Use Of Plasmapheresis Research Articles

5128 Role of Plasmapharesis- A Tale of Two Thyroid Storms

Abstract Disclosure: Z. Zhang: None. S. Joseph: None. A.M. Kodali: None. D. Eagerton: None. Background: Thyroid storm is an acute life threatening endocrinological emergency. The standard treatment for a thyroid storm is antithyroid medication, iodine, beta blockers and corticosteroids. However, when patients are refractory to standard treatment, plasmapheresis may be an effective and safe intervention. Case 1: A 49-year-old male with typical symptoms of hyperthyroidism, presented with a Ventricular tachycardia arrest. After the return of spontaneous circulation, he was febrile, and developed new onset atrial fibrillation (AF) and Amiodarone drip was started. Physical exam showed a thin gentleman with an enlarged thyroid. ECHO showed LVEF 50% with moderate to severe dilatation of left atrium and mild mitral and tricuspid valve regurgitation. CT abdomen/pelvis and CTA chest did not show any pathology. Labs showed TSH of 0.02 (reference range: 0.465-4.68); Free T 4 >6.99 (reference range: 0.78- 2.19). He was started on Propylthiouracil (PTU), Hydrocortisone, Potassium Iodide, and Cholestyramine. His blood pressure was too low to tolerate a beta blocker. He developed increased liver enzymes thought to be due to shocked liver. PTU was switched to Methimazole due to less risk of hepatotoxicity. A Thyroid Stimulating immunoglobulin was 13.40 (reference range 0.00-0.55), confirming Graves’ disease. He developed multiorgan failure and plasmapheresis was initiated. His Free T4 level prior to his first apheresis was still >6.99 but improved to 6.20 after two days. After the second session, free T4 level drastically improved to 2.65. He sustained a level of 2.09 until his hospital discharge. Case 2: A 59-year-old male with untreated hyperthyroidism and AF, who presented with shortness of breath. He was tachycardic, febrile and developed AF with rapid ventricular response. A TSH and free T4 level showed <0.02 (reference range: 0.465-4.68); and >6.99 (reference range: 0.78- 2.19), respectively. Physical exam shows a cachectic man with an enlarged thyroid. ECHO showed reduced ejection fraction of 35% with biventricular failure. He was initially started on PTU, Potassium Iodide, hydrocortisone and esmolol drip and eventually Cholestyramine. Patient developed multisystem organ failure and PTU was switched to methimazole due to continued deterioration of his liver enzymes. Plasmapheresis started with a total of 3 sessions. His free T4 improved from 4.32 to 3.59 by the second session. Round three was performed when his Free T4 increased back to 4.56. Unfortunately, he had a sudden decline in mentation and CT head showed an acute/subacute left anterior parietal stroke and after family discussion, was palliatively extubated and expired. Discussion: This case series shows two cases of thyroid storm treated with plasmapheresis and had two stark contrasts. Further studies should be done to elucidate the usefulness of plasmapheresis for thyroid storm when refractory to standard of treatment. Presentation: 6/2/2024

#2875 Experience in the use of plasmapheresis as a therapeutic tool in acute pathologies in a tertiary level hospital

Abstract Background and Aims Apheresis is a therapeutic modality which purpose is the elimination from plasma of the pathogenic components of a disease or its clinical manifestations. Plasmapheresis is an apheresis technique used in the treatment of various diseases with varying degrees of proven clinical efficacy. The aim of our work is to describe the experience in the use of plasmapheresis in different acute pathologies in our center, and if there are factors that favor the clinical response to treatment. Method Retrospective cohort study. We included plasmapheresis performed in the years 2017-2022. Variables such as age, sex, etiology, type of replacement performed, number of sessions, and American Society for Apheresis (ASFA) classification, among others, were collected. We defined response to treatment as partial or total clinical remission. Results We included 72 patients who received plasmapheresis during 2017-2022. Mean age 54 ± 18 years. 56.9% male. Disease etiology was neurological in 56.9%, nephrological in 36.1% and hematological in 6.9%. Between 2017-2019, 39.7% of the plasmapheresis were performed, while the remaining 60.3% were between 2020-2022. The number of sessions was 5.3 ± 2. 59.6% were performed with albumin, 29.2% with fresh frozen plasma and 13.9% with albumin and fresh frozen plasma. The ASFA indication was category I in 68.1%. 23.6% presented some complication, the most frequent being febrile in 6.9%, systemic infection and allergic reaction in 5.6%. A response to treatment was observed in 75%. Antibodies were positive before starting plasmapheresis in 29.2%, decreasing to 16.7% 3 months after treatment. There was recurrence in 19.4%, 16.7% received more cycles of plasmapheresis and there was 11.1% mortality due to disease. In patients with response to treatment, a higher percentage of response was observed in those diseases of non-nephrological origin (70.4% vs. 29.6%, p 0.04), lower percentage of complications (16.7% vs. 83.3%, p 0.01) and lower mortality associated with the disease (3.7% vs. 96.3%, p 0.001). Conclusion Plasmapheresis is a therapeutic plasma exchange technique that is increasingly used in our hospital, with a greater increase in recent years. It is a therapeutic option that should be considered in many occasions as first line or adjuvant treatment, with a low rate of complications and clinical improvement in most patients. Despite this, studies with a larger number of patients are required to increase and consolidate the existing evidence on the use of these procedures.

