Acute disseminated encephalomyelitis (ADEM) is a rare autoimmune disorder characterized by brain and spinal cord inflammation. In pediatric patients, ADEM presents unique challenges due to its potential for rapid progression and long-term neurological sequelae. Therapeutic plasma exchange (TPE) has emerged as a potential treatment option by targeting the underlying autoimmune process and modulating the inflammatory response. This comprehensive review evaluates the role of TPE in pediatric ADEM, synthesizing evidence from clinical studies and providing insights into its efficacy, safety, and potential benefits. The review highlights the variability in TPE efficacy based on disease severity and patient-specific factors. Implications for clinical practice include considering TPE as a therapeutic option, particularly in severe or refractory cases, and emphasizing the importance of early intervention. Recommendations for future research include long-term prospective studies, comparative effectiveness trials, and efforts to standardize TPE protocols. Overall, continued investigation and innovation in managing pediatric ADEM are essential for improving outcomes and quality of life for affected children and their families.

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.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare antibody-mediated neuroinflammatory disease of the central nervous system, typically manifesting in the optic nerves, spinal cord, and other regions of the central nervous system. We hereby report a case of a 16-year-old girl who presented with a six-month history of transverse myelitis with an acute episode of bilateral retrobulbar optic neuritis. MRI revealed patchy contrast enhancements over bilateral retrobulbar intraorbital optic nerves together with long-segment spinal cord hyperintensities (C2 to T2 level). Visual evoked potential testing during the acute presentation showed the absence of P100 bilaterally. However, both serum AQP4-IgG and MOG-IgG were reported to be negative. Despite remarkable improvement in bilateral optic nerve functions, she continued to have disabling bilateral lower limb spasticity, contractures, and loss of bilateral lower limb sensation after five cycles of plasma exchange. This case summarizes the challenges to diagnosing double seronegative NMOSD and its immediate therapeutic significance.

Guillain-Barré syndrome (GBS) is a rare and debilitating autoimmune disorder that affects the peripheral nervous system. Although the exact etiology of GBS is still unknown, it is thought to be triggered by a preceding gastrointestinal infection in most of the cases. Clinical manifestations include limb weakness, areflexia, and sensory loss that can further progress to neuromuscular paralysis affecting the respiratory, facial, and bulbar functions. Both plasmapheresis (PE) and intravenous immunoglobulin (IVIG) have shown effectiveness in the treatment of GBS, but it is still unclear which treatment approach is superior in terms of therapeutic efficacy. This systematic review acts per Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) 2020 guidelines. For appropriate studies and research, we searched PubMed, PubMed Central (PMC), Medical Literature Analysis and Retrieval System Online (MEDLINE), Science Direct, and Google Scholar. Screening of articles was performed based on relevance and inclusion and exclusion criteria. To check for bias, we used relevant quality appraisal tools. Initially, we found 2454 articles. After removing duplicates and irrelevant papers, we finalized 31 studies based on titles, abstracts, and reading entire articles. We excluded 14 studies because of poor quality; the remaining 17 papers were included in this review. IVIG is equally efficacious as PE in improving primary outcomes and secondary outcomes. IVIG showed a slight advantage over PE in reducing the need for mechanical ventilation (MV) and hospital stay duration. However, in children, PE demonstrated a slight edge in improving secondary outcomes. PE was associated with a slightly higher risk of adverse events and post-treatment worsening symptoms compared to IVIG. IVIG is considered more user-friendly with a significantly lower patient discontinuation rate than PE. IVIG treatment was found to be significantly more expensive than PE.

BACKGROUND: Therapeutic plasma exchange (TPE) is used to manage various life-threatening illnesses. It is widely performed by nephrologists, intensivists, pathologists, or experts in transfusion medicine worldwide. However, the costs of TPE sessions are exceedingly high, and they have a huge impact on patients\' financial burden. Herein, we investigated the outcomes of the reuse of plasma filters in TPE on several occasions.
METHODS: This is a retrospective analysis of patients receiving TPE from January 1, 2020, to April 30, 2023, in the Department of Nephrology. A formulation of 4.5% peracetic acid and 24% hydrogen peroxide acid with RO water dilution was used for reprocessing. Clinical outcomes, risks, and cost-benefit were evaluated and compared between the plasma filter reuse group (GP-1) and the no-reuse group (GP-2).
RESULTS: A total of 70 patients were included in this study. 200 and 112 TPE sessions were performed in GP-1 and GP-2, respectively. The most common indication for TPE in both groups was neurological. The clinical efficacy of TPE was similar in both groups. There was no difference in the clotting of the plasma filter, any allergic reaction, infection, or bleeding in the group. However, there was a significant difference in levels of fibrinogen (p=0.03) pre and post-procedure in both groups. The incidence of hypotension was found to be higher in GP-1 (26%) compared to GP-2 (15.6%), p = 0.05. The cost of overall treatment was 38% less in GP-1.
CONCLUSIONS: The reuse of plasma filters is a safe and effective method for cost minimization in patients requiring TPE. This method can be effectively utilized in resource-poor settings without any increased risk of adverse effects.

