Sessions Of Plasma Exchange Research Articles

6414 Refractory Hyperthyroidism Following COVID-19 Infection

Abstract Disclosure: A.T. Wahba: None. A.A. Shibli-Rahhal: None. A 40-year-old man presented with 2 weeks of palpitations, weight loss, decreased appetite and malaise. The symptoms were preceded by neck pain for 1-2 weeks that improved spontaneously. He was in atrial fibrillation at 126 bpm, but otherwise clinically stable. He had a mildly tender enlarged thyroid with no palpable nodules, and the rest of his exam was negative. A chest CT with IV contrast ruled out pulmonary embolism. His TSH was < 0.01 µIU/mL (N:0.27-4.20), free T4 > 7.77 ng/dL (N: 0.80-1.80) and free T3 of 29.7 (N: 2.6-4.4 pg/ml), obtained prior to contrast administration. TSI antibody was negative and thyroid ultrasound showed diffuse enlargement with heterogeneous echotexture and reduced vascularity on doppler images. Thyroid uptake and scan could not be obtained due to iodinated contrast exposure. The patient’s medical history was notable for nonischemic cardiomyopathy (EF 50%), cardiac arrest with implantable cardiac defibrillator, and paroxysmal atrial fibrillation. He was previously treated with amiodarone for 2 years, and it was stopped 2 months before presentation. Other relevant medical problems included subclinical hypothyroidism diagnosed within the previous year and an uncomplicated acute COVID-19 infection 1 month prior to presentation.Given his cardiac history and high Burch-Wartofsky score, he received PTU 200 mg Q4h, hydrocortisone 100mg Q8h and propranolol for 3 days. As his FT3 level declined to 9.8 pg/ml, he was transitioned to methimazole 40 mg daily, propranolol and prednisone 40 mg daily and discharged with outpatient follow up. The atrial fibrillation worsened again 1 week later, and his FT3 increased to 20.2 with a FT4 > 7.77. He was put back on PTU 200 mg TID, prednisone and propranolol were continued and cholestyramine 2g BID was added. 1 week later, his FT4 and FT3 were unchanged. The tests were performed using a different assay and the results were consistent. Two sessions of plasma exchange (PLEX) did not yield a significant response, so thyroidectomy was eventually performed. Pathology showed morphologic findings consistent with type 2 amiodarone-induced thyrotoxicosis. While pathology showed amiodarone-induced changes, the patient’s hyperthyroidism occurred after a COVID-19 infection and was associated with neck pain, pointing to severe viral thyroiditis. De novo thyroid hormone production has been reported following COVID-19 infection, but the minimal improvement in thyroid hormone levels with thionamide therapy suggests that de novo secretion had little contribution to this patient’s hyperthyroidism. Presentation: 6/3/2024

Decreased NETosis-related regulators in neuromyelitis optica spectrum disorders after plasma exchange

ObjectivesTo investigate the impact of plasma exchange (PLEX) on NETosis-related regulators and their correlation with neurological improvement in NMOSD patients. MethodsTwelve aquaporin-4 antibodies seropositive NMOSD patients were enrolled. NETosis-related regulators (myeloperoxidase [MPO], citrullinated histone H3 [CIT-H3], peptidyl arginine deiminase 4 [PAD4], neutrophil elastase [NE], CD64), pro-inflammatory cytokines (IL-1, IL-6, IL-12, TNF-α) and anti-inflammatory cytokines (IL-10, TGF-β1) were quantitatively assessed before and after PLEX treatment. Clinical assessments included expanded disability status scale (EDSS) and visual outcome scale (VOS) scores. ResultsFollowing PLEX, all patients showed symptom improvement, with 66.7 % achieving marked-to-moderate improvement (MMI) at 3 months. Key regulators, such as MPO, CIT-H3, PAD4, NE, and pro-inflammatory cytokines such as IL-1, IL-6, IL-12, and TNF-α, exhibited a statistically significant decrease immediately after the initial PLEX session (P < 0.05). Furthermore, CD64 levels demonstrated a substantial decline after the second PLEX session (P < 0.05). Conversely, the levels of anti-inflammatory cytokines, including IL-10 and TGF-β1, displayed an ascending trend post-PLEX. In clinical relevance analysis, among patients who reached MMI, the reductions in MPO, IL-1, and IL-6 exhibited statistically significant differences when compared to patients in the mild-to-no improvement group (P < 0.05). Pearson correlation analysis revealed that the percentage reduction in IL-6 levels after PLEX was positively correlated with the percentage reduction in patient EDSS/VOS scores (r = 0.638, P < 0.05). ConclusionsThis study highlights that reduced levels of NETosis-related regulators after PLEX contribute to clinical improvement, suggesting the potential involvement of NETosis in the acute neurological impairment observed in NMOSD.

