Comparative Analysis of Intravenous Immunoglobulins (IVIg) vs Plasmapheresis (PLEX) in the Management of Myasthenic Crisis.

Myasthenia gravis (MG) is an autoimmune disorder affecting postsynaptic membranes in neuromuscular junctions, presenting as fatigable muscle weakness. Myasthenic crisis is a life-threatening complication characterized by severe respiratory insufficiency necessitating invasive or noninvasive ventilation. Two rapid therapies used to manage myasthenic crises are intravenous immunoglobulins (IVIg) and plasmapheresis (PLEX). Their comparative effectiveness remains equivocal. Our article examines evidence from several clinical trials and observational studies, in order to determine the superiority of one treatment over the other. Multiple factors can complicate the choices between two treatments. We concluded that the choice between PLEX and IVIg is multifaceted, guided by individual patient characteristics, institutional resources, and clinician preference. While PLEX can be considered as first-line for rapid clinical outcomes, it is hard to pick one treatment over the other, and careful consideration of comorbidities and resource availability is crucial. Our article highlights the need for further research to establish definitive guidelines and enhance patient outcomes in myasthenic crisis patients.

A Case of Type 1 Cryoglobulinemia With Lymphoplasmacytic Lymphoma and Dry Gangrene.

Lymphoplasmacytic lymphoma (LPL) is an uncommon condition, accounting for only 2% of all non-Hodgkin's lymphoma cases. Individuals with LPL face the risk of vascular blockage when associated with type I cryoglobulinemia, leading to related symptoms. Until now, no instances of LPL with dry gangrene have been documented. However, we present a rare case involving LPL accompanied by dry gangrene in both the right upper extremity (RUE) and left lower extremity (LLE). The patient was effectively managed using a combination of chemotherapy, steroids, plasmapheresis, and salvage surgery.

Case report: Early onset de novo FSGS in a child after kidney transplantation-a successful treatment.

Background: Early onset de novo focal segmental glomerular sclerosis (FSGS) in the kidney allograft in patients without FSGS in the native kidney is a rare disorder in children. It usually occurs mostly beyond the first year after kidney transplantation and often leads to graft loss. Standardized treatment protocols have not yet been established. Case description: We describe a boy with early onset de novo FSGS in the transplanted kidney and non-selective glomerular proteinuria (maximum albumin-to-creatinine ratio of 3.8 g/g; normal range, ≤0.03 g/g creatinine). Manifestation occurred at 30 days posttransplant and was accompanied by a significant graft dysfunction (eGFR 61 ml/min per 1.73 m2). Treatment with 25 sessions of plasmapheresis over 14 weeks and three consecutive days of methylprednisolone pulse therapy (10 mg/kg per day) followed by oral prednisolone as rejection prophylaxis (3.73 mg/m2 per day) led to sustained remission of proteinuria (albumin-to-creatinine ratio of 0.028 g/g) and normalization of graft function (eGFR 92 ml/min per 1.73 m2) after 14 weeks. The follow-up period was 36 months. Conclusions: This case underlines the efficacy of immunosuppressive and antibody eliminating therapy in early onset de novo FSGS after kidney transplantation.

Measuring the Efficacy of Thymectomy for Pediatric Myasthenia Gravis Across Tertiary Children's Hospitals.