Evaluation of the Factors Influencing Mortality in Patients with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Multicenter Study of 166 Patients.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening acute mucocutaneous disorders usually triggered by drugs. In this study, we aimed to evaluate the factors affecting mortality in patients with SJS-TEN. Our study is a retrospective cohort study, analyzing data collected from a total of 12 tertiary care centers between April 2012 and April 2022. The study included 59 males and 107 females, a total of 166 patients, with an average age of 50.91 ± 21.25years. Disease classification was TEN in 50% of cases, SJS in 33.1%, and SJS-TEN overlap in 16.9%. The average SCORTEN within the first 24h was 2.44 ± 1.42. Supportive care was provided to 99.4% of patients. The most commonly used systemic immunomodulatory treatments were systemic steroids (84.3%), IVIG (intravenous immunoglobulin) (49.3%), and cyclosporine (38.6%). Plasmapheresis was administered to five patients. While 66.3% of patients were discharged, 24.1% resulted in exitus. Our comparative analysis of survivors and deceased patients found no effect of systemic steroids, IVIG, and cyclosporine treatments on mortality. Univariate analysis revealed that the SCORTEN scores on days 1 and 3 as well as the rates of detachment at the onset and during follow-up were significantly higher in deceased patients compared to survivors. The rates of fever, positive blood cultures, and systemic antibiotic use were higher in deceased patients compared to survivors. The presence of comorbidities, diabetes, and malignancy were significantly more common in deceased patients. Multivariate regression analysis indicated that over SCORTEN 2, the mortality risk exponentially rose with each SCORTEN increment, culminating in an 84-fold increase in mortality at SCORTEN 5-6 (odds ratio [95% confidence interval]: 13.902-507.537, p < 0.001) compared to SCORTEN 0-1. Additionally, the utilization of plasmapheresis was associated with a 22-fold increase in mortality (odds ratio [95% confidence interval]: 1.96-247.2, p = 0.012). Our study found that a high SCORTEN score within the first 24h and the use of plasmapheresis were related to increased mortality, while systemic steroids, IVIG, and cyclosporine treatments had no impact on mortality. We believe that data gathered from one of the most comprehensive studies which we conducted on SJS-TEN will enrich the literature, although additional research is warranted.

Plasmapheresis in post-COVID-19 myelitis: A case report.

Previous studies have delineated different neurological manifestations associated with coronavirus disease 2019 (COVID-19). Myelitis is identified as a rare neurological complication resulting from a COVID-19 infection. Limited information is available regarding the treatment of patients experiencing this condition. This report extracts data from the medical record of a post-COVID-19 myelitis patient at Buriram Hospital and follows up prospectively on the patient's symptoms after treatment. A 61-year-old man, previously vaccinated for COVID-19 and with a history of hypertension and dyslipidemia, experienced progressive bilateral lower-extremity weakness (recorded as muscle strength grade 2/5 in both lower extremities) for 6 weeks. He had a mild case of COVID-19 2 months earlier, which resolved in 10 days without specific treatment. However, 2 weeks after being diagnosed with COVID-19, he developed weakness in his lower limbs, numbness below the nipple, and urinary retention. Spinal magnetic resonance imaging revealed multifocal longitudinal myelitis. Despite initial treatment with methylprednisolone, the patient showed no clinical improvement. Consequently, he underwent five cycles of plasmapheresis. Three months after discharge, a notable improvement was observed, with his muscle strength graded at 4/5 in both lower extremities and the resolution of sensory and urinary symptoms. We presented the case of a COVID-19-vaccinated patient, in whom COVID-19 infection might have led to myelitis. We found promising results in treating prolonged COVID-19-related myelitis symptoms through the use of plasmapheresis.