Atypical Anti-GBM with ANCA Vasculitis- A Rarest of the Rare Entity: Index Case from Eastern India

Anti-glomerular basement membrane (GBM) antibody glomerulonephritis is an extremely rare glomerular disease. Around 90% of the patients are positive for serum anti-GBM antibodies while up to 10% can be negative. In such patients, only a kidney biopsy can reveal the anti-GBM disease it is then labeled as an atypical anti-GBM disease. Though anti-GBM disease can be associated with Anti Neutrophil Cytoplasmic Antibodies (ANCA) positivity, it is extremely rare to find atypical anti-GBM with ANCA positivity so much so that till now there are very few such cases reported from across the world. The case presented here is one such case where the patient presented with adult-onset nephrotic syndrome features with active urinary sediments and mildly deranged renal function. Myeloperoxidase (MPO) ANCA was positive and it was considered ANCA-associated crescentic glomerulonephritis (GN) but after the renal biopsy the picture was of anti-GBM disease. She was treated with pulse methylprednisolone but her creatinine increased in the meantime and considering anti-GBM she was put on Plasma Exchange (PLEX). She received 5 sessions of PLEX after which her renal function improved. She was also planned for Rituximab which could not be given due to local infection. As there are no protocols for treating such cases because of the extremely rare nature of the presentation, this case will increase the understanding of such presentations for the clinicians. This will help to plan for building the approach for such cases.

Anti-HLA Class II Antibodies Are the Most Resistant to Desensitization in Crossmatch-positive Living-donor Kidney Transplantations: A Patient Series.

In HLA-incompatible kidney transplantation, the efficacy of desensitization in terms of anti-HLA antibody kinetics is not well characterized. We present an overview of the course of anti-HLA antibodies throughout plasma exchange (PE) desensitization in a series of crossmatch-positive patients. All consecutive candidates in the Dutch HLA-incompatible kidney transplantation program between November 2012 and January 2022 were included. The eligibility criteria were a positive crossmatch with a living kidney donor and no options for compatible transplantation. Desensitization consisted of 5-10 PE with low-dose IVIg. A total of 16 patient-donor pairs were included. Patients had median virtual panel-reactive antibody of 99.58%. Cumulative donor-specific anti-HLA antibody (cumDSA) mean fluorescence intensity (MFI) was 31 399 median, and immunodominant DSA (iDSA) MFI was 18 677 for class I and 21 893 for class II. Median anti-HLA antibody MFI response to desensitization was worse in class II as compared with class I (P < 0.001), particularly for HLA-DQ. Class I cumDSA MFI decreased 68% after 4 PE versus 53% in class II. The decrease between the fifth and the 10th PE sessions was modest with 21% in class I versus 9% in class II. Antibody-mediated rejection occurred in 85% of patients, with the iDSA directed to the same mismatched HLA as before desensitization, except for 3 patients, of whom 2 had vigorous rebound of antibodies to repeated mismatches (RMMs). Rebound was highest (86%) in RMM-DSA with prior grafts removed (transplantectomy n = 7), lower (39%) in non-RMM-DSA (n = 30), and lowest (11%) for RMM-DSA with in situ grafts (n = 5; P = 0.018 for RMM-DSA transplantectomy versus RMM-DSA graft in situ). With a median follow-up of 59 mo, 1 patient had died resulting in a death-censored graft survival of 73%. Patients with class II DSA, and particularly those directed against HLA-DQ locus, were difficult to desensitize.

Plasma exchange-sensitive syncytial glomerulopathy in a kidney transplant patient.

Microvascular inflammation (MVI), defined as the presence of glomerulitis and/or peritubular capillaritis, is the key histological lesion of anti-HLA donor-specific antibodies (DSA)-related antibody mediated rejection, but recently other possible mechanisms of MVI have emerged. However, except for peritubular capillary C4d deposition that is more frequently observed in the presence of anti-HLA-DSA, histological features are similar regardless of MVI origin. Therefore, accurately describing patterns of MVI may help differentiate etiologies and drive therapeutic choices. We describe the case of a kidney transplant recipient (primary nephropathy: autosomal dominant polycystic kidney disease) who underwent kidney biopsy for worsening renal function and new onset hypertension. Histologic findings showed severe microvascular inflammation with intense glomerulitis and presence of intracapillary multinucleated cells, positive on immunostaining for endothelial marker ETS-related gene (ERG). Focal intense peritubular capillaritis and early glomerular basement membrane reduplication, C4d negative, were observed, consistent with early chronic active ABMR. HLA-DSA were absent, but high level of anti-angiotensin II type-1 receptor (AT1R) antibodies (Ab) were detected (78 U/L, normal levels < 10 U/L). Two subsequent biopsies showed intense microvascular inflammation with diffuse peritubular capillaritis, and multinucleated, ERG-positive, endothelial cells were still seen in glomerular capillary loops. The patient was started on angiotensin receptor blockers (ARBs) and plasma exchange (PEX) sessions obtaining normalization of blood pressure and AT1R Ab and proteinuria reduction, but, after subsequent liver transplant, rituximab therapy failed to maintain remission and the patient remained PEX-dependent.