BACKGROUND: Thymectomy is a treatment for pediatric myasthenia gravis, but the efficacy over time is unknown. Multi-institutional data are also lacking. Therefore, the objective of this study was to determine the efficacy of thymectomy for pediatric myasthenia gravis using medication burden and health care utilization as proxies for disease severity. METHODS: This was a cross-sectional study of the Pediatric Health Information System database among children who underwent thymectomy at one of 49 children's hospitals from 2004 to 2022. Differences in annual median number of doses of myasthenia-related medications, admissions, and health care costs in the year before thymectomy to three years after were compared. A comparison cohort that did not undergo thymectomy was utilized. Medians were compared using the Wilcoxon signed-rank test. Generalized linear regression estimated the effect of surgical approach on outcomes. RESULTS: A total of451 patients (238 patients who underwent thymectomy and 213 nonthymectomy patients) were identified. Following thymectomy, the decrease in annual median total number of myasthenia-related doses was 12.0 (interquartile range: 6 to 31) (P < 0.001). The decrease in number of annual admissions was 2.0 (1 to 4) (P < 0.001), which represented a cost difference of $5292 ($3533 to $8681) (P < 0.001). No differences were observed in the control cohort. In a generalized linear regression model, surgical approach was not associated with the efficacy of thymectomy (P = 0.55). CONCLUSIONS: Thymectomy is an effective treatment for pediatric myasthenia gravis, evidenced by the decreased medication burden and health care utilization after surgery. Surgical approach did not influence the success of surgery. Thymectomy should be considered earlier in the treatment algorithm.

Severe copper sulphate poisoning: A case rescued with plasmapheresis.

Acute copper sulphate poisoning is associated with multi-organ failure and high mortality. Patients typically present with gastrointestinal symptoms, haemolysis, methaemoglobinaemia, acute liver injury, rhabdomyolysis and renal failure. Management is usually supportive, and the role of chelation therapy has not been established. Copper is not dialysable. Plasmapheresis has been shown to remove protein-bound copper, reducing plasma and intracellular concentrations. We present a case of severe copper sulphate poisoning, who did not improve with chelation therapy with D-penicillamine and supportive care, but with therapeutic plasma exchange (four cycles) showed rapid clinical recovery.

Severe colchicine poisoning treated successfully with kidney replacement therapy and plasmapheresis: a case report.

Colchicine is commonly prescribed for treatment of inflammatory conditions but has a narrow therapeutic window and dangerous toxicity profile. Here we describe a case of survival after massive unintentional colchicine overdose treated with plasmapheresis and renal replacement therapy. A 37 year old male with history of pericarditis presented to the Emergency Department with a chief complaint of nausea, vomiting, and diarrhea after unintentionally ingesting 36 mg of colchicine 17 h prior to arrival. An initial colchicine concentration resulted at 5.1 ng/mL (30 h post-ingestion) and peaked at 12 ng/mL (40 h post-ingestion). He was treated with continuous kidney replacement therapy (CKRT) beginning on his first day of hospitalization and with plasmapheresis on hospital days two through four. The patient's course was complicated by multiorgan failure including coagulopathy, respiratory failure, neuropathy, renal failure, pancytopenia, and heart failure. He was discharged to inpatient rehabilitation on hospital day 24. On clinical follow up four months after discharge the patient was found to have no significant persistent morbidity related to colchicine overdose.

Treatment of Severe Hypertriglyceridemia During Pregnancy With High Doses of Omega-3 Fatty Acid and Plasmapheresis.

OBJECTIVE: Severe hypertriglyceridemia carries increased health risks, including the development of pancreatitis. The objective of this study was to report on management of 2 cases with severe gestational hypertriglyceridemia. CASES: In case 1, a 33-year-old pregnant woman presented with serum triglyceride level of 14 000 mg/dL after discontinuing hypolipidemic medications. She was treated with Lovaza 12 g/day, and serum triglyceride remained near normal at level of less than 800 mg mg/dL until delivery. In case 2, a 28-year-old patient (29th week gestation) presented with acute pancreatitis and triglycerides >4000 mg/dL. She was treated with Gemfibrozil, Lovaza, insulin infusion, subcutaneous heparin, and escalated to plasmapheresis. She successfully delivered a baby at the week of 36th and her triglyceride level was 304 mg/dL after that. DISCUSSION: Case 1 was treated with high-dose Lovaza and case 2 was treated with plasmapheresis successfully. Triglyceride levels were reduced to less than 500 mg/dL until delivery of healthy babies in both cases. CONCLUSION: Omega-3 fatty acids and plasmapheresis may be effective and safe to treat pregnant women with severe hypertriglyceridemia and pancreatitis.