Impact of therapeutic plasmapheresis on the duration of organ failure in patients with hypertriglyceridemia-associated acute pancreatitis

BackgroundPlasmapheresis is widely used for severe hypertriglyceridemia-associated acute pancreatitis (HTG-AP) to remove excessive triglycerides from plasma. This study aimed to evaluate whether plasmapheresis could improve the duration of organ failure in HTG-AP patients.MethodsWe analyzed a cohort of patients from a multicenter, prospective, long-running registry (the PERFORM) collecting HTG-AP patients admitted to the study sites within 72 h from the onset of symptoms. This study was based on data collected from November 2020 to March 2023. Patients who had organ failure at enrollment were involved in the analyses. The primary outcome was time to organ failure resolution within 14 days. Multivariable Cox regression model was used to evaluate the association between plasmapheresis and time to organ failure resolution. Directed acyclic graph (DAG) was used to identify potential confounders.ResultsA total of 122 HTG-AP patients were included (median [IQR] sequential organ failure assessment (SOFA) score at enrollment, 3.00 [2.00–4.00]). Among the study patients, 46 underwent plasmapheresis, and 76 received medical treatment. The DAG revealed that baseline serum triglyceride, APACHE II score, respiratory failure, cardiovascular failure, and renal failure were potential confounders. After adjusting for the selected confounders, there was no significant difference in time to organ failure resolution between patients undergoing plasmapheresis and those receiving exclusive medical treatment (HR = 1.07; 95%CI 0.68–1.68; P = 0.777). Moreover, the use of plasmapheresis was associated with higher ICU requirements (97.8% [45/46] vs. 65.8% [50/76]; OR, 19.33; 95%CI 2.20 to 169.81; P = 0.008).ConclusionsIn HTG-AP patients with early organ failure, plasmapheresis was not associated with accelerated organ failure resolution compared to medical treatment but may be associated with more ICU admissions.Trial registration: The PERFORM study was registered in the Chinese Clinical Trial Registry (ChiCTR2000039541). Registered 30 October 2020.

Use of plasmapheresis to lower anti-AAV antibodies in nonhuman primates with pre-existing immunity to AAVrh74

Patients with pre-existing immunity to adeno-associated virus (AAV) are currently unable to receive systemic gene transfer therapies. In this nonhuman primate study, we investigated the impact of immunosuppression strategies on gene transfer therapy safety and efficacy and analyzed plasmapheresis as a potential pretreatment for circumvention of pre-existing immunity or redosing. In part 1, animals received delandistrogene moxeparvovec (SRP-9001), an AAVrh74-based gene transfer therapy for Duchenne muscular dystrophy. Cohort 1 (control, n= 2) received no immunosuppression; cohorts 2-4 (n= 3 per cohort) received prednisone at different time points; and cohort 5 (n= 3) received rituximab, sirolimus, and prednisone before and after dosing. In part 2, cohorts 2-4 underwent plasmapheresis before redosing; cohort 5 was redosed without plasmapheresis. We analyzed safety, immune response (humoral and cell-mediated responses and complement activation), and vector genome distribution. After 2 or 3 plasmapheresis exchanges, circulating anti-AAVrh74 antibodies were reduced, and animals were redosed. Plasmapheresis was well tolerated, with no abnormal clinical or immunological observations. Cohort 5 (redosed with high anti-AAVrh74 antibody titers) had hypersensitivity reactions, which were controlled with treatment. These findings suggest that plasmapheresis is a safe and effective method to reduce anti-AAV antibody levels in nonhuman primates prior to gene transfer therapy. The results may inform human studies involving redosing or circumvention of pre-existing immunity.

Use of Plasmapheresis in Heparin Induced Thrombocytopenia in Patients Undergoing Urgent Cardiac Surgery