Anti-C5 monoclonal antibody treatment showing pathological resolution of complement-mediated atypical hemolytic uremic syndrome: a case report

BackgroundNo reports have shown histological changes before and after anti-C5 monoclonal antibody treatment in patients with atypical hemolytic uremic syndrome (aHUS). Here, we report a rare case of complement-mediated aHUS with a complement factor H (CFH) mutation and anti-CFH antibodies who underwent multiple kidney biopsies.Case presentationA 53-year-old woman developed aHUS with CFH gene mutation [c.3572C > T (p. Ser1191 Leu)] and anti-CFH antibodies. Her father had succumbed to acute kidney injury (AKI) in his 30 s. She exhibited AKI, thrombocytopenia, and hemolytic anemia with schistocytes. After improving the platelet count with one session of plasma exchange, a kidney biopsy was performed one month after the onset of symptoms. Blood vessel thrombosis, obvious endothelial swelling, endocapillary hypercellularity, and subendothelial exudative lesions in the glomeruli and arterioles were detected. Anti-C5 monoclonal antibody treatment with eculizumab immediately improved disease activity. A second biopsy 3 months later revealed marked improvement of endothelial injuries with residual membrane double contours and exudative lesions. A third biopsy at 17 months after gradual improvement of kidney function showed a further decrease of double contours along with alterations of the exudative lesions to fibrous intimal thickening.ConclusionsThis is the first report showing the pathophysiology of aHUS in the kidneys and the efficacy of anti-C5 monoclonal antibody treatment by presenting serial kidney pathological features before and after anti-C5 monoclonal antibody treatment. Since her CFH mutation was considered the most important pathological condition, treatment centered on eculizumab was administered, resulting in a good long-term prognosis. In addition, kidney pathological resolution in aHUS occurred over 1 year after anti-C5 monoclonal antibody treatment.

On-treatment decline in MELD score predicts one-month transplant-free survival in rodenticidal hepatotoxicity patients treated with low-volume plasma exchange.

Plasma exchange (PLEX) improves survival in patients with rodenticidal hepatotoxicity. However, predictors of treatment response are unknown. We aimed at assessing predictors of response to PLEX treatment in these patients. Patients with rodenticidal hepatotoxicity from 2014 to 2023 managed in our department were included in this study. Kochi criteria (model for end-stage liver disease [MELD] score 36 or international normalized ratio [INR] 6 with hepatic encephalopathy[HE]) derived specifically for rodenticidal hepatotoxicity (PubMed IDentifier [PMID]: 26310868) were used to assess need for liver transplantation. We analyzed predictors of survival at onemonth. ∆Bilirubin, ∆MELD score and ∆INR were calculated as percentage change of the parameter after third PLEX session (or after last PLEX if < 3 PLEX sessions done) from baseline pre-PLEX value. Of 200 patients with rodenticidal hepatotoxicity, 114 patients were treated with low-volume PLEX (PLEX-LV). No patient had liver transplantation. Of 78 patients who fulfilled Kochi criteria, 32 patients were PLEX-LV eligible and underwent PLEX-LV (M: 10; age: 20.5, 7-70years; median, range; acute liver failure: 24). Twenty-two (69%; acute liver failure: 14) of the 32 patients were alive at onemonth. Presence of HE (p = 0.03) and ∆MELD (p < 0.001) were significant predictors on univariate analysis, while ∆MELD (aOR = 0.88, 95% CI: 0.79-0.98, p = 0.01) was the only significant independentpredictor of one-month transplant-free survival. Area under receiver operating characteristic (ROC) for ∆MELD was 0.93 (95% CI:0.85-1.00) and a decrease of 20% in MELD score while on PLEX-LV had 90% sensitivity and 90% specificity in predicting one-month survival. Decline in MELD while on PLEX-LV independently predicted one-month transplant-free survival in rodenticidal hepatotoxicity patients. This may help guide decision on stopping PLEX-LV in patients predicted to respond to treatment and to consider alternate treatment options in non-responders.

