The purpose of this study was to evaluate the efficacy of Efgartigimod (EFG) in anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis patients during acute attacks.

A case-control study was designed to compare 26 anti-NMDAR encephalitis patients who were treated with EFG, and 15 patients with intravenous immunoglobulin (IVIG), and 23 patients with immunoadsorption with staphylococcal protein A column (SPA-IA) treatment.

At baseline, no significant differences in mRS scores were observed among the EFG, IVIG, and SPA-IA groups of anti-NMDAR encephalitis patients. When compared with the IVIG group, patients treated with EFG had significantly decreased serum IgG levels. Compared with the SPA-IA group, EFG-treated patients had lower CSF anti-NMDAR antibody titers at admission (p = 0.039) and higher post-treatment IgG levels (p = 0.002). When compared with the IVIG group, SPA-IA patients had higher CASE scores (p = 0.022) and baseline IgG levels (p = 0.023). All groups improved the symptoms of anti-NMDAR encephalitis patients after treatment during acute attacks, with significant decreases in mRS and CASE scores from admission to discharge (p < 0.01). In the EFG and SPA-IA groups, there was a significant reduction in anti-NMDAR antibody titers in both CSF and serum (p < 0.01), while no remarkable decrease was found in the IVIG group. Additionally, serum IgG levels significantly decreased in both the EFG and SPA-IA groups post treatment and during the 1-month follow-up. By the 3-month of follow-up, IgG levels in the blood of both groups remained below the baseline levels.

EFG could be an elegant alternative to both IVIG and SPA-IA therapies for anti-NMDAR encephalitis during acute attacks. It has a better effect on reducing antibody titers than IVIG and is comparable to SPA-IA therapy, and no serious adverse events were observed during infusion.

A thyroid storm is the most extreme and life-threatening presentation of thyrotoxicosis. Thyroidectomy can be used for definitive treatment. It should be performed after euthyroidism is accomplished. The use of therapeutic plasma exchange (TPE) is a last resort option in cases where standard pharmacological therapy proves to be ineffective. Due to its rare prevalence, there are limited data evaluating the usefulness and efficacy of TPE as a bridging therapy to thyroidectomy. The absence of relevant literature prompted us to conduct a scoping review. The following bibliographic databases were searched for articles dated 30 November 2023: Medline, EMBASE, Web of Science and Google Scholar. The search identified 1047 records, of which 42 articles were accepted with a total of 234 patients. The dominant indications for TPE were side effects due to conventional treatment. The mean fT4 level decreased 51.9% of baseline after TPE, while the mean fT3 level decreased 66.6% of baseline. The main side effects observed with FFP were allergic reactions, while the use of an albumin solution was associated with perioperative bleeding. Based on the limited data available in the literature, we recognize plasmapheresis as an effective treatment option for reducing thyroid hormone levels prior to thyroidectomy in patients with thyrotoxicosis. Available data suggest that it might be reasonable to limit the number of sessions in favor of an earlier surgical intervention. To reduce the risk of bleeding, FFP may be a better option as a replacement fluid, especially in the session prior to thyroidectomy.

Transfusion of red blood cells (RBCs) can be lifesaving for individuals living with sickle cell disease (SCD). However, alloimmunization after transfusion is more common with patients with SCD than in other patient populations, resulting in morbidity and mortality. Management of complications related to RBC alloantibodies, including delayed hemolytic transfusion reactions (DHTRs) and identifying compatible RBCs for future transfusions, remains a challenge for hematologists and transfusion medicine providers. Although transfusion guidelines from organizations, including the American Society for Hematology provide general recommendations, individual cases remain challenging. Antibody evanescence and the lack of widespread RBC alloantibody data sharing across hospitals pose unique challenges, as do RH variants in both transfusion recipients and blood donors. Further, as potentially curative therapies require RBC transfusions to lower the hemoglobin S before cellular therapy collections and infusions, patients who are highly alloimmunized may be deemed ineligible. The cases described are representative of clinical dilemmas the authors have encountered, and the approaches are as evidence-based as the literature and the authors' experiences allow. A future desired state is one in which RBC alloantibody data are efficiently shared across institutions, Rh alloimmunization can be mitigated, better treatments exist for DHTRs, and a label of difficult to transfuse does not prevent desired therapies.

RhD alloimmunization can result from blood transfusion or fetomaternal hemorrhage (FMH). Preventing alloimmunization in childbearing-age women with FMH via utilization of RhD immunoglobulin (RhIG) is well known; however, there are no established protocols for RhD-mismatched transfusions in emergent or traumatic settings. Here, we describe our hospital protocol for managing RhD negative women who receive RhD positive transfusions.

Pathology or Transfusion Medicine staff are notified of RhD-mismatched blood transfusions. Women with childbearing potential are evaluated by Obstetrics and Gynecology (ObGyn) to determine patients' childbearing desires and physical capabilities, as well as their ability to tolerate RhIG administration. Pathologists determine eligibility for therapy with RhIG: criteria include RhD negative females, <50 years old, without current or historical Anti-D, who have been transfused <20% of their total blood volume (TBV) with RhD positive blood.

Management strategy depends on red blood cell volume (RBCv) transfused. Patients who receive an RBCv ≤20% of their TBV are eligible to receive RhIG, while an RBCv >20% makes individuals ineligible for prophylaxis with RhIG. Red cell exchange (RCX) is not offered at our institution, regardless of RBCv transfused. Women who receive RhIG should be screened for the development of antibodies using direct and indirect antiglobulin tests for 6-12 months posttransfusion. Future pregnancies of alloimmunized women should be carefully monitored.

Our therapeutic plan involves identifying eligible patients based on set criteria. This is the first published protocol to prevent RhD alloimmunization in females of childbearing age due to RhD-mismatched transfusions.

The use of extracorporeal blood purification has become an important therapeutic tool in tertiary hospitals due to the spectrum of clinical applications that go beyond the need for renal replacement therapy. In the emergency room, extracorporeal therapies can be used for the treatment of acute intoxications to remove the circulating toxins before they cause clinical signs or organ failure. In the intensive care unit, extracorporeal therapies are being used more frequently to manage immune-mediated disease that fails conventional treatment with immunosuppressive therapy.