Abstract Heparin Induced Thrombocytopenia (HIT) is a potentially life-threatening but uncommon disease process characterized by IgG antibodies that recognize platelet factor 4/heparin immune complexes causing thrombocytopenia and thrombosis. Although therapeutic plasmapheresis (PLEX) appears to be effective, protocols regarding how and when to institute PLEX remain elusive. At our institution, we put together a standardized approach to assess the interventional success across patients that included 1 plasmapheresis procedure per day over 4 days, processing one plasma volume and using thawed plasma as the replacement fluid. Treatment effectiveness was evaluated by pre- and post-interventional HIPA OD levels and platelet count. Treatment aim was to decrease the HIPA OD to less than 0.6 and obtain a normal platelet count. The aim of this study was to design a protocol for PLEX to address urgent need for patients with HIT for which heparin is planned for intraoperative cardiac surgery requiring cardiopulmonary bypass (CPB) anticoagulation. We retrospectively examined the medical records of 2 HIT-positive patients who underwent PLEX prior to cardiac surgery at our large quaternary care hospital. Both patients had a 4Ts score of 5, with a medium probability of disease. One patient's HIT was confirmed by a positive serotonin release assay (SRA). The second patient had persistently negative confirmatory test but was deemed clinical HIT. The first patient is a 53-year-old female, who was admitted for mitral valve replacement, aortic valve replacement, and tricuspid valve repair. She was confirmed HIT by positive SRA. Three sessions of PLEX were performed on consecutive days. Her HIPA (heparin induced platelet antibody) O.D. value decreased from 1.925 to 0.534 and platelet counts normalized. The second patient is a 73-year-old male, who was admitted for LVAD implantation and LV thrombus resection. Two sessions of PLEX were performed on consecutive days. His HIPA O.D. value deceased from 1.261 to &amp;lt; 0.4 (negative) and platelet counts normalized. For both patients, the last PLEX procedure was performed the day of surgery, and the cardiac surgical procedure was completed successfully. Based on these 2 patients, we have proposed a protocol for PLEX in HIT patients as follows: daily plasmapheresis x 4 days will be performed until the OD has decreased to less than 0.6 and platelet count has normalized. Each plasmapheresis will consist of the processing of one plasma volume, using thawed plasma as the replacement fluid. The last plasmapheresis will occur on the day of surgery prior to surgery. Further assessment will be performed if the targeted parameters have not been met by the 4th plasmapheresis procedure. In conclusion, we present 2 patients with HIT who benefitted from PLEX prior to cardiac surgery under CPB. We will adopt the protocol used for these 2 patients as our standard of practice moving forward.

THU654 The Use Of Plasmapheresis In A Severe Case Of Amiodarone Induced Thyrotoxicosis

Abstract Disclosure: T. Moazezi: None. C. Koh: None. Thyroid storm is an endocrine emergency that results in a hypermetabolic state due to excess levels of circulating thyroid hormone and carries a high mortality rate. Therapeutic plasma exchange (TPE) can be used in cases of thyrotoxicosis refractory to medical management to achieve hemodynamic and clinical stability. We present a case of a 74-year-old male with severe amiodarone-induced thyrotoxicosis who presented with altered mentation and ultimately required intubation and intensive care for management of thyroid storm. Standard medical therapy for treatment was initiated immediately with antithyroid drugs, beta blockers, glucocorticoids, potassium iodide solution, and cholestyramine. However, the patient remained unresponsive and intubated with worsening biochemical parameters with increased total T3 levels and sustained elevated levels of free T4 even after five days of maximal medical therapy. Due to the lack of a clinical and biochemical response to conventional medical therapy, the patient was started on plasmapheresis and underwent a total of seven cycles of plasmapheresis over a period of ten days. He significantly improved with TPE and was successfully bridged to a total thyroidectomy, which was completed without complications. Our case demonstrated a decrease in total T3 concentration of roughly 45% after one session of plasmapheresis and roughly 76% with seven sessions of plasmapheresis by the time of thyroidectomy. Free T4 levels remained elevated above reference range until the day after the fifth session of plasmapheresis. This case is important because the literature surrounding the use of TPE in thyroid storm is sparse, and there have only been case reports and retrospective studies published on the subject. Ultimately, while TPE is not conventionally used as part of routine treatment for thyroid storm, endocrinologists should keep this tool at the forefront of their management strategies to deliver quicker treatment to critically ill patients and prevent complications of thyroid storm. Nonetheless, more research is needed to delineate concrete guidelines and specific protocols for use of TPE in thyroid storm. Presentation: Thursday, June 15, 2023

Severe Hypertriglyceridemia: A 10-Year Review in a Portuguese Hospital.

Severe hypertriglyceridemia (SHTG) is a rare condition associated with serious complications, such as acute pancreatitis (AP), and the best treatment is still a matter of discussion. The aim of this study is to outline the demographics, management, and outcomes (recurrence and mortality) of complications in patients with SHTG. A retrospective, observational, and analytical study was carried outby obtaining clinical data from the electronic health records of patients with SHTG admitted to the Internal and Intensive Medicine units from the 1st of January 2009 to the 31st of December 2020 in a university hospital. The cohort included 17 patients. The most common complication was AP (13/17 = 76.5%). Admission to the intensive care unit (ICU) was observed in 84.2%. Among patients with AP, the most commonly administered therapies were insulin (82.4%) and fibrates (76.5%). Plasmapheresis was used in 58.8%, and the criteria for using this technique were mainly based on clinical and laboratory abnormalities. There were no deaths. The readmission rate at 30 days was 36.3%. This study shows the morbidity profile associated with SHTG, with a high level of ICU admissions and also a high level of the use of plasmapheresis. In our population, this approach had good results, and this should be highlightedas there are no clear international guidelines for this intervention. Distinguishing between patients with familial chylomicronemia syndrome or with multifactorial chylomicronemia is important as recent specific therapy for lipoprotein lipase (LPL) genetic deficit is available. In the near future, the performance of a genetic study should be considered in patients with SHTG as an attempt to avoid the high recurrence rate of complications of this disease.