#3033 IgA vasculitis: a unexpected diagnosis of pulmonary renal syndrome

Abstract Background IgA vasculitis is an inflammatory condition affecting blood vessels that poses a significant diagnostic and therapeutic challenge in clinical practice. Characterized by the deposition of immunoglobulin A (IgA) on vessel walls, this pathology can manifest in various ways, presenting a broad and sometimes complex clinical spectrum. Ninety percent of cases occur in the pediatric age group. Severe lung involvement, such as pulmonary hemorrhage, is rare in patients with IgAV. We present a case of a 33-year-old patient with pulmonary renal syndrome and without purpura, an atypical presentation of IgA vasculitis. Case Report We describe the case of a 33-year-old patient with no relevant medical history or regular medication, who presented to the emergency department in November 2022 with symptoms of hemoptysis, fatigue, myalgias, and pallor. On physical examination, the patient was conscious, cooperative, normotensive, tachycardic, afebrile, with bilateral axillary adenopathies, and scattered crackles on lung auscultation. Laboratory tests revealed anemia with a hemoglobin level of 5.8 g/dL, non-oliguric acute kidney injury with a creatinine level of 2.25 mg/dL, microscopic hematuria (&amp;gt;50/hpf), and proteinuria (UPCR of 2.8 mg/mg and UACR of 1.6 mg/mg). A chest CT scan showed bilateral and scattered ground-glass opacities consistent with alveolar hemorrhage. The patient was admitted to the Nephrology service, underwent 3 sessions of plasma exchange, and started therapy with methylprednisolone 1000 mg for 3 days. A renal biopsy revealed 24 glomeruli, with 3 showing segmental sclerosis and 1 with fibrocellular crescents, mild interstitial nephritis; immunofluorescence showed IgA+++, C3++, IgM, and IgG+- Oxford Classification: M1 E0 S1 T0 C1. Bronchofibroscopy showed a rosy bronchoalveolar lavage and macrophages with hemosiderin pigment deposition. The patient continued with prednisolone at 1 mg/kg. In January 2023 (after two months), there was a &amp;gt;25% reduction in albuminuria with resolution of acute kidney injury. A gradual reduction of prednisolone was initiated, and it was definitively discontinued in October 2023. In the last outpatient visit in January 2024, the patient maintained normal renal function, with no hematuria, UPCR of 100 mg/g, UACR of 70 mg/g, and no further episodes of alveolar hemorrhage. Conclusion This case stands out for its atypical presentation: an adult with alveolar hemorrhage and without other more classical manifestations of IgA vasculitis, such as purpura. This is yet another case that emphasizes the importance of the broad spectrum of diagnoses that a nephrologist should consider in the face of acute kidney injury.

#2725 Chronic kidney disease is a risk factor for hyperchloremic metabolic acidosis after plasma exchange

Abstract Background and Aims Observational studies have demonstrated that metabolic acidosis due to hyperchloremia is an often encountered metabolic disorder following plasma exchange (PE) with an albumin-saline solution. Because of the reduced capacity for acid and chloride excretion, we hypothesized that patients with chronic kidney disease have the highest risk for hyperchloremic metabolic acidosis, in particular when multiple PE sessions are performed in a limited period. Method We prospectively collected data from all patients who received PE at Amsterdam UMC, between February 2023, and January 2024. We collected data on demographics, exchanged plasma volume, replacement fluids, and collected blood for analysis of sodium, chloride and bicarbonate before and after plasma exchange. We selected patients who received ≥5 plasma exchanges in an intensive cycle (≥4.2 sessions/week). We excluded patients who received Omniplasma, intravenous sodium bicarbonate or dialysis during the PE period. We assessed the effect of PE and kidney function on plasma bicarbonate and chloride concentration throughout the PE period with repeated measures mixed ANOVA, corrected for baseline plasma bicarbonate concentration, volume of exchanged plasma and PE intensity. Results Data from 26 patients, corresponding to 130 plasma exchanges, were included in the analysis. The mean age was 56 ± 14 years and 46% was female. Mean eGFR was 45 ± 14 ml/min/1.73 m2. Patients with an eGFR ≤59 ml/min/1.73 m2 (n = 17) had a significantly lower baseline plasma bicarbonate concentration (19.0 ± 2.8 versus 24.0 ± 1.9 mmol/L; p &amp;lt; 0.001) and used oral bicarbonate supplements (29% versus 0%; p = 0.02) more often than patients with an eGFR ≥60 ml/min/1.73 m2 (n = 9). Despite the lower baseline plasma bicarbonate and more frequent use of oral bicarbonate supplements, the plasma bicarbonate levels decreased the most in subjects with an eGFR ≤59 ml/min/1.73 m2 (mean difference −4.2; 95% CI −7.1 to −1.3; p = 0.006; Fig. 1A) during a cycle of 5 PE sessions. Baseline plasma chloride concentration was similar in both groups (103 ± 7 vs 103 ± 4 mmol/L) and increased the most in patients with an eGFR ≤59 ml/min/1.73 m2 (mean difference −1.4; 95% CI −2.2 to 5.0; p = 0.426; Fig. 1B). Conclusion Repeated plasma exchange with albumin-saline as a replacement fluid is associated with a significant decrease in plasma bicarbonate concentration in patients with impaired kidney function. Future studies should determine whether this hyperchloremic metabolic acidosis has impact on the patients’ symptoms or long-term outcome.