Haemolytic disease of the fetus and newborn (HDFN) remains an important cause of perinatal mortality and morbidity. The pathogenesis underlying this condition is maternal red cell alloimmunization, with immunoglobulin G (IgG) antibodies produced in response to 'non-self', inherited paternal antigens expressed upon fetal erythrocytes. The IgG antibodies cross the placenta into the fetal circulation causing red cell destruction and fetal anaemia. Intrauterine transfusion (IUT) remains the cornerstone therapy with fetal survival rates up to 97%, but it is an invasive, technically challenging surgical procedure performed at specialized medical centres. The procedure-related risk of IUTs is increased at gestational age before 24 weeks. This has stimulated interest in maternal medical therapies that attenuate the risk of severe fetal anaemia, increase the gestational age of first IUTs, and reduce perinatal mortality and morbidity. This review summarizes current evidence for such treatments: intravenous immunoglobulin therapy and neonatal fragment crystallizable (Fc) receptor blockade for managing severe HDFN.

Adult-onset IgA vasculitis (IgAV) often presents with severe renal and gastrointestinal (GI) complications, yet therapeutic guidelines for life-threatening manifestations remain unclear.

We conducted a systematic text-mining analysis of all PubMed-indexed case reports of IgAV treated with therapeutic plasma exchange (TPE).

We describe an 80-year-old woman with refractory GI bleeding and rapidly progressive glomerulonephritis. Despite high-dose corticosteroids and cyclophosphamide, GI bleeding persisted and necessitated multiple transcatheter embolization. A total of seven sessions of TPE using fresh frozen plasma successfully controlled disease activity, resulting in improvement of GI bleeding. Literature review suggests that TPE may provide additive benefits in IgAV patients, particularly in those unresponsive to standard immunosuppressants.

This case supports the utility of TPE as a valuable adjunctive therapy in severe IgAV with organ-threatening manifestations and highlights the need for further studies to define optimal indications.

Cell surface mesothelin (MSLN) can be solubilized and released into the systemic circulation. The resulting soluble MSLN (sMSLN) may interfere with therapies targeting surface MSLN. We investigated the effects of sMSLN on anetumab, an antibody-based therapy against MSLN, anetumab ravtansine, an antibody drug conjugate, and mechanisms to decrease sMSLN. Whole blood samples were collected before and after one plasma volume of therapeutic plasma exchange (TPE). sMSLN levels were measured with ELISA assays in matched pre- and post-TPE plasma samples, and anetumab-immunoprecipitated samples. We also used protease inhibitors (PIs) as a mechanism to stabilize surface MSLN, then evaluated the cytotoxic effects of anetumab ravtansine. Our findings indicate that sMSLN sequesters and may impair the efficacy of this anti-MSLN antibody based on results showing that anetumab decreases the concentration of MSLN in plasma (p < 0.05) and reduced cytotoxicity of anetumab ravtansine in the presence of recombinant MSLN in cell lines, a surrogate for sMSLN. TPE consistently reduced sMSLN (p < 0.05) with an average decrease of 43.6% (15.4 ng/mL). Surface MSLN stabilization was inconsistently observed with PIs. Overall, sMSLN could represent a predictive biomarker for MSLN directed therapies. TPE may be more reliable than PIs to reduce sMSLN and ultimately restore sensitivity to these therapies in patients with high sMSLN.

Therapeutic plasma exchange (TPE) and/or the administration of endovenous immunoglobulins (IGEV) are considered the first line treatment for multiple autoimmune-based neurological diseases. According to the scientific evidence collected in several recent guidelines, the efficacy of both treatments is very similar for many of them, however, the current situation of non-self-sufficiency and the real risk of IGEV shortages make it essential to assess TPE as the first therapeutic option. The objective of this work is to estimate the basic direct costs derived from treatment with RPT compared to treatment with IGEV in immune-mediated neurological diseases in a situation of supposed therapeutic equivalence.

Patients who are treated with IGEV receive a standard dose of 0.4 g/kg weight for 5 consecutive days. Patients treated with RPT with the Terumo-BCT® Optia model cell separator undergo between 5 and 7 sessions, every other day, with a substitution equivalent to 1-1.5 volumes, using 4%-5% albumin as replacement fluid. The calculation of the economic cost, for both types of treatments, in simulation of therapeutic equivalence and safety, has been carried out considering pharmaceutical expenses, calculation of the cost for each dose of IGEV, the detailed costs of consumables, replacement fluids and anticoagulant for RPT, in the worst-case scenario, with central venous catheter (CVC) placement. The price of albumin and immunoglobulins has been adjusted based on the situation of self-sufficiency or dependency and the average value of the last 4 years has been referenced for the calculations. The costs of personnel, hospitalisation, or complications derived from the treatments have not been considered. The prices are indicated in euros including VAT of 4% or 21% as appropriate.

For a patient with a mean weight of 70 kg, the estimated final cost per TPR session, with CVC placement, was €612.66; while the cost for each dose of IGEV. (0.4 g/kg) was €1191. The difference is favorable to the RPT: €2279 [€1,666.4-€2,891.7]. The economic difference presented is probably greater in real clinical practice, since many of the patients do not require CVC placement to perform the TPR, and sessions were performed on an outpatient basis.

The use of TPE in the first line in pathologies in which the clinical results do not present significant differences with the IGEV, optimises the use of blood products and can lead to economic savings. It is necessary to expand this study by including an analysis of the efficacy in our series, as well as the adverse events associated with each type of treatment together with other expenses derived from personnel and hospital admission costs versus the use of outpatient resources (pheresis room).

Post-COVID syndrome (PCS) affects up to 43% of all SARS-CoV-2-infected persons and describes ongoing symptoms months after the acute infection. Despite the large number of affected people, there is still very little evidence about therapeutic options. Some studies suggest at least partially a role of autoantibody-mediated autoimmunity. Immunoadsorption is an extracorporeal therapy to remove circulating antibodies which is used successfully in several autoimmune diseases. We conceived the IAMPOCO trial to evaluate the therapeutic effect of immunoadsorption in patients with PCS.