#6663 PRIMARY SJöGREN SYNDROME WITH BIOPSY PROVEN RENAL INVOLVEMENT: A SINGLE CENTER EXPERIENCE

Abstract Background and Aims Primary Sjögren's syndrome (pSS) is a chronic systemic autoimmune disease, characterized by inflammation and destruction of the exocrine glands and the involvement of multiple organs. A wide variety of kidney manifestations associated with pSS have been described. The prevalence of kidney involvement is not clear. Our aim was to describe the renal involvement of patients with pSS diagnosed by kidney biopsy (KB). We also analyzed the clinical manifestations, laboratory and immunological characteristics, clinical outcomes and treatments received in patients with pSS referred to a specialized kidney centre. Method Observational, retrospective study of adult patients (age &amp;gt; 18 years) diagnosed with pSS (EULAR criteria) referred and treated at a kidney referral centre (Fundació Puigvert) in Barcelona, Spain. We collected data from the clinical records registry including demographic variables, laboratory parameters, biopsy results and adverse outcomes, defined by the need of renal replacement therapy (RTT) and/or death. Absolute frequencies, percentages, means and standard deviations were used for statistical analysis. Multivariate analysis was performed as appropriate in SPSS V28.0 Results A total of 27 patients with pSS underwent KB from January 1994 to July 2022; all patients were female, with a median age of 58.4 years (SD ±12.4); 85% were Caucasian. The mean baseline glomerular filtration rate (eGFR) was 65.9 mL/min (SD ±16) (before nephrologist referral), with 8 of 27 patients (29.6%) having eGFR less than 60 mL/min at baseline. The main indication for nephrological evaluation was acute kidney injury (AKI) (63%), followed by the presence of non-nephrotic proteinuria with dysmorphic haematuria (29.6%). The most common finding in KB was acute interstitial nephritis (AIN) (55.6%), as an isolated AIN kidney lesion in twelve patients (44.4%). Mild interstitial nephritis was noted in the context of a predominant glomerular lesion in three other patients (one membranoproliferative glomerulonephritis, one IgA nephropathy and one AA amyloidosis). Nine patients (33.3%) had glomerular lesion (see Table 1). In 18.5% of patients, the diagnosis of pSS was made after the renal biopsy. The percentage of patients receiving ACEI/ARB was 77.8%. A total of 25 patients received some type of immunosuppression (IS). Corticosteroids being the most frequently used (77.8%), followed by the combination IS treatment (44%) and rituximab (33.3%). At the time of the KB, the mean eGFR was 46.3 mL/min (SD ±24), compared to eFGR of 45.2 mL/min (SD ±22) one-year after KB. During follow-up, seven patients (25.9%) required RRT and three (11.1%) died from non-renal causes, mainly by infections associated with immunosuppression. Factors associated with adverse renal outcomes were AKI (p = 0.01), baseline eGFR CKD-EPI less than 60 mL/min (p = 0.005), presence of anti-Ro60 antibodies (p = 0.046), use of plasmapheresis (p = 0.013), use of cyclophosphamide (p = 0.013) and the presence of tubulointerstitial atrophy with glomerular sclerosis on kidney biopsy (p = 0.010). Conclusion Our study included a larger cohort than previously reported before in a single centre. In our cohort of patients with pSS who were evaluated by KB, AIN was the leading cause of renal involvement, as has been seen in other studies. The presence of eGFR below 60 mil/min at baseline and the finding of chronicity in the KB are associated with an adverse outcome, as well as is the presence of AKI. Early referral to nephrologist may be important for prognosis, and KB is necessary for accurate diagnosis of kidney involvement to allow targeted treatment.

Efficacy of plasmapheresis in neutropenic patients suffering from cytokine storm because of severe COVID-19 infection.