Visual Function Improvement after Plasma Exchange Therapy for Acute Optic Neuritis in Neuromyelitis Optica Spectrum Disorders: Case Series and Review.

Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorder (NMOSD) are autoimmune-mediated central nervous system disorders distinguished by the presence of serum aquaporine-4 IgG antibody (AQP4-Ab). The clinical panel comprises severe optic neuritis (ON) and transverse myelitis, which can result in incomplete recovery and a high risk of recurrence. This study aimed to evaluate the visual outcomes of three patients with severe acute ON in NMOSD that was non-responsive to intravenous methylprednisolone (IVMP), who received plasma exchange therapy (PLEX). We included three patients (P1, P2 and P3) with severe acute ON who had no improvement after IVMP treatment and were admitted to the ophthalmology department at the Emergency University Hospital Bucharest from January 2022 to September 2023. All three patients with ON were diagnosed in accordance with the criteria described by the Optic Neuritis Treatment Trial. All the subjects were experiencing their first attack. The mean recruitment age was 35.3 ± 7.71. All patients were seropositive for the AQP4 antibody. All patients were tested for serum myelin oligodendrocyte glycoprotein (MOG) antibody but only one showed a positive test (P3). Lesions visible in orbital MRI indicated the involvement of retrobulbar, canalicular and/or intracranial segments. All three subjects had no response or incomplete remission after an IVMP protocol (5 days of 1000 mg intravenous methylprednisolone in sodium chloride 0.9%). The mean time from onset of optic neuritis to PLEX was 37.6 days. The PLEX treatment protocol comprised five cycles of plasma exchange treatment over 10 days, with a plasma exchange session every other day. An amount of 1 to 1.5 volumes of circulating plasma were dialyzed for 2-4 h. At 1 month after the completion of PLEX therapy, BCVA and VF parameters were improved in all three patients. The treatment of ON remains subject to debate and is somewhat controversial. Plasma exchange must be considered as a rescue therapy when IVMP is insufficient for AQP4-ON patients. This study revealed that PLEX treatment effectively improves the visual outcomes of patients experiencing their first attack of severe acute isolated ON after high-dose IVMP treatment. This study suggests that PLEX may be associated with improved visual outcomes in NMOSD acute optic neuritis.

Hypertriglyceridemia induced acute pancreatitis and efficacy of therapeutic plasma exchange

Acute pancreatitis is a condition in which the pancreas becomes inflamed suddenly within a short period of time. Typically, this condition lasts less than 6 weeks, with most cases resolving within 1-2 weeks. The major causes of acute pancreatitis are ethanol, gallstones, and hypertriglyceridemia. Acute pancreatitis that is caused by hypertriglyceridemia can be severe and life-threatening. Compared to other causes of pancreatitis, studies have shown that the acute pancreatitis induced by hypertriglyceridemia is more serious and can lead to recurrent episodes of pancreatitis, eventually leading to chronic pancreatitis. Here, we report a clinical experience with a patient of high triglyceride induced pancreatitis. He was treated with two sessions of Therapeutic Plasma exchange along with intravenous insulin, statins, and fibrates. In recent times, plasmapheresis has been used to treat severe cases of hypertriglyceridemia-induced acute pancreatitis with success. Early initiation, along with appropriate supportive measures, may lead to favourable outcomes and reduce the risk of complications. However, further research is needed to determine the exact role and timing of plasmapheresis in managing acute pancreatitis caused by hypertriglyceridemia. Bangladesh Crit Care J March 2024; 12 (1): 58-61

Therapeutic apheresis: is it safe in children with kidney disease?

BackgroundTherapeutic 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.MethodsRecords 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.ResultsA 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).ConclusionsOur study showed that complications occurred in only 4.8% of TA sessions. The most common complication was technical problems.Graphical A higher resolution version of the Graphical abstract is available as Supplementary information

Management of Bivalirudin Dosing and Replacement Fluid During Therapeutic Plasma Exchange in Children on Extracorporeal Membrane Oxygenation.