Thrombotic thrombocytopenic purpura (TTP) is one of the rarely encountered complications of hump-nosed viper bites, which requires early detection and specific management. Hump-nosed viper bites are well known to affect multiple systems, and it is imperative to identify and manage each complication simultaneously. A 48-year-old patient presented to the hospital following a hump-nosed viper bite, where he subsequently developed local necrosis, acute kidney injury (AKI), and TTP. A diagnosis of TTP was made using the PLASMIC score (which refers to the score\'s seven components: platelet count; combined hemolysis variable; absence of active cancer; absence of stem-cell or solid organ transplant; mean corpuscular volume (MCV); international normalized ratio (INR); and creatinine) and supporting blood picture findings despite the diagnostic difficulties encountered due to the misleadingly normal automated platelet counts. The patient underwent multiple blood transfusions, 12 cycles of hemodialysis, and two cycles of therapeutic plasma exchange, the latter contributing to a significant improvement in his overall clinical and biochemical markers. In this case presentation, we report a rare case of TTP occurring after a hump-nosed viper bite, with the outcome of the report focusing on the diagnostic difficulties and available therapeutic modalities.

Tumefactive demyelinating lesions (TDLs) can present as an isolated clinical incidence or could represent the initial presentation of multiple sclerosis. Radiological TDLs are characterized by large tumors like >2 cm space-occupying lesions with mass effect and perilesional edema. Diagnosis is based on MRI imaging and extensive work to exclude other causes and a biopsy of the lesion is often required. First-line treatments include pulsed methylprednisolone. We present a case of a refractory TDL treated successfully with therapeutic plasma exchange.

Thrombocytopenia with concomitant anemia is a serious condition with a high mortality risk. Destruction of platelets, i.e., thrombocytopenia, can be secondary to either auto-antibodies (immune-mediated) or mechanical destruction (non-immune-mediated). The Coombs test is a widespread tool to differentiate between the two categories, resulting in different specific treatment approaches for each diagnosis. A peripheral blood smear can also help make the diagnosis; for instance, in cases of mechanical destruction such as thrombotic thrombocytopenic purpura (TTP), the red blood cell (RBC) shape looks fragmented, forming schistocytes. In rare instances, TTP can present with both schistocytes and a positive Coombs test, challenging the diagnosis of TTP. TTP is a hematological emergency requiring appropriate anticipation and the initiation of treatment prior to the confirmatory ADAMTS-13 test results. Mild forms of TTP can be managed with glucocorticoids and therapeutic plasma exchange. Refractory cases need more aggressive additional treatment with caplacizumab and rituximab. Caplacizumab is an expensive medication that is usually reserved for use after confirmation of a TTP diagnosis. The advantage of caplacizumab lies in its targeted mechanism of action against the A1 domain of the von Willebrand multimers that are normally destructed by the ADAMTS-13 enzyme. Here, we present a young female patient with confirmed TTP, and the initial diagnosis was challenged by the presence of antibodies with the Coombs test. Very little research has studied this rare instance and the appropriate treatment. Our case will save many future lives, as clinicians should be more aggressive in treating refractory TTP with a positive Coombs test.

Thrombotic microangiopathy (TMA) is a severe systemic disorder with multiorgan manifestations due to thrombosis of the microvasculature. Pregnancy and post-partum are particularly high-risk periods for many forms of TMA. The disease progression is rapid and can lead to organ failure and even death; therefore, urgent recognition and treatment are paramount. The presence of other triggers such as infections or autoimmune diseases like systematic lupus erythematosus (SLE) can add further complexity, which emphasizes the need for definitive diagnostic investigations such as kidney biopsy to promptly direct further diagnosis and management. We describe a case of a 27-year-old female with post-partum severe acute kidney injury and nephrotic range proteinuria. She had a new diagnosis of active SLE and was found to have TMA on kidney biopsy without conclusive features of lupus nephritis. She was managed successfully with plasma exchange with rapid improvement of her kidney markers.

Snakebites affect a lot of people in India. Of these, the hemotoxic snakebites may induce a consumptive coagulopathy, which has been termed now as \"Venom-Induced Consumptive Coagulopathy\" (VICC). Some patients with VICC develop Thrombotic Microangiopathy (TMA). The primary end-organ damage in TMA is renal, for which hemodialysis is the mainstay of treatment. Recently there has been some focus on plasma exchange as an adjunctive treatment for TMA. Here we present a case of a young male who developed snakebite-induced TMA and who was successfully managed with plasma exchange.