With the emergence of the coronavirus disease 2019 (COVID-19) and inability of healthcare systems to control the disease, various therapeutic theories with controversial responses have been proposed. Plasmapheresis was administered as a medication. However, the knowledge of its efficacy and indications is inadequate. This study evaluated the use of plasmapheresis in critically ill patients with cancer. This randomized clinical trial was conducted on 86 patients with malignancies, including a control group (N=41) and an intervention group (N=45) with severe COVID-19 during 2020-21. Both groups were treated with routine medications for COVID-19 management according to national guidelines, and plasmapheresis was applied to the intervention group. C-reactive protein (CRP), D-dimer, ferritin, lactate dehydrogenase, hemoglobin, and white blood cell, polymorphonuclear, lymphocyte, and platelet levels were measured at admission and at the end of plasmapheresis. Other variables included neutrophil recovery, intensive care unit admission, intubation requirements, length of hospital stay, and hospitalization outcomes. CRP(P<0.001), D-dimer (P<0.001), ferritin (P=0.039), and hemoglobin (P=0.006) levels were significantly different between the groups after the intervention. Neutrophil recovery was remarkably higher in the case than in the control group (P<0.001). However, plasmapheresis did not affect the length of hospital stay (P=0.076), which could have significantly increased survival rates (P<0.001). Based on the study findings, plasmapheresis led to a significant improvement in laboratory markers and survival rate in patients with severe COVID-19. These findings reinforce the value of plasmapheresis in cancer patients as a critical population suffering from neutropenia and insufficient immune responses.

Results of Surgical Treatment of Elderly and Senile Patients With Acute Ischemia of the Lower Extremities of Embologenic Origin

BACKGROUND Today, one of the urgent problems of emergency vascular surgery is the high mortality rate in acute ischemia of the lower extremities in elderly and senile patients.AIM OF STUDY To evaluate the incidence of complications in elderly and senile patients with acute ischemia of the lower extremities of embologenic origin.MATERIAL AND METHODS As an object of study, three groups of elderly and senile patients admitted with a clinical picture of acute ischemia of the lower extremities were studied. Group I — patients who underwent reconstructive interventions in the scope of the so-called proximal reconstruction; group II — patients who, for the purpose of complete revascularization, underwent extended reconstructive interventions using an additional surgical approach; group III — patients who underwent a “classic” embolectomy.RESULTS Good results were statistically significantly more common in group II. Satisfactory and poor results were observed in group III. Amputations were statistically significantly more common in group III. In 69.6% of cases, the use of plasmapheresis and infusion therapy prevented the development of acute renal injury.CONCLUSION Performing extended arterial reconstructions, including using an additional surgical approach, makes it possible to more fully restore the main blood flow in the ischemic limb and thereby completely restore the main blood flow in the ischemic limb and reduce the degree of ischemia, as well as reduce the severity of the manifestation and course of the post-ischemic syndrome.

Clinical efficacy and safety of efgartigimod for treatment of myasthenia gravis.

Treatment of acute exacerbations and refractory myasthenia gravis (MG) remains challenging despite advances in immunotherapy. Frequent use of plasmapheresis and immunoglobulinsare associated with adverse events and strain on resources. The neonatal Fc receptor (FcRn) facilitates IgG recycling and FcRn antagonism enhances the degradation of IgG pathogenic autoantibodies without compromising adaptive and innate immunity. Efgartigimod, anFcRN antagonist, has been shown in well-designed clinical trials to improve clinical status and reduce autoantibody levels without significant safety concerns. Efgartigimod has received approvals for use in the United States, Japan and Europe. It is plausible that efgartigimod is effective across different subgroups and varied spectrums of MG severity. Novel strategies involving FcRn modulationand long-term follow-up studies will help provide further insights and expand the therapeutic repertoire.

Pregnancy-induced severe hypertriglyceridemia effectively treated with therapeutic plasmapheresis

IntroductionHypertriglyceridemia is associated with significant morbidity during pregnancy. Hypertriglyceridemia-induced pancreatitis (HTGP) is associated with genetically determined dyslipidemia or a secondary condition such as diabetes, alcohol, pregnancy, or medication use. The lack of data on the safety of drugs to be used to decrease triglyceride levels during pregnancy dictates that other strategies must be chosen. Patient and methodsWe describe a case of a pregnant woman with severe hypertriglyceridemia treated with two different techniques of plasmapheresis (Dual Filtration apheresis and Centrifugal Plasma Separation). ResultsThe patient could be treated throughout the pregnancy, with good control of the triglycerides, and a healthy baby was born. ConclusionHypertriglyceridemia is a major issue during pregnancy. The use of plasmapheresis is a safe and efficient tool in that clinical scenario.

An inquiry into the treatment of sepsis using plasma exchange therapy: A systematic review and meta-analysis.

