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1.
Ann Intensive Care ; 14(1): 117, 2024 Jul 29.
Article in English | MEDLINE | ID: mdl-39073720

ABSTRACT

BACKGROUND: Therapeutic plasma exchanges (TPE), which affect the humoral response, are often performed in combination with immunosuppressive drugs. For this reason, TPE may be associated with an increased susceptibility to infections. We aimed to describe blood stream infection (BSI) incidence in ICU patients treated with TPE and to identify associated risk factors. METHODS: We retrospectively included patients that had received at least one session of TPE in the ICU of one of the 4 participating centers (all in Paris, France) between January 1st 2010 and December 31th 2019. Patients presenting with a BSI during ICU stay were compared to patients without such an infection. Risk factors for BSI were identified by a multivariate logistic regression model. RESULTS: Over 10 years in the 4 ICUs, 387 patients were included, with a median of 5 [2-7] TPE sessions per patient. Most frequent indications for TPE were thrombotic microangiopathy (47%), central nervous system inflammatory disorders (11%), hyperviscosity syndrome (11%) and ANCA associated vasculitis (8.5%). Thirty-one patients (8%) presented with a BSI during their ICU stay, a median of 7 [3-11] days after start of TPE. In a multivariate logistic regression model, diabetes (OR 3.32 [1.21-8.32]) and total number of TPE sessions (OR 1.14 [1.08-1.20]) were independent risk factors for BSI. There was no difference between TPE catheter infection related BSI (n = 11 (35%)) and other sources of BSI (n = 20 (65%)) regarding catheter insertion site (p = 0.458) or rate of TPE catheter related deep vein thrombosis (p = 0.601). ICU course was severe in patients presenting with BSI when compared to patients without BSI, with higher need for mechanical ventilation (45% vs 18%, p = 0.001), renal replacement therapy (42% vs 20%, p = 0.011), vasopressors (32% vs 12%, p = 0.004) and a higher mortality (19% vs 5%, p = 0.010). CONCLUSION: Blood stream infections are frequent in patients receiving TPE in the ICU, and are associated with a severe ICU course. Vigilant monitoring is crucial particularly for patients receiving a high number of TPE sessions.

2.
Turk J Med Sci ; 54(3): 508-516, 2024.
Article in English | MEDLINE | ID: mdl-39050002

ABSTRACT

Background/aim: Therapeutic plasma exchange (TPE) is an extracorporeal treatment method that removes large molecular weight substances from plasma. In our study, we aimed to retrospectively examine the indications and procedural methods of the patients who had undergone TPE, and the complications that occurred during the procedure. Materials and methods: Forty-one patients who were monitored in thePICU of Gazi Yasargil Training and Research Hospital and had indications for TPE between 2017 and 2021 were included in the study. Laboratory parameters were checked before and after the TPE procedure. In addition to these, patients' diagnosis, weight, type of procedure and type of device, where the procedure was performed, duration of the procedure, amount of blood and plasma processed, complications, number of procedures, and death during the procedure or independent of the procedure were evaluated. Results: The median age was 93.0 (14.0-167.0) months. Hemolytic uremic syndrome (HUS) was the most common TPE indication with nine patients. The most common complication related to TPE was fever (11 patients), while no complication was observed in 18 patients.When laboratory results were evaluated according to American Society for Apheresis (ASFA) categories, a significant improvement was observed in the values of platelet, AST, ALT, LDH, urea, and creatinine in ASFA1 after TPE. No significant improvement was observed in ASFA2 (p > 0.05). In ASFA3, a significant improvement was observed in INR, AST, ALT, LDH, total bilirubin, creatinine, pH, and lactate values after TPE (p < 0.05). Five patients died from ASFA1, one from ASFA2, and three patients from ASFA3. Conclusion: Since significant adjustments are observed in clinical and laboratory values in sepsis-MOF, which is in the ASFA3 category, we believe that it should be evaluated in the ASFA2 or ASFA1 category in the early treatment of these diseases. In addition, we think that MIS-C cases, which have not been in any category according to ASFA, should be included in the ASFA2 or ASFA3 category, considering our TPE results.