The use of bivalirudin as the primary anticoagulant for children supported on extracorporeal membrane oxygenation (ECMO) is growing. Ideal management of bivalirudin dosing during therapeutic plasma exchange (TPE) on ECMO is unknown. We performed a single-center retrospective study of ECMO patients anticoagulated with bivalirudin who underwent TPE from January 2019 to December 2021. Therapeutic plasma exchange sessions were analyzed individually by bivalirudin dosing strategy (no change [NC] versus increased dose [dose change {DC} bivalirudin group]) and replacement fluid (all fresh-frozen plasma [FFP] versus all albumin or FFP and albumin [FFP/Albumin]). Primary outcomes included bleeding, coagulopathy, and circuit thrombosis within 24 hours of TPE. Secondary outcomes included change in bivalirudin dose and coagulation parameters following TPE. There were 60 unique TPE sessions. Bivalirudin dosing or replacement fluid strategies were not associated with bleeding, coagulopathy, or thrombosis post-TPE. All albumin or fresh frozen plasma and albumin combinations (FFP/Albumin) group had longer post-TPE thromboelastography (TEG) reaction time, clot time, and more acute angle. The FFP/Albumin group had increased post-TPE international normalization ratio (INR) and partial thrombin time (PTT). Therapeutic plasma exchange for children on ECMO and bivalirudin anticoagulation is feasible; however, optimal dosing during TPE requires further investigation. Replacement fluid with FFP/Albumin is associated with more coagulopathic laboratory parameters. Patients may benefit from all FFP fluid replacement strategy. Further investigation is needed to prove generalizability.

Adverse Events and Infectious Complications in the Critically Ill Treated by Plasma Exchange: A Five-Year Multicenter Cohort Study.

The aim of this study was to determine, in critically ill patients treated with therapeutic plasma exchange (TPE), the incidence of adverse events as well as the incidence of secondary infections and its predictive factors. A multicenter retrospective cohort study of an intensive care population treated with TPE to collect adverse events and infectious complications. The characteristics of patients who developed an infection after plasma exchange were compared with those of patients who did not. Four ICUs of French university hospitals. All adults admitted between January 1, 2015, and December 31, 2019, who received at least one plasma exchange session were included. None. A total of 711 TPE sessions were performed on 124 patients. The most frequent TPE indications were thrombotic microangiopathies (n = 32, 26%), myasthenia gravis (n = 25, 20%), and acute polyradiculoneuropathy (n = 12, 10%). Among the 124 patients, 22 (21%) developed arterial hypotension, 12 (12%) fever, and 9 (9%) electrolyte disturbance during TPE. Moreover, 60 (48%) presented at least one infectious complication: ventilator-associated pneumonia 42, pneumonia 13, bacteremia 18 (of which 6 catheter-related infections) viral reactivation 14. Independent risk factors for ICU-acquired infection were the ICU length of stay (24 vs. 7 d; hazard ratio [HR]: 1.09 [1.04-1.15], p < 0.001) and invasive mechanical ventilation (92% vs. 35%; HR: 16.2 [5.0-53.0], p < 0.001). In critically ill patients treated with TPE, adverse events occurring during the procedure remain moderately frequent and are mostly not life-threatening. Infectious complications, mainly ventilation-associated pneumonia, are frequent in this population. The need of mechanical ventilation and longer ICU stay is associated with an increased risk of infection.

Caplacizumab for immune thrombotic thrombocytopenic purpura: real-world multicenter data.

Given the limited real-world data of caplacizumab, our multicenter real-world study was designed to assess the safety and efficacy of caplacizumab in immune thrombotic thrombocytopenic pupura (iTTP), compared to historic controls. We have studied 70 patients: 23 in the caplacizumab and 47 in the historic control group. Plasma exchange was applied in all episodes except for two patients that denied plasma exchange. Rituximab as first-line treatment was more common in the caplacizumab group compared to historic control. Caplacizumab (10 mg daily) was given at a median on day 7 (1-43) from initial diagnosis for 32 (6-47) dosages. In the caplacizumab group, a median of 12 (8-23) patients required plasma exchange sessions versus 14 (6-32) in the control group. Caplacizumab administration did not produce any grade 3 complications or major hemorrhagic events. After a median of 19.0 (2.6-320) months since the iTTP diagnosis, 5 deaths occurred (4 in the control group and 1 in the caplacizumab group, p = 0.310). Caplacizumab patients achieved early platelet normalization and ADAMTS13 activity normalization at the end of treatment. Relapse was observed only in 2/23 (9%) caplacizumab patients, compared to 29/47 (62%) historic controls (p < 0.001). Overall, caplacizumab is safe and effective in treating iTTP, including cases refractory to plasma exchange, re-administration, and cases without previous plasma exchange treatment. No major hemorrhagic events were observed. Cessation of dosing guided by ADAMTS13 has ensured a low relapse rate.