IAMPOCO is a single-center randomized sham-controlled trial with a crossover design which will enroll 40 participants with PCS and a symptom severity of at least 2 on post-COVID functional scale. All participants will undergo 5 immunoadsorption treatments and after a washout period of 8 weeks 5 sham treatments or vice versa. Which modality is conducted first will be randomized. Patients but not providers of therapy are blinded for which modality is conducted. Primary outcome is the efficacy of IA to the severity of PCS measured by the change of several symptom scores and hand grip strength. Secondary outcomes are the frequency of adverse events and the prevalence of relevant autoantibodies in participants with PCS as well as the concentration of autoantibodies before and after therapy and sham treatment.

The trial addresses the lack of evidence for treatment options in PCS. By using a crossover design and including a sham treatment arm, the study aims to compare the effects of immunoadsorption and sham therapy within the same patients. The trial also benefits from recruiting participants from a cohort study on PCS prevalence, ensuring a thorough evaluation of symptoms. Objective assessments of symptoms are challenging due to their subjective nature, but various scoring systems and tests are being utilized. Despite the lack of data from RCTs, the results of this study have the potential to significantly improve PCS therapy and support evidence-based treatment decisions.

ClinicalTrials.gov NCT05841498. Registered on May 3, 2023.

This case report describes therapeutic plasma exchange as treatment for a rare instance of severe hypertriglyceridemia following high-dose intravenous lipid emulsion (ILE) therapy for bupivacaine-induced local anesthetic systemic toxicity (LAST). The patient received a cumulative ILE dose of 28.0 mL/kg to control seizures, exceeding the typical recommended maximum dose of 10 mL/kg. Clinical presentation included severe headache, visual disturbances, and photophobia, concerning for hyperviscosity syndrome. Laboratory findings revealed a markedly elevated serum triglyceride concentration (> 10 350 mg/dL). Therapeutic plasma exchange with plasma was successfully employed to rapidly reduce serum triglycerides and alleviate symptoms. This case highlights the potential for severe iatrogenic hypertriglyceridemia associated with high-dose ILE therapy for LAST and emphasizes the importance of close clinical monitoring and timely intervention.

The complex process of amyloid-β (Aβ) transportation across the blood-brain and blood-cerebrospinal fluid barriers is crucial for preventing Aβ accumulation, which linked to dementia and neurodegeneration. This review explores therapeutic plasma exchange with albumin replacement in Alzheimer's disease, based on the dynamics of amyloid-β between the brain, plasma, and cerebrospinal fluid.

A comprehensive literature review was conducted using PubMed/Medline, Cochrane Library, and open databases (bioRxiv, MedRixv, preprint.org) up to April 30, 2023. The first search utilized the following MeSH terms and keywords: 'Plasma Exchange', 'Plasmapheresis', 'Therapeutic plasma exchange', 'Apheresis', 'Aβ', 'p-tau', 'Total-tau', 'Alzheimer's disease', 'Cognitive dysfunction', 'neurodegenerative diseases', 'centrifugation', 'membranous', and 'filtration' in the Title/Abstract, yielding 146 results. A second search with the keywords: 'Albumin', 'Aβ', 'BBB', 'Alzheimer's dementia', and 'Nerve degeneration' resulted in 125 additional articles for analysis. Finally, a third search using keywords: 'Albumin structural domains', 'Albumin-Aβ interactions', 'Albumin-endothelial interactions', and 'Post-Translational Modification' produced 193 results for further review.

Therapeutic plasma exchange shows potential as a disease-modifying therapy for dementia, specifically for Alzheimer's disease. Additionally, the promising role of albumin supplementation in cognitive improvement has attracted attention. However, clinical evidence supporting therapeutic plasma exchange for dementia remains limited, necessitating further research and development to mitigate potential adverse effects. A deeper understanding of the molecular dynamics of Aβ transportation and the mechanisms of therapeutic plasma exchange is essential. A critical evaluation of existing evidence highlights the importance of balancing potential benefits with associated risks, which will guide the development and application of these treatments in neurodegenerative diseases.

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is an autoimmune encephalitis, and 46% of women with anti-NMDAR encephalitis have tumors, of which 94% are ovarian teratomas. Patients with anti-NMDAR encephalitis rarely have extra-ovarian teratomas, particularly extra-ovarian mature cystic teratomas arising from the retroperitoneum. We herein report a 27-year-old woman who was diagnosed with anti-NMDAR encephalitis caused by a teratoma arising from the retroperitoneum. In patients with anti-NMDAR encephalitis, it is important to perform a systemic search, including for extra-ovarian teratomas. Plasma exchange significantly improved the patient's clinical symptoms.

The demand for double-filtration plasmapheresis (DFPP) in clinical settings is growing steadily, yet the range and availability of specialized equipment designed to support DFPP are relatively limited. We aimed to assess the efficacy and safety of a designed auxiliary line for DFPP treatment using standard continuous renal replacement treatment (CRRT) machines. This prospective self-controlled study was conducted between May 2021 and April 2024. Patients who underwent DFPP treatment using both specialized DFPP machines and standard CRRT machines (using designed auxiliary line) were enrolled in the study. DFPP sessions were divided into the specialized DFPP machine group and the standard CRRT machine group. The rates of completed DFPP treatments, DFPP cost, circuit clotting, hypotension, anaphylaxis, hypocalcemia, and nurse operating time were compared. A total of 440 DFPP sessions were performed for 80 patients, with 330 (75.0%) sessions in the specialized DFPP machine group and 110 (25.0%) in the standard CRRT machine group. There were no statistically significant differences between the two groups in terms of the completed DFPP treatment rate (89.4% vs. 88.2%, p = 0.724), circuit clotting (8.2% vs. 7.3%, p = 0.760), anaphylaxis (12.7% vs. 11.8%, p = 0.803), hypotension (10.6% vs. 9.1%, p = 0.650), hypocalcemia (17.3% vs. 20.0%, p = 0.519), and nurse operation time (35.08 ± 2.27 min vs. 36.62 ± 1.94 min, p = 0.082). However, the cost per DFPP session in the standard CRRT machine group was lower than in the specialized DFPP machine (976.81 ± 14.38 $ vs. 1007.43 ± 35.30 $, p < 0.001). Standard CRRT machines can effectively and safely perform DFPP treatment using a specially designed auxiliary line, which is more cost-effective. Even in primary hospitals without specialized DFPP machines, CRRT machines can be used to perform DFPP treatment.