Subject(s)
Intensive Care Units, Pediatric , Plasmapheresis , Humans , Plasmapheresis/methods , Female , Child , Male , Retrospective Studies , Child, Preschool , Infant , Adolescent , Hemolytic-Uremic Syndrome/therapy , Treatment Outcome
3.
Ann Blood ; 92024 Jun 30.
Article in English | MEDLINE | ID: mdl-39049905

ABSTRACT

Background and Objective: Thrombotic thrombocytopenic purpura (TTP) is a rare but debilitating thrombotic microangiopathy that results from severe deficiency of the enzyme ADAMTS13. The disorder was first described in the early 20th century, but the pathophysiology of the disease has only been elucidated in the past three decades. In this narrative review, we will summarize the milestone moments in the history of TTP research and discovery. Methods: We searched literature using PubMed from 1924 to 2023 using the following free text searches: "thrombotic thrombocytopenic purpura", "Moschcowitz disease", and "thrombotic microangiopathy". We found 6,917 peer-reviewed articles and sorted through these for relevant literature pertinent to the review. A total of 46 articles were included for review and the remainder were excluded. Key Content and Findings: The history of TTP research was reviewed, with a sampling of major events in the evolution of the understanding of the pathophysiology and treatment of the disease discussed here. There remains much to be learned about the nature of the disease in order to develop more specific and less harmful treatments. Conclusions: An overview of the major discoveries that have led to our current understanding of TTP reveals the results of collaboration of multiple groups of physicians and scientists through the past century, with additional breakthroughs likely to occur in the future because of that same collaborative spirit.

4.
Ther Apher Dial ; 2024 Jul 25.
Article in English | MEDLINE | ID: mdl-39053908

ABSTRACT

INTRODUCTION: Autoimmune neurological diseases (ANDs) involve the immune system attacking the nervous system, leading to various symptoms. Therapeutic plasma exchange (TPE) is used to remove pathogenic autoantibodies, aiming to improve clinical outcomes. METHODS: This ambispective observational study included 99 patients with ANDs who underwent TPE from January 2018 to June 2022 at a tertiary care center in India. Clinical outcomes were measured using the modified Rankin Scale (mRS) scores at admission, post-TPE, at 3-months, 6-months, and 1-year follow-up post-discharge. Data were analyzed using Epi Info version 7.0. RESULTS: The median mRS score improved significantly from 5 (IQR 4-5) before TPE to 3 (IQR 2-4) post-TPE (p < 0.001). Complications occurred in 5.95% of procedures, with allergic reactions being the most common. The in-hospital mortality rate was 9%. CONCLUSION: TPE is a safe and effective treatment modality for autoimmune neurological diseases, especially in resource-constrained settings. It aids in both symptomatic relief and reducing long-term functional disability.

5.
J Family Med Prim Care ; 13(6): 2503-2506, 2024 Jun.
Article in English | MEDLINE | ID: mdl-39027862

ABSTRACT

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease present with the classic pentad of microangiopathic hemolytic anemia (MAHA), fever, neurologic changes, thrombocytopenia, and renal dysfunction. In a diagnostic dilemma, therapeutic plasma exchange (TPE) is a choice of life-saving intervention. In this, we assess the efficacy of TPE in a suspected case of post-partum TTP. A 27 years old female was admitted in an emergency on day 8 after a lower segment cesarian section (LSCS) with unresponsive behavior for 3 days and with TTP. She was normal 32 days back with her second, 7-month pregnancy. Ultrasonography (USG) showed an umbilical cord around the neck of the baby. On the fifth post-operative day, she was shifted to emergency with fever, generalized anasarca, gastrointestinal tract (GI) bleeding, low platelet count, and low Hb, with a poor Glasgow coma scale (GCS) of 6. On the bases of serum urea and serum creatinine, she presented acute kidney injury with encephalopathy. At emergency, she was unresponsive to mechanical ventilation and supportive treatment; hence, therapeutic plasma exchange was performed. After eight TPE cycles, the patient presented with an improved hematological and renal profile with good GCS. TPE is helpful and life-saving for suspected TTP patients with AKI.