A dramatic recovery in a patient initially expected to die of TTP & its complications.

Thrombotic thrombocytopenic purpura (TTP) has a high fatality rate if not caught early and treated with plasmapheresis. When TTP patients present late in their sequelae with neuro symptoms, an elevated lactate dehydrogenase and systemic symptoms, there is a high mortality rate. This report describes the case of a young female who had no significant medical problems and presented to our hospital after several days of hematuria, new onset blurry vision and dizziness. She was found to have thrombocytopenia and microangiopathic hemolytic anemia consistent with TTP and was thus started on plasmapheresis. Her course was further complicated with seizures and development of bilateral basal ganglia infarcts which lead to the need for mechanical ventilation. This was followed by worsening renal functions which was managed with intermittent hemodialysis. To add to her multi-organ failure, she developed shock liver along with demand ischemia evidenced by significant elevations in liver enzymes and troponin leaks, respectively. However, on Day 4 it was fascinating to see the beginning of her recovery pathway. It began with response to simple commands followed by discontinuing invasive ventilation and gradual improvement in her renal functions evidenced by increasing urine output. Soon her platelets started rising consistently and she did not require plasmapheresis or hemodialysis by the time she was discharged. This case highlights the rapid recovery of a young female with new onset TTP which was complicated by involvement and severe damage of more than five different organs but was followed by complete recovery of each organ system.

Early versus late institution of plasma exchange therapy in pregnancy induced thrombotic microangiopathy and its effects on clinical outcomes-A case series.

PURPOSE: To look for incidence of pregnancy associated TMA, clinical presentation and impact of early diagnosis and institution of plasma exchange on overall renal outcomes METHODS: -This is a retrospective study among all female patients who presented with acute kidney injury post pregnancy between October 2002 to April 2016 in department of nephrology in a tertiary care hospital in northern India and diagnosed as pregnancy induced TMA. The patient were assessed for duration of onset of renal failure to time of diagnosis of TMA, role of modality of treatment ie plasmpaharesis to outcome. These patients were assessed for complete, partial or no recovery in renal functions at 60 days after admission. RESULTS: Patients whose time of onset of renal failure to a correct diagnosis of TMA was ≤15 days and age less than 30 years was also associated with good prognosis. The patients who received plasma exchange and that to within 72 h of admission had more chances of recovery. CONCLUSIONS: Early diagnosis of disease and early institution of plasma exchange therapy improves renal outcomes in postpartum TMA.

Successful long-term treatment of paraneoplastic optic neuropathy with mycophenolate mofetil, prednisone, and plasmapheresis.

PURPOSE: To report long-term follow-up of a case of paraneoplastic optic neuropathy (PON) successfully treated with combination therapy. OBSERVATIONS: We present a confirmed case of PON from cutaneous melanoma with nearly six years of meticulous follow-up in which vision was preserved and disease remission was successfully induced with combination mycophenolate mofetil, prednisone, and plasmapheresis therapy. CONCLUSIONS AND IMPORTANCE: Treatment of PON and long-term follow-up are not well described in the literature. In our case of PON, vision was preserved and disease remission was achieved with mycophenolate mofetil, prednisone, and plasmapheresis. Such information may be useful for future cases of PON.

Polyparaneoplastic Manifestations of Malignant Thymoma: A Unique Case of Myasthenia, Autoimmune Hepatitis, Pure Red Cell Aplasia, and Keratoconjunctivitis Sicca.