Sepsis is a potentially lethal condition that occurs when the body's response to infection damages tissue and organs. The production of inflammatory mediators typically assists in defending the body against infection; however, an overreaction to inflammation can cause coagulation problems, vascular endothelial damage, and organ hypoperfusion. Blood purification methods, such as plasmapheresis, can effectively remove inflammatory mediators from plasma. The purpose of this meta-analysis was to explore the efficacy of plasma exchange for sepsis treatment as noted in recent studies. The authors searched the Pubmed (Medline), Cochrane Central Register of Controlled Trials (The Cochrane Library), Embase (Ovid), and Scopus databases and included controlled clinical studies that compared plasmapheresis or plasma filtration with conventional treatment in patients with severe sepsis. The Newcastle-Ottawa Scale literature quality assessment tool was used to assess the risk of bias. The primary study outcome was all-cause mortality. The random effects model was adopted for conducting the meta-analysis. Among the 1013 records found, the study included 5 trials, all of which carried a low risk of bias. The use of plasmapheresis was associated with a longer stay in the intensive care unit (odds ratio [OR], 0.85, 95% confidence interval [CI], 0.39-1.32, heterogeneity [I2 ]=0%), a significant reduction in all-cause mortality (OR, 0.54, 95% CI, 0.33-0.89, I2 =70%), and reduced mortality (OR, 0.29, 95% CI, 0.13-0.67, I2 =0%) in adults; the results for children differed from this (OR, 0.79, 95% CI, 0.36-1.72, I2 =89%). Four trials reported no adverse events; one trial reported an adverse event related to plasma exchange, including an instance of hypotension in one patient. Plasmapheresis appeared to be an effective treatment for patients suffering from sepsis. A large number of additional randomised controlled trials are needed to confirm this finding.

Preventive plasmapheresis for rituximab related flare in cryoglobulinemic vasculitis

IntroductionRituximab monotherapy represents the main therapeutic option for cryoglobulinemic vasculitis (CV) with severe organ involvement. However, initial worsening of the CV, known as rituximab-associated CV flare (=CV flare), has been described and are associated with high mortality rates. The aim of the present study is to evaluate the outcomes of plasmapheresis initiated before or during rituximab treatment, as prevention of CV flare. MethodsWe conducted a retrospecttive study in our tertiary referral center from 2001 to 2020. We have included all patients with CV receiving rituximab and divided them in two groups whether they had flare prevention by plasmapheresis or not. We evaluated rituximab-related CV flare incidence in both groups. CV flare was defined as the onset of a new organ involvement or worsening of the initial manifestations within 4 weeks following rituximab. ResultsAmong the 71 patients included, 44 received rituximab without plasmapheresis (control = CT cohort) and 27 received plasmapheresis before or during rituximab treatment (preventive plasmapheresis = PP cohort). PP was given to patients thought to have a high risk of CV flare, with significantly more severe diseases than patients in the CT cohort. Despite this, no CV flare was observed in the PP group. In the other hand, 5 flares occurred in the CT cohort. ConclusionOur results show that plasmapheresis is efficient and well tolerated to prevent rituximab-associated CV flare. We believe that our data support the use of plasmapheresis in this indication, especially in patients with high risk of CV flare.

LBODP104 Thyroid Plasmapheresis: An Alternative Option For Thyroid Storm

Abstract Introduction Thyroid storm is a rare, life-threatening condition with a high mortality rate approaching 10-30%. The mainstay of treatment includes initiation of therapy directed against the thyroid, supportive intensive care, and treatment of any precipitating factors. We report three cases of patients with thyroid storm and successful therapeutic use of plasmapheresis when traditional therapy was contraindicated. Clinical Cases Case 1 A 22-year-old male with no known past medical history, presented with fever, shortness of breath, and syncope. Labs showed TSH 0. 014 mIU/L (range 0.4-4.5), FT4 4.30 ng/dL (range 0.9-2.2), TSI 546% (range &amp;lt;140), and TPO 20 U/mL (range &amp;lt;60). He was diagnosed with thyroid storm due to Graves’ disease (Burch-Wartofsky score of 60). He was initially treated with propylthiouracil (PTU), hydrocortisone, propranolol, and Lugol's iodine solution. He developed transaminitis and shock liver so was not able to continue PTU or trial methimazole. He underwent 4 rounds of plasmapheresis with improvement in thyroid hormone levels and eventually had thyroidectomy. Case 2 A 37-year-old female with known Graves’ disease (non-adherent to antithyroid drug), presented with shortness of breath and palpitations. Labs showed TSH &amp;lt;0. 01 mIU/L (range 0.4-4.5), FT4 4.83 ng/dL (range 0.9-2.2), and FT3 6. 0 pg/mL (range 2.3-4.2). Burch-Wartofsky score was 40 and supported the diagnosis of thyroid storm. She was initially treated with methimazole, hydrocortisone, propranolol, and Lugol's iodine solution. She developed transaminitis and there was also concern for methimazole-induced insulin autoimmune syndrome, so methimazole was discontinued. She underwent 3 rounds of plasmapheresis with improvement in thyroid hormone levels and eventually had thyroidectomy. Case 3 A 31-year-old female with no known past medical history, presented with shortness of breath and altered mental status requiring intubation. Labs showed TSH &amp;lt;0. 01 mIU/L (range 0.4-4.5), FT4 4.65 ng/dL (range 0.9-2.2), FT3 25.2 pg/mL (range 2.3-4.2), TSI 467% (range &amp;lt;140), TPO 375 U/mL (range &amp;lt;60). She was diagnosed with thyroid storm due to Graves’ disease (Burch-Wartofsky score of 90). She was initially treated with methimazole, hydrocortisone, propranolol, and Lugol's iodine solution. She developed pulmonary alveolar hemorrhage and it was unclear if this was due to methimazole, thus methimazole was discontinued. She underwent 5 rounds of plasmapheresis with improvement in thyroid hormone levels and eventually had thyroidectomy. Conclusion Plasmapheresis can be an effective and safe treatment option in thyroid storm when there are contraindications for antithyroid drugs or when rapid normalization of thyroid hormone levels is needed. It should be considered as a stabilizing measure as it leads to marked improvement of thyrotoxicosis within 3-5 days, allowing thyroidectomy for definitive therapy. Presentation: No date and time listed