6.
Acta Neurol Belg ; 2024 Jul 24.
Article in English | MEDLINE | ID: mdl-39044118

ABSTRACT

PURPOSE: Therapeutic plasma exchange (PLEX) is effective as a second-line treatment of severe relapses of multiple sclerosis (MS) that failed to respond to standard steroid therapy. Our objective was to evaluate the effectiveness of PLEX in the severe MS relapses in a cohort of patients treated at Neurology Clinic, University Clinical Centre of Serbia, Belgrade, from 2007 until 2020. METHODS: This retrospective study comprised 107 MS patients with 127 severe relapses treated with PLEX. Majority of our patients suffered from relapsing remitting MS (83.2%), 12.1% had secondary progressive MS and 4.7% had primary progressive MS. Mean age was 39.2 years (range, 19-79 years), female/male ratio 2.3:1. Pulse corticosteroid treatment was used before PLEX in 99.3% of patients. Median EDSS score at nadire during relapse was 6.0 (range 2.0-10.0). After PLEX, 73.8% relapses showed a marked clinical improvement, 7.1% showed mild improvement and in 19.0% there was no improvement. Median EDSS at discharge was 4.0 (6.0 at nadir of relapse vs. 4.0 at discharge; p<0.0001) and it was sustained at the same level, 6 month after PLEX. Multivariate regression analysis showed that higher EDSS at nadir during relapse (OR=0.63, 95% CI 0.41-0.96, p=0.039) and older age (OR=1.07, 95% CI 1.02- 1.12, p=0.010) were significantly associated with poor treatment response after 6- month follow-up. Adverse events occurred in 17.3 of procedures and they were completely resolved. CONCLUSION: Our study in a large cohort of MS patients confirmed that PLEX is effective.

7.
Transfus Apher Sci ; 63(4): 103960, 2024 Jun 14.
Article in English | MEDLINE | ID: mdl-38885577

ABSTRACT

Brown recluse spider bites can lead to severe reactions such as skin necrosis,hemolytic anemia, and multiorgan failure, which can be life-threatening. Therapeutic plasma exchange has been reported to provide clinical benefit for such cases. In thisreport, we present a case of a brown recluse spider bite that was successfully treated with therapeutic plasma exchange and compare it with previous case reports.

8.
Ir J Med Sci ; 2024 Jun 03.
Article in English | MEDLINE | ID: mdl-38831241

ABSTRACT

BACKGROUND AND AIM: The use of therapeutic plasma exchange (TPE) for treatment of hypertriglyceridemia-induced acute pancreatitis (HTGP) remains controversial in the literature. This study compared the clinical outcomes of TPE versus conventional therapy in patients with HTGP. METHODS: Fifty-five patients with HTGP were included. Patients were retrospectively compared in pairs: those who received TPE treatment and those who did not, those whose triglyceride level fell below 500 mg/dL within 48 h, and those who did not, those with and without persistent organ failure. The primary outcome was the percentage of triglyceride reduction within 48 h. Secondary outcomes were the length of hospital stay, mortality, cost-effectiveness, and persistent organ failure. RESULTS: Percentage decrease in triglyceride levels, medical hospitalization costs, and length of hospital stay were higher in the TPE group compared to the non-TPE group (p < 0.05, for each). However, there was no difference regarding persistent organ failure and mortality (p > 0.05, for each). The length of hospital stay, average cost, persistent organ failure, and mortality were similar in both groups whose triglyceride level fell below 500 mg/dL within 48 h and those who did not (p > 0.05, for each). Among patients with persistent organ failure, average cost was higher in the TPE group compared to the non-TPE group (p < 0.05). An independent relation was found between the average cost and persistent organ failure, TPE, length of hospital stay, albumin, and urea values in all patients (p < 0.05, for each). CONCLUSIONS: The approach of using TPE for treatment of HTGP was not found to be superior to the conventional treatment. Randomized controlled studies with larger number of patients are needed to gain better understanding of this issue.

