractory shock, delay in initiating procedure and acidosis were independent predictors of mortality. Conclusion: A well‐timed LV‐TPE improves the survival of patients with ALF due to YP poisoning. [ABSTRACT FROM AUTHOR]

Introduction: Hyperviscosity syndrome (HVS) is a significant complication in Waldenström's macroglobulinemia (WM), although the significance of plasmapheresis in clinical practice has not been clarified. To confirm the efficacy and safety of plasmapheresis followed by primary therapy for WM with HVS, we retrospectively conducted this study. Methods: Untreated WM with HVS, or serum immunoglobulin M (IgM) levels ≥4000 mg/dL, were analyzed. The basic therapeutic flow was conducted as follows: (1) performing plasmapheresis, (2) followed by primary therapy without rituximab, and (3) performing the second cycle. The serum IgM reduction rate and adverse events (AEs) were evaluated. Results: Ten patients were enrolled. The median serum IgM levels at diagnosis, post‐plasmapheresis, after the first cycle of primary therapy, and after the second cycle were 5174, 2900, 3286, and 2657 mg/dL, respectively. No patients experienced IgM flare or bleeding AEs. Conclusion: The therapeutic flow offers sufficient efficacy and safety in WM with HVS. [ABSTRACT FROM AUTHOR]

Introduction: Clinically amyopathic dermatomyositis (CADM) with anti‐melanoma differentiation‐associated gene 5 (MDA5) antibody (Ab) with rapidly progressive interstitial lung disease (RP‐ILD) is often refractory for intensive immunosuppression. In this study, we verified the effectiveness and safety of plasma exchange (PEx) for this lethal disease. Methods: We retrospectively examined the clinical course and adverse effect (AE) of 12 patients with anti‐MDA5 Ab–positive CADM between January 2017 and December 2021 in our hospital. Results: Five out of six patients treated with simple PEx using fresh frozen plasma or 5% albumin survived with or without home oxygen therapy. Multiple PEx (15–20 times) were required to achieve satisfactory improvement as well as remission of CADM. The AEs caused by PEx were resolved using conventional methods. Conclusion: PEx might be a promising option for controlling the disease activity of anti‐MDA5 Ab–positive CADM with severe RP‐ILD and may contribute to better survival. [ABSTRACT FROM AUTHOR]

PURPOSE: To evaluate the effectiveness of plasma exchange (PLEX) for optic neuritis (ON). METHODS: We conducted an international multicenter retrospective study evaluating the outcomes of ON following PLEX. Outcomes were compared to raw data from the Optic Neuritis Treatment Trial (ONTT) using a matched subset. RESULTS: A total of 395 ON attack treated with PLEX from 317 patients were evaluated. The median age was 37 years (range 9-75), and 71% were female. Causes of ON included multiple sclerosis (108), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) (92), aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (AQP4+NMOSD) (75), seronegative-NMOSD (34), idiopathic (83), and other (3). Median time from onset of vision loss to PLEX was 2.6 weeks (interquartile range [IQR], 1.4-4.0). Median visual acuity (VA) at the time of PLEX was count fingers (IQR, 20/200-hand motion), and median final VA was 20/25 (IQR, 20/20-20/60) with no differences among etiologies except MOGAD-ON, which had better outcomes. In 81 (20.5%) ON attacks, the final VA was 20/200 or worse. Patients with poor outcomes were older (P = .002), had worse VA at the time of PLEX (P < .001), and longer delay to PLEX (P < .001). In comparison with the ONTT subset with severe corticosteroid-unresponsive ON, a final VA of worse than 20/40 occurred in 6 of 50 (12%) PLEX-treated ON vs 7 of 19 (37%) from the ONTT treated with intravenous methylprednisolone without PLEX (P = .04). CONCLUSION: Most ON attacks improved with PLEX, and outcomes were better than attacks with similar severity in the ONTT. The presence of severe vision loss at nadir, older age, and longer delay to PLEX predicted a worse outcome whereas MOGAD-ON had a more favorable prognosis. NOTE: Publication of this article is sponsored by the American Ophthalmological Society.

IntroductionAcute fatty liver of pregnancy (AFLP) is a fatal disease occurring in 3rd trimester. The safety and efficacy of plasmapheresis/plasma exchange (PP/PE) as an adjunctive treatment in patients of AFLP has been studied. We performed systematic review and meta-analysis to estimate the clinical parameters that included mortality rates and improvement of the biochemical parameters including Liver and Renal function enzymes, coagulopathy factors of AFLP patients.MethodsWe searched PubMed, Ovid MEDLINE, Cochrane, CINAHL and Scopus, ClinicalTrials.gov. RevMan statistical software was used for meta-analysis.ResultsPooled survival proportion for AFLP patients treated with PP/PE was 87.74% (95% CI: 82.84 to 91.65). Efficacy of PP/PE was studied by its effect on mortality. PE/PP was associated with the reduction in the mortality with pooled odds ratio of 0.51 (95% CI: 0.08 to 3.09) with I2 = 86%. Sensitivity analysis after excluding outlier study, yielded a pooled odds ratio of 0.19 (95% CI: 0.02 to 1.52) with reduced heterogeneity (I2 = 63%). Biochemical parameter analysis demonstrated significant improvement post-PP/PE treatment, including decreased bilirubin (MD: 8.30, 95% CI: 6.75 to 9.84), AST (MD: 107.25, 95% CI: 52.45 to 162.06), ALT (MD: 111.08, 95% CI: 27.18 to 194.97), creatinine (MD: 1.66, 95% CI: 1.39 to 1.93), and Prothrombin time (MD: 5.08, 95% CI: 2.93 to 7.22).DiscussionDespite some heterogeneity, PP/PE shows promise in improving biochemical parameters in AFLP patients. PE can serve as a therapeutic approach for AFLP particularly in severe or refractory cases. PE provides the time for organ to recover and helps in creating a homeostatic environment for liver. Large RCTs and propensity matched studies are needed to better understand the safety and efficacy of the treatment.Systematic review registrationhttps://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42022315698.