Thymomas are relatively uncommon malignancies of the anterior mediastinum and present with four distinct histological types based on the specific epithelial to lymphocyte ratio: spindle cell, epithelial predominant, lymphocyte predominant, or mixed. Each histologic type of thymoma has a propensity for local invasion and metastasis and can have a wide variety of paraneoplastic manifestations, myasthenia being the most common. We present a unique case of a 34-year-old African-American female who initially presented with a history of profound weakness with repetitive motion, shortness of breath, horizontal nystagmus, persistent anemia, keratoconjunctivitis sicca, and what was initially thought to be azithromycin-induced hepatitis. Upon left anterior thoracotomy with biopsy of the mediastinal mass, pathology yielded a lymphocyte-predominant (B1), Masaoka stage IVA invasive thymoma with pericardial extension. This case illustrates the clinical significance of considering a multitude of extrathymic paraneoplastic manifestations, each with a unique physiological mechanism.

Treatment of meloxicam overdose in a dog via therapeutic plasma exchange.

OBJECTIVE: To describe the treatment of a meloxicam overdose in a dog with therapeutic plasma exchange (TPE). CASE SUMMARY: A 6-month-old female Bulldog, presented for routine laparoscopic ovariectomy. Postoperatively the dog received an accidental overdose of meloxicam (1 mg/kg IV [intravenously]). The patient was treated with supportive medical therapy and TPE over 210 minutes achieving 1.2 plasma volume exchanges. During therapy, heparinized blood and effluent samples were collected. Meloxicam concentrations were determined in the samples by high pressure liquid chromatography. Post TPE, the dog continued to receive supportive medical therapy and was discharged 48 hours after the overdose. The dog remained asymptomatic for meloxicam intoxication. Follow-up rechecks at 1 and 6 weeks were unremarkable with no further treatment required. NEW OR UNIQUE INFORMATION: This report describes the successful use of TPE adjunctively following an acute meloxicam overdose. An 82% reduction of plasma meloxicam concentration was achieved over 210 minutes. Twenty-four hours after therapy, a 47% sustained reduction of plasma meloxicam was measured after redistribution of drug between body compartments.

Guillain-Barre syndrome complicating chikungunya virus infection.

Chikungunya virus (CHIKV) is a mosquito-borne alphavirus which presents with symptoms of fever, rash, arthralgia, and occasional neurologic disease. While outbreaks have been earlier reported from India and other parts of the world, the recent outbreak in India witnessed more than 1000 cases. Various systemic and rarely neurological complications have been reported with CHIKV. We report two cases of Guillain-Barré syndrome (GBS) with CHIKV. GBS is a rare neurological complication which may occur after subsidence of fever and constitutional symptoms by several neurotropic viruses. We describe two cases of severe GBS which presented with rapidly progressive flaccid quadriparesis progressing to difficulty in swallowing and breathing. Both required mechanical ventilation and improved partly with plasmapharesis. The cases emphasize on (1) description of the rare complication in a setting of outbreak with CHIKV, (2) acute axonal as well as demyelinating neuropathy may occur with CHIKV, (3) accurate identification of this entity during outbreaks with dengue, both of which are vector borne and may present with similar complications.

Encephalopathy following melphalan administration.

OBJECTIVE: To describe a rare case of encephalopathy following melphalan administration. Presentation and intervention:: A 59-year-old female with multiple myeloma developed encephalopathy following administration of melphalan. After ruling out other aetiologies, we hypothesized elevated cytokines from systemic inflammatory response to melphalan as the likely aetiology. The TNF-alpha level was found to be significantly elevated. Plasmapharesis was performed which reduced the level of cytokines, and also improved the patient's neurological status. CONCLUSION: Melphalan administration, especially in renally impaired patients, may lead to development of encephalopathy. Based on our case report, we suggest that elevated levels of cytokines could be the underlying mechanism of worsening mental status.

Diabetic Ketoacidosis-induced Hypertriglyceridemic Acute Pancreatitis Treated with Plasmapheresis-Recipe for Biochemical Disaster Management.

Diabetic ketoacidosis (DKA)-induced hypertriglyceridemia causing pancreatitis is an interesting phenomenon that has rarely been reported in literature. Plasmapharesis is a well known treatment modality for hypertriglyceridemia-induced pancreatitis. We report a patient with DKA-induced hypertriglyceridemic acute pancreatitis treated successfully with plasmapharesis.