Mixed cryoglobulinaemia vasculitis secondary to marginal zone lymphoma in a patient with end-stage renal failure on haemodialysis

Cryoglobulinaemia vasculitis can present with a variety of symptoms and there is limited data on the incidence and presentation of cryoglobulinaemia vasculitis in haemodialysis patients. We report a case of a 63-year-old male who had a series of presentations with rash, visual changes, abdominal pain, weight loss, fevers and digital ischaemia. This is on a background of a congenital single kidney with end-stage renal failure secondary to diabetes and hypertension, receiving haemodialysis for nearly 5 years. He initially experienced a leukocytoclastic vasculitis rash confirmed on skin biopsy, followed by multiple hospital presentations for undifferentiated abdominal pain and fever of unknown source. Jejunal biopsy revealed intestinal vasculitis. His peripheral blood flow cytometry and bone marrow biopsy were consistent with marginal zone lymphoma (indolent subtype, IgM kappa clone). Further testing revealed a type II cryoglobulinaemia consisting of an IgM kappa monoclonal band with polyclonal IgG (cryocrit 5%). A diagnosis of cryoglobulinaemia vasculitis was established and he was treated with pulsed methylprednisolone and rituximab therapy. However, after receiving three doses of rituximab the patient developed a presumed vasculitis-associated pulmonary haemorrhage for which he received treatment with five sessions of plasma exchange. His symptoms resolved and cryocrit reduced to < 1% after his final dose of rituximab. The clinical features of cryoglobulinaemia may be difficult to detect in chronic haemodialysis patients and vigilance is required.

Therapeutic Plasma Exchange among Myasthenia Gravis Patients Attended at Referral Neurosciences Hospital in Bangladesh: A Single Centre Experience

Background: Therapeutic Plasma Exchange (TPE) is an effective therapeutic procedure for treating Myasthenia Gravis (MG). Objectives: The aim of study was to evaluate demographics, indications, adverse reactions and outcome of therapeutic plasma exchange in myasthenia gravis patients. Methodology: This prospective observational study was conducted in the Department of Transfusion Medicine at National Institute of Neurosciences and Hospital (NINS&amp;H), Dhaka, Bangladesh from August 2014 to June 2022. All patients with Myasthenia Gravis who received therapeutic plasma exchange as a treatment during hospitalization were included in this study. Data were systematically recorded and these were variables of demographics, clinical indications, numbers of sessions, volume of exchanged plasma, clinical responses and complications during or after the procedure. Results: A total number of 72 patients had undergone 275 sessions of therapeutic plasma exchange among which 52(72.2%) cases were male and the mean age was 39.5±13.11 years. For each patient the number of therapeutic plasma exchange session and volume of plasma exchange were 3.69±1.016 (Range 2 to 7) and 2266.7±361.53 ml (Range 1400 to 3100) respectively. Anti-acetylcholine receptor antibody (Anti-ACR Ab) was positive in 44(61.1%) cases. Myasthenic crisis (n=45, 62.5%) was the most common indication of therapeutic plasma exchange followed by preoperative preparation for thymectomy (n=16, 22.2%) and worsening of myasthenic weakness (n=11, 15.3%). In 275 sessions of therapeutic plasma exchange, overall incidence of adverse reaction was in 26(9.45%) cases. Mild allergy (n=9, 3.27%) and pyrexia (n=5, 1.82%) were most commonly reported. Hypotension was reported in 4(1.46%) cases. Reactions were mild and were reversed by bed side managements. Response to treatment was observed in 80.5% (n=58) patients. 2(2.8%) patients of Myasthenia Gravis died; but death was unrelated to therapeutic plasma exchange. Conclusion: Therapeutic plasma exchange is rapidly effective therapy for myasthenic crisis, progressive myasthenia gravis and prior to thymectomy operation and it has adequate safety profile. Journal of National Institute of Neurosciences Bangladesh, January 2023;9(1):16-23

Establishment of an adverse effect prevention protocol on plasma exchange using fresh frozen plasma prior to ABO-incompatible living donor kidney transplantation at our hospital.