Traditionally, patients requiring therapeutic apheresis from rural and remote areas have been compelled to seek treatment at metropolitan hospitals, at great social and economic cost to the patient and the health service, or to receive in-patient apheresis in local intensive care units using renal replacement therapy machines. We outline our experience establishing an apheresis service in Western New South Wales. Our experience demonstrates that a clinically and economically viable apheresis service in the rural setting can be established.

Therapeutic plasma exchange (TPE), an extracorporeal method targeting the removal of large molecular weight pathogens, is explored in this study for indications, complications, prognosis, safety, and effectiveness.

The patients' data were collected retrospectively.

Overall, 334 sessions of TPE were applied to 57 patients. Per the American Society for Apheresis classification, 24.6% of indications fell under Category I, 14% Category II, and 50.9% Category III. Sepsis-induced multiorgan dysfunction syndrome (MODS) emerged as the leading indication, correlating with elevated needs for mechanical ventilation (MV), increased failed organs, and heightened mortality. Patients undergoing continuous renal replacement therapy faced a 16.06 times higher mortality risk. Non-survivors exhibited higher comorbidity, prolonged MV, increased inotropic drug requirement, more failed organs, and a higher PRISM score. 33.2% of complications occurred, primarily catheter-related.

Sepsis-induced MODS and extracorporeal modalities are associated with increased mortality in TPE patients, with comorbidities, ventilation, and PRISM scores potentially influencing outcomes.

Therapeutic plasma exchange (PEX) has emerged as a potential treatment option for patients with acute liver failure (ALF). The effect of PEX on survival outcomes outside of clinical trials is not yet well established. In this study we aimed to evaluate the real-world use and outcomes of PEX for the treatment of ALF.

This multicentre retrospective cohort study included consecutive patients with ALF admitted to all seven tertiary liver transplant centres in the UK between June 2013 and December 2021. Changes in clinical variables following PEX treatment were assessed, while overall survival and transplant-free survival up to hospital discharge in patients receiving PEX were compared to those receiving standard medical therapy Propensity score matching was performed to control for intergroup covariates and selection bias.

We included 378 patients with ALF (median [IQR] age 36 (28-48), 64% [n = 242] female) of whom 120 received PEX. There was a significant improvement in most clinical variables following PEX, including median dose of noradrenaline (reduction from 0.35 μg/kg/min [0.19-0.70 μg/kg/min] to 0.16 μg/kg/min [0.08-0.49], p = 0.001). There was no significant difference between PEX and standard medical therapy groups in overall survival (51.4% vs. 62.6%, respectively, p = 0.12) or transplant-free survival (42.6% vs. 53.1%, p = 0.24).

PEX is now frequently used in the management of patients with ALF in the UK. It is associated with significant improvement in haemodynamic parameters but not survival benefit.

Therapeutic plasma exchange is frequently used in the management of patients with acute liver failure in the UK. This real-world study demonstrates significant improvement in haemodynamic status but has not confirmed the survival benefit seen in previous published literature. These results should help guide the future use of plasma exchange in this patient population.

Sudden sensorineural hearing loss (SSHL) is an abrupt hearing loss, often of unknown cause. Apheresis is a treatment option aimed at improving blood hemorheology by removing pathogenic blood components. There are currently no previous meta-analyses on its efficacy. Therefore, the aim of this study was to evaluate the efficacy of apheresis in achieving total, complete, and partial remission of SSHL, as well as remission outcomes based on the type of apheresis used.

A systematic search was performed in PubMed, Scopus, Web of Science, and the Cochrane Library until March 2024. Random-effects models were used to calculate pooled estimates of treatment success rates (TSR) and their respective 95% CI to analyze the efficacy of apheresis in the remission of SSHL. Subgroup analyses were performed by type of apheresis (HELP-apheresis and rheopheresis).

The systematic review included 12 studies (10 in the meta-analysis) involving 786 adults with SSHL. The effect of apheresis showed significant total remission (TSR: 0.55; 95% CI: 0.47, 0.64), complete remission (TSR: 0.21; 95% CI: 0.11, 0.30), and partial remission (TSR: 0.43; 95% CI: 0.37, 0.48). Subgroup analysis revealed significant remission rates for HELP-apheresis (TSR: 0.58; 95% CI: 0.52, 0.64) and rheopheresis (TSR: 0.51; 95% CI: 0.30, 0.72).

These findings support apheresis as an equally or more effective treatment for SSHL, particularly in cases where corticosteroid therapy fails. However, due to the unknown etiology of SSHL, further clinical trials with larger, diverse populations are essential to confirm these results.

Paraneoplastic (p) TTP is a rare syndrome characterized by an immune-induced, generalized microangiopathy associated with solid or hematological tumors. This case, reporting a patient with a metastatic HER2+ breast cancer and a pTTP, highlights the rarity of this entity, its difficult and challenging diagnosis, and the complexity of its management.

Thrombotic microangiopathy (TMA) following lung transplantation (LTx) is a rare but severe complication. The pathogenesis is poorly understood, and various risk factors have been suggested. In this study, we aimed to evaluate diagnostic accuracy, identify risk factors, and assess renal, pulmonary, and overall survival of TMA in this patient group.

We performed a case-control retrospective study of patients with TMA after LTX between January 1, 2000, and January 1, 2021. Controls were selected based on underlying lung disease, age, sex, cytomegalovirus risk, and immunosuppressive regimen. Overall survival data were collected for the whole lung transplant group.

A total of 29 TMA cases (2.9%) were identified out of 1025 LTx. Median time to development of TMA was 5.9 mo, 76% occurred in the first 12 mo. In the TMA group a higher rate of HLA donor-specific antibodies (11% versus 1%; P = 0.05), a lower median time to onset of chronic lung allograft dysfunction (37 versus 91 mo; P = 0.0017), a higher rate of cytomegalovirus infection (45% versus 19%; P = 0.02), and a higher prevalence of end-stage renal disease (24% versus 6%; P = 0.03) and overall death (97% versus 44%; P < 0.0001) was found. Diagnostic assessment of TMA was complete in 48% of patients, with Coombs testing missing in 52% and a disintegrin and metalloproteinase with thrombospondin type 1 motif 13 activity not assessed in 59%.