Plasma selenium status in a group of Australian blood donors and fresh blood components.

The purpose of this study was to assess plasma selenium levels in an Australian blood donor population and measure extra-cellular selenium levels in fresh manufactured blood components. Selenium levels were measured using graphite furnace atomic absorption spectrometry with Zeeman background correction. The mean plasma selenium level in healthy plasmapharesis donors was 85.6±0.5 µg/L and a regional difference was observed between donors in South East Queensland and Far North Queensland. Although participants had selenium levels within the normal range (55.3-110.5 µg/L), 88.5% had levels below 100 µg/L, a level that has been associated with sub-optimal activity of the antioxidant enzyme glutathione peroxidase (GPx). Extra-cellular selenium levels in clinical fresh frozen plasma (cFFP) and apheresis-derived platelets (APH Plt) were within the normal range. Packed red blood cells (PRBC) and pooled buffy coat-derived platelets (BC Plt) had levels at the lower limit of detection, which may have clinical implications to the massively transfused patient.

Clinical evaluation of the endothelial tie-2 crossmatch in ABO compatible and ABO incompatible renal transplants.

The necessity of detection of other than the classical major histocompatibility complex (MHC) and MHC class I-related chain A (MICA) directed antibodies prior to organ transplantation has already been repeatedly reported. A commercial flow cytometric endothelial crossmatch (CM) using isolated peripheral blood tie-2 positive cells provides a tool to detect non-MHC antibodies in addition to antibodies directed to MHC class I and II. The vast majority of circulating tie-2 positive cells expresses HLA-DR but not the A, B blood group antigens. Tie-2 cells are circulating surrogate endothelial cells. In this retrospective study we evaluated the endothelial CM in 51 renal transplantations, 30 with ABO compatible grafts and 21 with ABO incompatible grafts. Fifteen of the ABO compatible recipients (group A) developed unexplained rejection episodes (RE) while the remaining 15 had no RE (group B). Five cases of group A and none of group B had a positive tie-2 CM before transplantation (p=0.042). A positive tie-2 CM was also correlated with graft failure in ABO compatible transplants (p=0.02). No significant correlation was found between a positive pre-transplant tie-2 CM and RE in the ABO incompatible group. This study strongly suggest that a positive tie-2 CM may predict post-transplantation complications in ABO compatible grafts while negative reactions are not predictive. The test is not significantly correlated with RE in ABO incompatible grafts possibly due to applied desensitization.

Clinical characteristics of childhood guillain-barré syndrome.

OBJECTIVES: To find the incidence, clinical pattern and outcome of Guillain-Barre syndrome in the Sultanate of Oman in children less than 15 years of age. METHODS: All children under fifteen years with acute flaccid paralysis were admitted to identify the underlying cause. The diagnosis of Gullain Barre syndrome was made by clinical criteria, cerebrospinal fluid findings and nerve conduction studies. Intravenous immunoglobulins were given to all and two needed plasmapharesis. RESULTS: Sixty-one children were diagnosed as Guillan-Barré syndrome and constituted 20% of cases of acute flaccid paralysis. Males 39 (63.9%) outnumbered females (36.1%).The annual incidence below 15 years was 0.45/100,000. Cranial nerves were involved in 31 (50.8%) children. Albumino-cytological dissociation in cerebrospinal fluid was seen in 42/45(93.3%) cases. Acute relapse was seen in six (9.8%) cases. Eleven children (18.3%) needed ventilation. Complete recovery was seen in 45 to 310 days (mean 69.1 days). Three children (4.9%) were left with minimal residual deficit. There was no mortality. CONCLUSIONS: Guillain Barre syndrome is a serious disease, although recovery is the rule in children. The disease is associated with very low mortality and long term morbidity. Immunoglobulins have reduced the duration of hospital stay and the total time needed for recovery.