IntroductionAD is a progressive neurodegenerative disorder causing significant cognitive decline and impaired daily functioning. Current treatments offer only modest relief, and many amyloid-targeting therapies have failed, prompting exploration of alternative approaches such as PE with albumin replacement.ObjectivesThis scoping review systematically maps the literature on PE with albumin replacement in AD management, focusing on outcomes, methodologies, and reported benefits and risks.MethodsA comprehensive search in PubMed, supplemented by reference scanning and hand-searching, identified studies involving PE with albumin replacement in AD patients. Data charting and critical appraisal were conducted using standardized tools.ResultsSeven primary studies from the AMBAR (Alzheimer Management by Albumin Replacement) trial met the inclusion criteria, consistently reporting improvements in cognitive function, positive neuroimaging results, and favorable neuropsychiatric outcomes. For instance, one study found a significant slowing of cognitive decline (p

Therapeutic plasma exchange (TPE) is a cornerstone treatment for antibody‐mediated rejection (AMR) post‐organ transplantation, aiming to eliminate pathogenic donor‐specific HLA antibodies (DSA). However, limitations in HLA antibody interpretation due to the prozone‐like effect (PLE) can lead to inaccurate assessment of treatment efficacy. We present a case of a heart transplant recipient with suspected AMR, where an unexpected increase in DSA levels post‐TPE prompted investigation into PLE. Solid‐phase Luminex assays were employed to detect HLA antibodies. Serum was run neat as well as after treatment with ethylenediaminetetraacetic acid (EDTA). Nephelometry was used to detect complement levels. Laboratory analysis of pre‐TPE serum revealed higher DSA levels with EDTA treatment, characteristic of PLE. Complement measurements supported complement‐mediated interference in the pre‐TPE sample. This case underscores the importance of being aware that PLE can occur in HLA testing and can impact the interpretation of TPE efficacy for AMR. [ABSTRACT FROM AUTHOR]

Objective To statistically analyze the perioperative results of patients with ABO-incompatible kidney transplantation (ABOi-KT), in order to explore the changes in blood group antibody of type-A/B recipients. Methods A total of 33 cases of blood group A/B ABOi-KT recipients in our hospital from January 2021 to October 2023 were recruited and divided into two groups of group A(n=18) and group B(n=15) according to the different blood types of recipient. The effects of preoperative plasmapheresis on antibody titer, antibody rebound and renal function after operation(serum urea nitrogen, creatinine and estimated glomerular filtration rate on the 1st, 3rd, 7th and 14th day) were analyzed between the two groups. According to the postoperative rebound of blood type antibodies, 33 recipients were divided into antibody rebound group(n=7) and non rebound group(n=26), and the differences in initial blood type antibody titers between the two groups were analyzed. Results There was no significant difference in the clearance rate of IgM with preoperative plasma exchange between the two groups (Z=-0.26, P>0.05); Levels of serum urea nitrogen and creatinine on the 1st, 3rd, 7th and 14th day after operation between group A and group B were not statistically significant(P>0.05), the same as eGFR. Group B was more prone to rebound antibody compared with group A (P0.05) between the two groups was found. Conclusion The patients type B receiving type AB kidney donors are more prone to rebound antibody after ABOi-KT operation compared to the the patients type A receiving type AB.

Objective To analyze the characteristics of blood transfusion consultation cases and establish the consultation route, so as to provide reference for blood transfusion doctors to participate in blood transfusion consultation practice. Methods The cases involved in clinical transfusion consultation in the blood transfusion department of our hospital from 2020 to 2023 were collected from the hospital information system (HIS), and then classified by department and consultation type to summarize the main points of transfusion consultation, formulate transfusion consultation routes, and conduct typical cases analysis. Results There were 315 clinical transfusion consultations from 2020 to 2023, with an increasing trend year by year (26 in 2020, 67 in 2021, 81 in 2022, 141 in 2023). The consultations involved 24 departments, including cardiovascular medicine 14.0%(44/315), orthopedics 12.7%(40/315), intensive care medicine 8.9%(28/315), general medicine 8.3%(28/315), cardiopulmonary disease 6.0%(19/315), etc. There were 8 categories of consultations, including 35.6%(112/315) autologous ozonized blood transfusion, 23.8%(75/315) plasma exchange, 14.9%(47/315) perioperative mass blood preparation (transfusion), 11.4%(36/315) platelet- rich plasma therapy and 6.3%(20/315) autologous blood collection, etc. The clinical blood transfusion consultation route was formulated according to the consultation points. Six patients with various diseases were treated by blood transfusion department. With effective treatment measures taken, all of them improved and were discharged. Conclusion The summary of key points of clinical blood transfusion consultation and formulation of the blood transfusion consultation route by department of blood transfusion are conducive to the implementation of blood transfusion consultation and guarantee the safety of patients.