TMA poses a significant risk of end-stage renal disease and mortality after LTx. Challenges remain in standardizing diagnostic criteria and understanding its pathogenesis, underscoring the need for unified protocols in diagnosis and standardized screening. This study identifies potential risk factors and temporal patterns for TMA occurrence, providing crucial insights for future treatment strategies.

The objective of this study is to critically assess the diverse indications for HAS administration over the past 8 years at a prominent tertiary care institution in the United Kingdom.

This is timely and relevant, given recent developments in the field. The International Collaborative Transfusion Medicine Group (ICTMG) issued updated guidance on intravenous albumin use in March 2024, reflecting a shift towards more stringent criteria for its application, which necessitates a re-evaluation of current practices.

We conducted a retrospective analysis of the usage of HAS from August 2015 to March 2023. Records of all patients who received HAS during this study period were extracted from the Electronic Patient Records (EPR) of Guys and St Thomas' NHS Foundation Trust London (GSTFT). Data analysis was performed using SPSS version 20.

A total of 4816 patients received HAS during the audit period, with 21 231 HAS infusions. The majority of infusions were for 20% HAS, with 16 772 (79% of total) infusions for 20% HAS in 4145 patients. A total of 4459 (21%) infusions were recorded for 4.5 and 5% HAS.

Our study provides a comprehensive analysis of albumin utilisation and TPE indications over the past 8 years at our institution, establishing a crucial baseline for refining our practices.

To evaluate the prognostic significance and safety of therapeutic plasma exchange (TPE) in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) with severe organ or system involvement.

Data of patients diagnosed with AAV between 2011 and 2021 were evaluated retrospectively. Patients with baseline estimated glomerular filtration rate (eGFR) ≤ 50 mL/min/1.73m2 or diffuse alveolar haemorrhage (DAH) were included in the analysis (n = 71). Patients who underwent TPE were compared with others in the groups formed after two models of propensity score matching (PSM). Initial PSM was performed according to the presence of DAH, age, gender, eGFR, and BVAS. A data-driven approach was conducted for the second model.

In the initial PSM cohort (n = 48), the remission rate at 6 months was lower in the TPE group (p = 0.04). There were no significant differences in mortality and improvement of eGFR at 6 and 12 months. No severe complications due to TPE were observed. Rates of serious infections (SIs) and end-stage renal disease (ESRD) at 12 months were higher in the TPE group (p = 0.016 and 0.02, respectively). Data-driven PSM analysis (n = 44) revealed no significant differences between groups.

We did not demonstrate a positive effect of TPE on remission, ESRD, and mortality in AAV in this study. Despite low short-term complication rates, the increased risk of SIs possibly associated with ESRD was remarkable. The limited long-term benefits of TPE should be carefully weighed against its associated risks when selecting patients for treatment.

Familial chylomicronemia syndrome (FCS) is a rare Mendelian autosomal recessive disorder (MIM 238600) characterized by extreme and sustained hypertriglyceridemia due to profound reduction of lipoprotein lipase (LPL) activity. This expert opinion statement synthesizes current knowledge on the definition, pathophysiology, genetics, prevalence, diagnosis, and management of FCS. FCS typically manifests at a young age with persistent severe hypertriglyceridemia-defined as ≥10 mmol/L (≥885 mg/dL), or ≥1000 mg/dL (≥11.2 mmol/L) depending on region and whether Systeme International (SI) units are utilized-in the absence of secondary factors, resistance to conventional lipid-lowering therapies, and a high lifetime risk of acute pancreatitis. It is caused by biallelic pathogenic variants in the LPL gene encoding LPL, or 1 of 4 other related genes that encode proteins that interact with LPL. Affected individuals require a strict, lifelong very low-fat diet with <15% of energy from fat. Emerging therapies inhibiting apolipoprotein C-III show promise in reducing serum triglycerides and pancreatitis risk in patients with FCS. A multidisciplinary approach, encompassing dietary management, pharmacotherapy, and patient education, is pivotal in mitigating the significant morbidity associated with FCS.

While a dysregulated immune response is at the center of the sepsis definition, standard care is still solely focussed on prompt administration of antimicrobial therapy, source control, resuscitation and organ supportive therapies. Extracorporeal blood purification therapies, such as haemoadsorption, have been proposed as a possible adjunctive therapy to standard care in sepsis. These adsorption devices aim to rebalance the dysregulated immune response by removal of excessive amounts of circulating inflammatory mediators, including cytokines and endotoxins. Thus far, the effects of haemoadsorption on clinical outcomes have been insufficiently studied and although its routine use is not justified based on the current evidence, multiple centers use these devices in patients with severe septic shock. This narrative review describes the most well-studied adsorption devices as well as a novel selective adsorption device called the 'IL-6-Sieve', including in vitro data showing its capturing potential. Finally, it addresses important considerations for future trials on haemoadsorption in septic patients.

Snake bites are a major cause of emergency visits in tropical countries like India, with actual mortality and morbidity likely higher due to underreporting. The aim of the study was to analyze the clinical and demographic profiles of snake bites at the Department of Emergency Medicine, AIIMS Rishikesh, over two years (July 2021 to July 2023).

Patients aged over 18 with witnessed or suspected snake bites were included. Data on demographics, clinical history, laboratory parameters, treatment, and outcomes were collected.

Most patients were male (68.3%) and aged 31-50 years (35.6%). Farmers made up 57.4% of the cohort. Bites occurred mostly in the evening (46.5%) and during the monsoon (71.3%). Symptoms varied: 48.5% were asymptomatic, 31.7% had hemotoxic symptoms, and 15.8% experienced neurotoxic symptoms, including ptosis. Hemotoxic bites frequently involved bleeding at the bite site (93.8%) and gum bleeding (46.9%). Local complications were noted in 7.9% of cases. Neuroparalytic bites required ventilatory support in 62.5%. Blood products were administered to 31% of patients with hemotoxic bites, hemodialysis to 19%, and plasmapheresis and hyperbaric oxygen therapy to 6.3%. Out of the 69 symptomatic patients (68.3%) who received anti-snake venom (ASV), 28 (40.6%) patients developed adverse reactions.