9.
Ther Apher Dial ; 2024 Jun 25.
Article in English | MEDLINE | ID: mdl-38924694

ABSTRACT

INTRODUCTION: Therapeutic plasma exchange (TPE) may involve complications. We aimed to review the demographic data, indications, technical information, and complications. METHODS: Data for TPE procedures (TPEPs) performed between 2004 and 2018 were retrospectively. RESULTS: This study covered 2505 TPEPs performed on 338 patients; 55% of them were female (n = 186), and the median age was 36 years (range, 11-93 years). Most TPEPs were administered for hematological (40.6%) indications. The incidence of complications on the first procedure was 3.2% (n = 80); only 16 procedures (0.6%) were failed. The complication incidence was 19.8% (n = 497), with 789 total complications. Most of the complications were patient-related (90.4%), and the most of them were urticaria (29.1%), occlusion (3.2%), and faulty systems (1.01%), respectively. The use of only fresh frozen plasma as replacement fluid caused a higher complication rate (22.1%, p < 0.01). CONCLUSION: The number of TPEPs is increasing every day. Hematologic indications for TPE and the use of fresh frozen plasma may increase the risk of complications.

11.
Transfus Apher Sci ; 63(4): 103958, 2024 Jun 05.
Article in English | MEDLINE | ID: mdl-38880037

ABSTRACT

Amlodipine poisoning is a nightmare for treating clinicians because of the intractable hypotension and bradycardia induced by the drug, which requires a balanced treatment algorithm. We encountered a case of severe Amlodipine toxicity (450 mg) who presented with complaints of nausea, multiple episodes of vomiting, and chest discomfort. On arrival at the EMD, the patient had significant hypotension (80/46 mmHg), bradycardia (40 beats/min), and a fall in oxygen saturation (75 %). He was symptomatically managed with inotropes, IV calcium, IV fluids, and oxygen supplementation. We decided to go forward with Therapeutic Plasma Exchange (TPE) in an attempt to remove the inciting agent. Two sessions of TPE were performed and the patient showed significant improvement post-procedure which led to the discharge of the patient within 10 days of admission. This case report highlights the noteworthiness of TPE in treating significantly high doses of drug poisoning.

12.
13.
Metab Brain Dis ; 39(5): 985-1004, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38842660

ABSTRACT

Neurodegeneration, known as the progressive loss of neurons in terms of their structure and function, is the principal pathophysiological change found in the majority of brain-related disorders. Ageing has been considered the most well-established risk factor in most common neurodegenerative diseases, such as Parkinson's disease (PD) and Alzheimer's disease (AD). There is currently no effective treatment or cure for these diseases; the approved therapeutic options to date are only for palliative care. Ageing and neurodegenerative diseases are closely intertwined; reversing the aspects of brain ageing could theoretically mitigate age-related neurodegeneration. Ever since the regenerative properties of young blood on aged tissues came to light, substantial efforts have been focused on identifying and characterizing the circulating factors in the young and old systemic milieu that may attenuate or accentuate brain ageing and neurodegeneration. Later studies discovered the superiority of old plasma dilution in tissue rejuvenation, which is achieved through a molecular reset of the systemic proteome. These findings supported the use of therapeutic blood exchange for the treatment of degenerative diseases in older individuals. The first objective of this article is to explore the rejuvenating properties of blood-based therapies in the ageing brains and their therapeutic effects on AD. Then, we also look into the clinical applications, various limitations, and challenges associated with blood-based therapies for AD patients.


Subject(s)
Aging , Neurodegenerative Diseases , Humans , Neurodegenerative Diseases/therapy , Neurodegenerative Diseases/metabolism , Aging/physiology , Animals , Brain/metabolism , Brain/pathology , Alzheimer Disease/therapy , Alzheimer Disease/metabolism
14.
Hematol Oncol ; 42(4): e3293, 2024 07.
Article in English | MEDLINE | ID: mdl-38872511