Simple plasma exchange (PE) with fresh-frozen plasma replacement allows antibody removal for ABO-incompatible living donor kidney transplantation, but is associated with a high incidence of allergic reactions. We developed, implemented, and evaluated a protocol for safe preoperative PE. The protocol comprised pretreatment (125 mg methylprednisolone infusion, 400 mg acetaminophen and 30 mg diphenhydramine orally) with a replacement fluid rate < 20 mL/min. Allergic reaction incidence was investigated in controls who underwent ABO-incompatible living donor kidney transplantation between 2016 and March 2020 (group C) and patients who underwent the protocol and procedure between April 2020 and February 2023 (group N). Ten (group C) and 19 (group N) patients performed 11 and 30 sessions of PE, respectively. Allergic reactions occurred in 81.8% and 36.7% (p = 0.014), respectively, with an odds ratio of the protocol was 0.056 (95% CI 0.0059-0.5380, P = 0.013). Our protocol resulted in a significantly lower incidence of allergic reactions.

Rituximab administration one week before ABO-incompatible liver transplantation due to drug-induced acute liver failure with hepatic coma: a case report.

In cases of acute liver failure (ALF) with hepatic coma, early liver transplantation, including ABO-incompatible (ABOi) living donor liver transplantation (LDLT), should be considered. The ABO antibody barrier can be reduced using plasma exchange (PE) and the anti-CD20 antibody rituximab. Plasma exchange is also performed for drug-induced ALF and is effective for desensitization. Rituximab treatment usually requires 14days. There is presently no established desensitization protocol for ABOi-LDLT for ALF. Here, we report a case of drug-induced ALF with hepatic coma, which was treated with ABOi-LDLT using PE and rituximab 8days prior to surgery. A 33-year-old female, with a history of headaches for which she was taking analgesics daily, developed drug-induced ALF with hepatic coma. Her ABOi sister desired to become a liver donor. We initiated desensitization using rituximab (500mg) and mycophenolate mofetil (MMF, 2000mg/day), followed by five sessions of PE. Eight days after rituximab administration, ABOi-LDLT with splenectomy was performed. Postoperatively, the patient received local infusion via portal vein for 14days and immunosuppression with tacrolimus, methylprednisolone, and MMF. No episode of cellular or antibody-mediated rejection (AMR) was observed. The patient was discharged uneventfully 56days after ABOi-LDLT with no problems up to 15months after the transplant.

#3242 OUTCOMES OF THERAPEUTIC PLASMA EXCHANGE: A ONE-YEAR EXPERIENCE IN AN INTERNAL MEDICINE INTENSIVE CARE UNIT

Abstract Background and Aims There is uncertainty regarding the best indication, device type, frequency, length of time, kind of replacement fluid, and criteria for ceasing therapeutic plasma exchange (TPE) for various disorders. TPE is an intrusive technique that has the potential to save lives, but it also has a risk of unfavorable outcomes and complications, necessitating close supervision by professional teams. In the intensive care unit (ICU), there are three categories of indications for TPE: (1) absolute, well-established, and evidence-based, for which TPE is recognized as first-line therapy; (2) relative; for which TPE is a recognized second-line treatment (alone or in combination) and (3) rescue therapy; for which TPE is used with a limited or theoretical evidence base. The purpose of this study is to continue our evaluation of the outcome of nephrology care in internal medicine ICU regarding bedside membrane therapeutic plasma exchange. Method In the continuity of our previous work in the analysis of the outcome of TPE. From August 2021 to August 2022, 425 membrane plasma exchange sessions were performed on 69 patients in the internal medicine department's ICU-Cairo University Hospitals-Egypt. Results Closely similar to what we found before; the most frequent diagnosis was thrombotic microangiopathies (24 (34.8%) patients) with complete recovery seen in 18/24 patients (75%); followed by systemic vasculitis with pulmonary renal involvement (14 (20.3%) patients) with partial remission was seen in 9/14 patients (64.3%); Guillain-Barré syndrome (13 (18.8%) patients) with complete recovery was seen in 8/13 patients (61.5%); Proliferative Lupus Nephritis with crescents [10 (14.5%) patients] with partial remission in 8/10 patients (80%), and myasthenia gravis [8 (11.6%) patients] with complete recovery in 6/8 patients (75%). Hypotension occurred during 69/425 (16.2%) sessions and hypocalcemia in 8/69 (11.5%) patients during treatment. Infection of vascular access occurred in 4/69 (5.8%) patients. 6/69 (8.7%) patients died during their stay in ICU none during treatment with TPE, all of them were diagnosed to have severe sepsis as a cause of death. Conclusion According to our experience, membrane therapeutic plasma exchange is a successful and secure treatment for a variety of illnesses including thrombotic microangiopathies, systemic vasculitis, myasthenia gravis, proliferative lupus nephritis, and Guillain-Barré syndrome. The main reason for death was sepsis.