This study provides a detailed analysis of suspected snakebites in Uttarakhand and surrounding areas, highlighting the importance of early recognition, prompt treatment, and timely referral to prevent fatalities. The administration of anti-snake venom (ASV) is identified as the most critical intervention, though lack of awareness in rural areas complicates management. The study calls for targeted public health campaigns to educate communities about early snakebite recognition and the role of ASV. It also stresses the need for region-specific protocols and improved healthcare access, emphasizing the importance of referral systems for advanced interventions like hemodialysis and intubation. Overall, the study advocates for enhanced public awareness and healthcare infrastructure to reduce snakebite incidence and mortality in rural populations.

Therapeutic plasma exchange (TPE) has emerged as a promising treatment option for pediatric liver failure (PLF) either as a standalone therapy or as a bridge to liver transplant; however, its precise impact on survival outcomes has not been investigated systematically to date. This meta-analysis aims to evaluate the effect of TPE on survival of pediatric patients with liver failure.  METHODS: PubMed, Scopus and Embase databases were searched to include all studies till August 2024 reporting the effect of TPE on survival of acute and acute-on-chronic liver failure patients of age < 18 years. Primary outcome measures were overall survival (OS) and transplant-free survival (TFS) at Day ≥ 28 in pediatric acute liver failure (PALF) and pediatric acute-on-chronic liver failure (pACLF) patients undergoing TPE. The secondary outcome measure was to determine changes in biochemical parameters (international nrmalized ratio [INR], bilirubin and ammonia) pre and post-TPE in them.

Twelve studies (8 = exclusive PALF cohorts and 4 = combined PALF + pACLF cohorts) comprising 310 patients (273 = PALF and 37 = pACLF) who received TPE were included. Pooled OS at Day ≥ 28 for PLF after TPE is 61% (95% CI: 55-66%, p = 0.03, I2 = 49%). The estimated pooled TFS in them was 35% (95% CI: 29-41%, p = < 0.01, I2 = 84%). On sub-group analysis, the standard-volume TPE group had both higher OS and TFS in comparison to the high-volume sub-group. There was a significant improvement in all three biochemical parameters post-TPE compared to pre-TPE values. None of the included studies reported any TPE-related mortality or potentially fatal side effects.

TPE shows the potential to improve overall survival in pediatric liver failure, mostly acting as a bridge to liver transplant or native liver recovery. Further, well-designed, adequately powered, randomized-controlled trials are needed to confirm TPE's survival benefit in PLF.

Red blood cell (RBC) utilization in patients with sickle cell disease (SCD) in Canada is poorly defined. This study describes RBC utilization in an SCD cohort at a single Canadian center.

All adults with SCD who received care at the Ottawa Hospital between January 2006 and May 2019 were included, and followed until December 2021. Data on hospital encounters and RBC transfusions were obtained from hospital databases.

Overall, 273 patients were included (median age: 25 years; 53.8% female; median follow-up: 8.1 years). During the study period, there were 23,127 hospital encounters (median: 45 [interquartile range (IQR) 18, 100] per patient), with 165 patients (60.4% of cohort) receiving 22,538 RBC units. Most RBC units (86.5%) were transfused in the outpatient setting. Although only 2.9% of patients had O-negative blood type, O-negative RBC units accounted for 29.1% of units transfused. One hundred forty-seven patients received 2205 RBC units (9.8% of total) as simple transfusions (median: 5 [IQR 3, 13] per patient), and 88 patients received 20,333 RBC units (90.2% of total) during 2702 red cell exchange (RCE) sessions (median: 14.5 (IQR 1, 47.5) RCE per transfused patient). A median of 7 RBC units (IQR 6, 9) were transfused per RCE session, with a median of 30 days (IQR 28, 40) between sessions.

Patients with SCD at our center frequently received O-negative units, and large RBC volumes were used for RCE. These results provide a utilization baseline for future education and quality improvement initiatives to optimize RBC stewardship.

Management of severe circulatory collapse in the setting of amlodipine toxicity can be challenging. High doses of vasopressors and conventional therapies fail to improve hemodynamics, resulting in the use of veno-arterial extracorporeal membrane oxygenation (VA-ECMO) to treat severe cardiogenic shock and peripheral vasodilatation. Therapeutic plasma exchange (TPE), which helps remove plasma protein-bound toxins and significantly reduces mortality, may be a useful adjunct to invasive hemodynamic support in severe cases of amlodipine poisoning.

A 32-year-old female with a history of intentional consumption of ninety 5-mg amlodipine tablets (totaling 450 mg) was admitted to our intensive care unit (ICU) after 3 h. Her amlodipine serum concentration was 147 ng/mL. She presented with cardiogenic shock and fatal vasoplegia and received VA-ECMO and TPE. The patient was weaned off ECMO after 4 days and discharged home on Day 10 of hospitalization.

Amlodipine toxicity can result in severe cardiac failure with circulatory collapse. We describe the case of a patient with cardiovascular collapse who successfully bridged to recovery from refractory shock secondary to severe amlodipine toxicity as a result of ECMO and TPE treatment.

Leukapheresis is a potentially life-saving therapy for children with symptomatic hyperleukocytosis. However, the standard centrifugation-based approach exposes pediatric patients to significant complications due to its large extracorporeal volume, high flow rates, and considerable platelet loss. Here, we tested whether performing cell separation with a high-throughput microfluidic technology could alleviate these limitations. In vitro, our microfluidic devices removed ~85% of large leukocytes and ~90% of spiked leukemic blasts from undiluted human whole blood, while minimizing platelet losses. Multiplexed devices connected in parallel allowed for faster, clinically relevant flow rates in vitro with no difference in leukocyte collection efficiency. When connected to Sprague-Dawley rats, the devices removed large leukocytes with ~80% collection efficiency, reducing the leukocyte count in recirculating blood by nearly half after a 3-hour procedure. Evaluation of plasma biomarkers and end-organ histology revealed no adverse effects compared to sham control. Overall, our study suggests that microfluidics-based leukapheresis is safe and effective at selectively removing leukocytes from circulation, with separation performance sufficiently high to ultimately enable low extracorporeal volume leukapheresis in children.