Risk of tuberculosis among renal transplant recipients receiving rituximab therapy.

Rituximab is an anti-CD 20 agent used widely in renal transplant recipients. Its use is associated with various infections; however, its association with tuberculosis (TB) is not well established and has not been studied in post renal transplantation patients. This is a single-center, retrospective analysis of 56 renal transplant recipients who received rituximab as a part of desensitization protocol or as rescue therapy for rejections and 287 post-renal transplant patients who did not receive rituximab during the study period from January 2013 to June 2017. The association between use of rituximab and occurance of TB was studied. Other factors associated with TB were also investigated. Baseline characteristics were similar in both the groups. Mean time for occurrence of TB was 18.4 ± 10.6months after renal transplantation. Rituximab use was not significantly associated with TB or any other infection. Higher number of rejection episodes (60% vs. 32.72%, p = .029) was the only factor associated with greater incidence of TB. However, no specific type of rejection was associated with TB. Use of plasmapheresis in post-transplant period for treatment of humoral rejections was associated with significantly higher incidence of TB (33.33% vs. 13.41%, p = .031); however, when pre-transplant plasmapheresis was also considered, there was no significant difference. The choice of induction agent was not associated with higher incidence of TB. Use of rituximab is not associated with higher incidence of TB when compared to other immunosuppressive agents. Routine screening and prophylaxis may not be advisable, especially in a country like India with high prevalence of TB, as it will further delay transplantation and may adversely affect the outcome of the patients.

PLASMAPHERESIS, A USEFUL TOOL FOR BETTER OUTCOME OF PATIENTS UNDERGOING THYMECTOMY FOR MYASTHENIA GRAVIS

Background: Thymectomy for widespread Myasthenia gravis may be associated with a turbulent postoperative course especially if preoperative circulating acetylcholine receptor autoantibody levels are towering. Plasmapheresis remove these antibodies. The aim of this study was to explore the results of preoperative Plamapheresis on postoperative course after thymectomy. Material And Method This is a retrospective study at tertiary care center where 13 patients of myasthenia gravis were operated in 3 year duration (2017-2020). All patients had undergone 2 cycles of plasmapheresis before surgery. Result was evaluated postoperatively in ICU, at the time of discharge, and at follow up after 7 days, 1 month and 6 month in terms of weaning from ventilator, ICU stay, level of autoantibodies and length of hospital stay, improvement in symptoms, and mortality. Results Out of 13 patients 5 (38.46%) were male and 8 (61.54%) were female in the age range of 20 to 60 years. 6 (46.15%) were in stage IIA and 7 (53.85%) in stage IIB. Preoperative autoantibody levels were in the range of 30-50 nmol/ml. Almost total thymectomy was done in all patients. Patients were extubated with in 4-6 hrs after surgery and there was no mortality. Patients were discharged at postoperative day 4-7. Conclusion The study shows that preoperative use of plasmapheresis to reduce the autoantibody level is associated with an early extubation, early discharge and less complication after thymectomy.

HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS SECONDARY TO DISSEMINATED HISTOPLASMOSIS PRESENTING AS DIFFUSE ALVEOLAR HEMORRHAGE IN A 32-YEAR-OLD WOMAN

HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS SECONDARY TO DISSEMINATED HISTOPLASMOSIS PRESENTING AS DIFFUSE ALVEOLAR HEMORRHAGE IN A 32-YEAR-OLD WOMAN