ABSTRACT

Current treatment guidelines of myeloma cast nephropathy (MCN) recommend the institution of plasma cell-directed therapy and consideration of therapeutic plasma exchange (TPE), with the goal of rapid reduction of the serum free light chain (sFLC). However, the role of TPE continues to remain a subject of debate. The goal of this retrospective bi-institutional study was to evaluate the clinical outcomes of TPE in combination with systemic therapy. Eighty patients were included in this analysis, of whom 72.5% had ≥50% drop in their initial involved sFLC. At 3 months from TPE initiation, the overall hematologic response rate (ORR) was 67.5% with a very good partial response or better (≥VGPR) rate of 40%. At 6 months, ORR was 57.5%, with ≥VGPR rate of 49%. The renal response rate at 3 and 6 months was 47.5% and 43.75%, respectively; the overall renal response rate was 48.75%. On multivariable analysis, every one unit increase in baseline creatinine (odds ratio [OR] 0.76, p = 0.006), and achievement of ≥VGPR (OR 21.7 p < 0.0001) were significantly associated with renal response. Also, a ≥50% drop in sFLC was favorably associated with renal response (OR 3.39, p = 0.09). With a median follow-up of 36.4 months, the median overall survival (OS) was 11 months. On multivariable analysis, achievement of renal response (hazard ratio [HR] 0.3, p < 0.0001) and newly diagnosed disease (NDMM; HR 0.43, p = 0.0055) were associated with improved OS. Among NDMM patients, those treated with daratumumab-based regimens had a trend for better OS (p = 0.15), compared to other regimens, but the difference was not significant. At the end of follow-up, an estimated 40.4% of patients who were on dialysis were able to become dialysis independent. In conclusion, our study highlights the poor survival of patients with MCN. Achievement of early renal response is crucial for prolonged OS, with daratumumab-based therapies showing promise.


Subject(s)
Multiple Myeloma , Plasma Exchange , Humans , Multiple Myeloma/therapy , Multiple Myeloma/mortality , Male , Female , Plasma Exchange/methods , Middle Aged , Aged , Retrospective Studies , Treatment Outcome , Adult , Aged, 80 and over , Kidney Diseases/therapy , Kidney Diseases/etiology
15.
Cureus ; 16(5): e60947, 2024 May.
Article in English | MEDLINE | ID: mdl-38910774

ABSTRACT

Sepsis is a life-threatening condition that occurs when the body's immune response to infection becomes unregulated, causing organ dysfunction and a heightened risk of mortality. Despite increased awareness campaigns, its prevalence escalates, annually afflicting over 1.7 million adults in the United States. This research explores the potential of therapeutic plasma exchange (TPE) in septic shock management, aiming to highlight its capacity to improve patient outcomes and reduce mortality. Adhering to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines, our comprehensive search across 51,534 studies, using keywords such as plasmapheresis, plasma exchange therapy, therapeutic plasma exchange, septic shock, and reduction in mortality integrated with medical subject headings terms, led to the meticulous selection of six pivotal studies. Through rigorous evaluation with tools such as the revised Cochrane Risk-of-Bias tool, Newcastle-Ottawa Scale, and Assessment of Methodological Quality of Systematic Reviews, we extracted strong evidence supporting TPE's significant impact on decreasing mortality in septic shock patients compared to standard care, as demonstrated in three randomized controlled trials and one cohort study, with an odds ratio (OR) of 0.43 (95% confidence interval (CI) = 0.26-0.72). Additionally, two meta-analyses further validate TPE's effectiveness, showing a mortality reduction with an OR of 0.30 (95% CI = 0.20-0.46). This advantage also extends to critically ill COVID-19 patients, underscoring TPE's crucial role in modulating the coagulation cascade, decreasing sepsis-related complications, and reducing the risk of bleeding and organ failure. Nevertheless, the benefits of TPE must be carefully balanced against potential risks such as hypocalcemia, hypotension, and citrate toxicity, especially in patients with underlying renal or liver issues, emphasizing the importance of shared decision-making. While TPE emerges as a promising therapy, its formal integration into standard care protocols awaits further confirmation, highlighting the critical need for more in-depth research to conclusively determine its efficacy and safety in septic shock management.