Therapeutic Plasma Exchange (TPE) is an extracorporeal treatment modality used to manage certain conditions caused by plasma deficiencies, autoantibodies, alloantibodies, toxins, and immune complexes. We describe our experience of using TPE for various disease indications and associated complications.

This is a retrospective, single-center review of centrifuge-based TPE performed by the division of nephrology at a tertiary care academic center between July 2018 to June 2022. 1219 TPE treatments in 145 patients were included.

The most common indications for TPE were Antibody-Mediated Rejection (AMR) of a kidney transplant (20%), autoimmune encephalitis (16%), and neuromyelitis optica (11%). Rare indications for TPE included Chronic Relapsing Inflammatory Optic Neuropathy (CRION), AMR of a pancreas transplant, osmotic demyelination, and belatacept removal in the setting of COVID-19. The most common complications were depletion coagulopathy (47.6%), hypocalcemia (44.1%), and hypokalemia (36.6%). Rare complications included stiff person crisis and pseudohypertriglyceridemia. 31.7% of patients received TPE for conditions managed by nephrologists.

TPE is an extracorporeal treatment modality for managing various renal and non-renal diseases. The study demonstrated that 18.7% of the patients at our center received TPE for conditions in which its role is not yet established, suggesting the need for further research on the use of TPE. In addition, this study supports the necessity of nephrology training program to include education on TPE as almost one-third of the indications for TPE in our center were for conditions managed by nephrologists.

Thrombotic microangiopathy (TMA) is associated with carfilzomib, but the potential association between bortezomib or ixazomib exposure and TMA is still unknown. Besides, the knowledge of carfilzomib-induced TMA is based mainly on case reports. We aim to quantify the risk and better characterize the clinical features of proteasome inhibitor (PI)-induced TMA.

Data from 2004 to 2023 on TMA events induced by PIs were retrieved from the FDA Adverse Event Reporting System (FAERS) database and conducted disproportionality analyse. Case reports/series from 2004 to 2023 on PI-induced TMA were extracted and analyzed retrospectively.

FAERS pharmacovigilance data identified 225 TMA cases across 213 individuals related to PIs therapy. PIs were significantly associated with TMA (n = 213, ROR 1.71 [1.49-1.96]; EBGM 1.70 [1.52]), and carfilzomib had the greatest proportion (58.7%) and highest positive signal values (n = 125, ROR 17.97 [15.04-21.47]; EBGM 17.49 [15.07]) of TMA. Sixty cases (median age: 63 years) from 35 studies showed evidence of TMA, with 37 (61.7%) were male. The typical initial symptoms were gastrointestinal symptoms (45.3%), fever (24.5%), fatigue/asthenia (20.8%), neurological signs (18.9%), and dyspnea (17.0%). The median time to TMA onset was 8 days. Most patients presented with hemolytic anemia (98.1%), thrombocytopenia (96.6%), and acute kidney injury (96.7%). Cessation of PIs and treatment with plasma exchange therapy (25.0%), hemodialysis (31.7%), and eculizumab (26.7%) were associated with improved hematologic outcomes (96.3%) and renal outcomes (93.3%).

This study identified PIs agents with significant reporting associations with TMA. A prompt diagnosis of TMA and supportive treatments are necessary for patients receiving PIs concurrent with anemia, thrombocytopenia, and acute kidney injury.

To date, there is no shared national guideline in Italy for the management of reproductive health in rheumatic diseases (RHRD). The Italian Society for Rheumatology (SIR) has committed to developing clinical practice recommendations to provide guidance on both management and treatment regarding RHRD in Italy.

Using the GRADE-ADOLOPMENT methodology, a systematic literature review was conducted to update the scientific evidence that emerged after the publication of the reference recommendations from the American College of Rheumatology. A multidisciplinary group of 18 clinicians with specialist experience in rheumatology, allergy and clinical immunology, internal medicine, nephrology, gynecology and obstetrics, and neonatology, a professional nurse, a clinical psychologist, and a representative from the National Association of Rheumatic Patients discussed the recommendations in collaboration with the evidence review working group. Subsequently, a group of stakeholders was consulted to examine and externally evaluate the developed recommendations.

Recommendations were formulated for each area of interest: contraception, assisted reproductive technology, preconception counseling, and use of drugs before, during, and after pregnancy and during breastfeeding, considering both paternal and maternal exposure.

The new SIR recommendations provide the rheumatology community with a practical guide based on updated scientific evidence for the management of RHRD.

Severe Guillain-Barré syndrome (GBS) patients may not show improvement after a single course of intravenous immunoglobulin (IVIg) therapy. Current treatment options include either a second course of IVIg or therapeutic plasma exchange (TPE). This systematic review aims to evaluate the current literature on the use of a second course of IVIg or TPE in patients who fail to show clinical improvement after the first IVIg course.

We searched PubMed, Embase and Medline databases up until 26 October 2023. Studies that evaluated adult patients with confirmed GBS who have failed one full course of IVIg and subsequently received either repeat IVIg or TPE were included. Risk of bias was performed using study-specific checklists. A narrative synthesis of results is presented.

A total of 37 articles were identified (1 randomised controlled trial (RCT), 3 observational and 33 case reports/series), consisting of 422 patients in total. 12 studies evaluated repeat IVIg and 24 studies evaluated TPE after IVIg. There was no superiority of a repeat course of IVIg or TPE in all clinical outcome measures.

The evidence suggests with a low degree of certainty that there is no beneficial effect of further IVIg in unresponsive GBS. The quality of evidence regarding TPE after IVIg is insufficient to suggest any efficacy due to a lack of RCTs. We recommend standardised case reporting with consideration for a multinational case registry and RCTs to determine the efficacy of TPE after initial IVIg unresponsiveness.

We aimed to evaluate the characteristics of the patients with a rheumatologic disease who underwent TPE.