16.
Ther Apher Dial ; 2024 May 15.
Article in English | MEDLINE | ID: mdl-38751182

ABSTRACT

INTRODUCTION: Therapeutic plasma exchange (TPE), an effective method to eliminate harmful soluble mediators associated with tissue injury, serves as a crucial intervention for systemic rheumatologic diseases (SRDs). However, its value in critically ill SRDs remains uncertain. This retrospective study aims to evaluate the efficacy of TPE in SRDs. METHODS: Critically ill SRD patients admitted to the department of intensive care unit of a large tertiary hospital receiving TPE from January 2011 to December 2019 were included. RESULTS: A total of 91 critically ill SRD patients received TPE were enrolled. Their mean age was 47.67 ± 16.35 years with a female predominance (n = 68). Significant decrease in SOFA score post-TPE treatment was observed (p < 0.05). There were no TPE-related fatalities. Improvement was observed in 64 (70.32%) patients. CONCLUSION: This study shows favorable clinical outcomes. TPE may be an acceptable treatment option for critically ill SRD patients.

17.
Ther Apher Dial ; 2024 May 15.
Article in English | MEDLINE | ID: mdl-38747186

ABSTRACT

INTRODUCTION: Therapeutic plasma exchange (TPE) is used in a wide spectrum of diseases in critically ill pediatric patients. We aim to review the indications, complications, safety, and outcomes of critically ill children who received TPE. METHODS: All of the TPE procedures performed in a pediatric intensive care unit providing tertiary care during 19 years (January 2013-January 2023) were evaluated retrospectively. A total of 154 patients underwent 486 TPE sessions. RESULTS: Median age was 6 years (2-12.5) and 35 children had a body weight of <10 kg (22.7%). Number of organ failure was 4 (2-6). Liver diseases were the most common indication for TPE (31.2%) followed by sepsis with multiorgan dysfunction syndrome (27.3%). Overall survival rate was 72.7%. The highest mortality was observed in hemophagocytic lymphohistiocytosis group. Non-survivors had significantly higher number of organ failure (p < 0.001), higher PRISM score (p < 0.001), and higher PELOD score on admission (p < 0.001). Adverse events were observed in 68 (13.9%) sessions. Hypotension (7.8%) and hypocalcemia (5.1%) were the most frequent adverse events. CONCLUSION: TPE is safe for critically ill pediatric patients with experienced staff. Survival rate may vary depending on the underlying disease. Survival decreases with the increase in the number of failed organs.

18.
Cureus ; 16(5): e60019, 2024 May.
Article in English | MEDLINE | ID: mdl-38736760

ABSTRACT

We present the case of a female who developed cerebral venous thrombosis with thrombocytopenia after inoculation with the anti-coronavirus disease 2019 (COVID-19) Vaxzevria vaccine, followed by splanchnic thrombosis and diffuse hemorrhages. Despite receiving treatment, the complications increased, and hence therapeutic plasma exchange (TPE) was attempted, leading to laboratory and clinical improvements and discharge after a period of intensive care. Almost two years after the first episode, in the interim of which the patient complained of only minor symptoms such as asthenia and difficulty concentrating, she developed an epileptic syndrome that required neurological treatment. In addition, her fatigue and difficulty concentrating worsened and other serious symptoms of dysautonomia appeared, such as trembling of her right arm, loss of stability, and postural orthostatic tachycardia. As serum analysis revealed a significant number of alterations in autoantibodies against various G-protein-coupled receptors (GPCRs) and RAS-related proteins, two further TPEs were performed, resulting in rapid and sustained clinical improvement. This report highlights the role of the different types of autoantibodies produced in response to anti-COVID-19 vaccination, which can have functional, regulatory, and possibly pathogenic effects on the vascular and nervous systems.