A single-center, retrospective study was conducted between January 2016 and June 2023.

Twenty patients with a median age of 51 years received a median of 6 TPE sessions. Concurrently, immunosuppressive therapy was administered to 18 (90%) of them. During the follow-up period, 9 patients (45%) died. Creatinine (p = 0.001), C-reactive protein (p = 0.001), sedimentation rate (p = 0.002), leukocyte (p = 0.003), thrombocyte (p = 0.003), and neutrophil (p = 0.003) counts was decreased after TPE. Similarly, in the ROC analysis of post TPE laboratory parameters, urea, creatinine, CRP, hemoglobin, platelets, and lymphocytes predicted mortality with areas under the curve values ranging from 0.747 to 0.869. In the Cox regression analysis for mortality, creatinine was predictive for mortality (p = 0.030), HR 1.59 (95% CI: 1.05-2.41).

In rheumatologic conditions, TPE is beneficial to fill the gap until the effects of immunosuppressants become apparent.

Therapeutic plasma exchange (TPE) is generally well tolerated but Anticoagulant Citrate Dextrose Solution A (ACD-A) can cause citrate mediated hypocalcaemia so, adding calcium gluconate to the replacement fluid is effective in preventing this complication. We aimed to compare the effect of different concentrations of 10 % calcium gluconate (9.3 mg of elemental calcium/100 ml Vs 18.6 mg of elemental calcium/100 ml) added to 5 % Human Serum Albumin (HSA) on intraprocedural and post procedural ionised calcium (iCa2+) levels in patients with neurological conditions undergoing TPE.

This study comprised of 100 TPE procedures divided into two groups of 50 each. In group 1, 5 ml of 10 % calcium gluconate was added in 500 ml of 5 % HSA (9.3 mg of elemental calcium/100 ml) and in group 2, 10 ml of 10 % calcium gluconate was added (18.6 mg of elemental calcium/100 ml) in 5 % of HSA. Ionised calcium was noted-pre, intra and post-procedure and compared within the groups along with other procedural parameters and adverse events if any.

We observed that mean intraprocedural ionised calcium levels were comparable (p = 0.579) in both the groups, but post-procedural iCa2+ levels significantly decreased (p = 0.003) in group-1 as compared to group-2. Symptomatic hypocalcaemia was seen in 14 % of patients group 1 compared to 2 % in group-2. Vasovagal reactions were 8 % in group-1 % and 2 % in group-2.

Prophylactic addition of 18.6 mg of elemental calcium/100 ml of 5 % HSA is safe to maintain levels of iCa2+ throughout the procedure with lower chances of adverse events related to hypocalcaemia.

Acute liver failure poses a significant challenge in surgical critically ill patients. Treatments typically focus on physiological support and alleviation of hepatic insult. This study aims to evaluate the role of high-volume plasma exchange (HVPE) in surgical critically ill patients with medical jaundice and hepatic failure.

A retrospective review was conducted on surgical critically ill patients with hepatic failure unresponsive to conventional therapy, excluding those with obstructive jaundice. HVPE was considered for patients with persistent hyperbilirubinemia (> 10 mg/dL) and coexisting conditions such as coagulopathy, hyperammonemia, more than Grade II hepato-encephalopathy, or exacerbated sepsis/septic shock status or multiple organ failure. Patients were categorized into standard medical treatment (SMT) and SMT + HVPE groups. Demographics and laboratory data were collected for analysis.

A total of 117 patients were enrolled, with 79 in the SMT group and 38 in the SMT + HVPE group. There were no significant differences in laboratory data and MELD score upon admission. Before treatment, patients in the SMT + HVPE group exhibited higher levels of T-bil., D-bil., and sugar than the SMT group. After treatment, the SMT + HVPE group showed lower serum D-bil. and AST levels but higher levels of albumin and platelets compared to the SMT group. The SMT + HVPE group demonstrated significantly lower delta T-bil., delta D-bil., and higher delta platelet levels. The survival rate was 31.6% (12/38) in the SMT + HVPE group and 1.3% (1/79) in the SMT group. The in-hospital mortality rate in the SMT + HVPE group was lower than that in the SMT group, with a hazard ratio of 0.42 in the crude model and 0.34 (95% CI = 0.20-0.60 and p = 0.0002) in the adjusted model.

Our findings suggest that HVPE improves survival rates in surgical critically ill patients with medical jaundice and hepatic failure. However, due to its retrospective nature, further studies were warranted.

A 75-year-old man with blurred vision and nasal bleeding was diagnosed with hyperviscosity syndrome and central retinal vein occlusion secondary to Waldenström macroglobulinemia. Serum total protein and IgM levels were undetectable. Because of the severe symptoms, we determined that immediate plasma-exchange treatment was required to decrease the blood viscosity. The initial plasma exchange was performed using the membrane isolation method with a predilution standby. A saline predilution replacement was prepared to decrease the total membrane pressure (TMP); however, the predilution protocol was not used because the planned treatment volume could be achieved without increasing the TMP. After two consecutive days of membrane plasma exchange, all serum biochemical tests were measurable, and IgM was below 4000 mg/dL. After chemotherapy, his visual symptoms improved, and he was discharged. Since it is difficult to assess the risk of elevated TMP prior to initial plasma exchange, membrane plasma exchange with a predilution standby may be a useful strategy for initial plasma exchange for hyperviscosity syndrome in terms of safety and efficiency.

Chronic pancreatitis is a fibroinflammatory disease of the pancreas with heterogeneous clinical features and a significant socioeconomic burden. Assessing its aetiology and early diagnosis of associated complications remain challenging. Personalized therapy necessitates precise knowledge of the genetic, biological, and clinical differences within a patient population. In this context, the identification of the underlying aetiology represents an essential cornerstone. This review elucidates current standards for identifying underlying aetiologies and the diagnostic work-up for idiopathic cases. It provides an overview of general therapeutic approaches and highlights individual treatment options. Additionally, the follow-up management of pancreatitis-associated complications, namely exocrine pancreatic insufficiency, post-pancreatitis diabetes mellitus, pain management, pancreatic fluid collections, and pancreatic cancer risk, is summarized.