19.
Int Immunopharmacol ; 134: 112178, 2024 Jun 15.
Article in English | MEDLINE | ID: mdl-38728883

ABSTRACT

BACKGROUND: Thrombotic microangiopathy (TMA) is associated with carfilzomib, and knowledge of carfilzomib-induced TMA is based mainly on case reports. This study investigated the clinical characteristics of patients with carfilzomib-induced TMA and provided a reference for the rational use of carfilzomib. METHODS: Reports of carfilzomib-induced TMA were collected for retrospective analysis by searching the Chinese and English databases from inception to January 31, 2024. RESULTS: Sixty-six patients were included, with a median age of 63 years (range 39, 85). The median time to onset of TMA was 42 days (range 1, 1825) from initial administration, and the median number of cycles was 3 cycles (range 1, 15). Hemolytic anemia was recorded in 64 patients, with a median of 8.3 g/dL (range 4.6, 13). Sixty-three patients had thrombocytopenia with a median of 18 × 109/L (range 1, 139). The median value of increased LDH was 1192 IU/L (range 141, 5378). ADAMTS13 activity was normal in 41 (62.1 %) of the 42 patients. Mutations were found in 9 (13.6 %) of the 15 patients. Fifty-seven patients achieved a clinical response after discontinuing carfilzomib and receiving therapeutic plasma exchange (53.0 %), eculizumab (24.2 %), or hemodialysis (39.4 %). CONCLUSION: Carfilzomib-induced TMA is an important adverse event that should be considered in patients receiving carfilzomib for multiple myeloma with anemia, thrombocytopenia, and acute kidney injury. Withdrawal of carfilzomib and treatment with eculizumab have proven successful in some patients.


Subject(s)
Oligopeptides , Thrombotic Microangiopathies , Humans , Thrombotic Microangiopathies/chemically induced , Male , Middle Aged , Female , Oligopeptides/adverse effects , Oligopeptides/therapeutic use , Aged , Adult , Retrospective Studies , Aged, 80 and over , Treatment Outcome , ADAMTS13 Protein/genetics , Multiple Myeloma/drug therapy , Antibodies, Monoclonal, Humanized/adverse effects , Antibodies, Monoclonal, Humanized/therapeutic use , Thrombocytopenia/chemically induced
20.
Eur J Pediatr ; 183(8): 3289-3297, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38717620

ABSTRACT

Patients with acute liver failure (ALF) and acute on chronic liver failure (ACLF) have significant morbidity and mortality. They require extracorporeal blood purification modalities like continuous renal replacement therapy (CRRT) and therapeutic plasma exchange (TPE) as a bridge to recovery or liver transplantation. Limited data are available on the outcomes of patients treated with these therapies. This is a retrospective single-center study of 23 patients from 2015 to 2022 with ALF/ACLF who underwent CRRT and TPE. We aimed to describe the clinical characteristics and outcomes of these patients. Median (IQR) age was 0.93 years (0.57, 9.88), range 16 days to 20 years. Ten (43%) had ALF and 13 (57%) ACLF. Most (n = 19, 82%) started CRRT for hyperammonemia and/or hepatic encephalopathy and all received TPE for refractory coagulopathy. CRRT was started at a median of 2 days from ICU admission, and TPE started on the same day in most. The liver transplant was done in 17 (74%), and 2 recovered native liver function. Four patients, all with ACLF, died prior to ICU discharge without a liver transplant. The median peak ammonia pre-CRRT was 131 µmol/L for the whole cohort. The mean (SD) drop in ammonia after 48 h of CRRT was 95.45 (43.72) µmol/L in those who survived and 69.50 (21.70) µmol/L in those who did not (p 0.26). Those who survived had 0 median co-morbidities compared to 2.5 in non-survivors (aOR (95% CI) for mortality risk of 2.5 (1.1-5.7), p 0.028). Conclusion: In this cohort of 23 pediatric patients with ALF or ACLF who received CRRT and TPE, 83% survived with a liver transplant or recovered with their native liver. Survival was worse in those who had ACLF and those with co-morbid conditions. What is Known: •  Pediatric acute liver failure is associated with high mortality. •  Patients may require extracorporeal liver assist therapies (like CRRT, TPE, MARS, SPAD) to bridge them over to a transplant or recovery of native liver function. What is New: • Standard volume plasma exhange has not been evaluated against high volume plasma exchange for ALF. • The role, dose, and duration of therapeutic plasma exchange in patients with acute on chronic liver failure is not well described.


Subject(s)
Acute-On-Chronic Liver Failure , Continuous Renal Replacement Therapy , Liver Failure, Acute , Plasma Exchange , Humans , Plasma Exchange/methods , Retrospective Studies , Male , Female , Child , Child, Preschool , Infant , Adolescent , Continuous Renal Replacement Therapy/methods , Liver Failure, Acute/therapy , Liver Failure, Acute/mortality , Young Adult , Infant, Newborn , Acute-On-Chronic Liver Failure/therapy , Acute-On-Chronic Liver Failure/mortality , Treatment Outcome , Liver Transplantation
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