Sessions Of Plasmapheresis Research Articles

Recurrent Pneumothorax and Pneumomediastinum as a Complication of COVID-19 in a Young Athlete

A novel coronavirus, SARS-CoV-2 was identified as the cause of a cluster of pneumonia cases in Wuhan, China in December 2019. Coronavirus Disease 2019 (COVID-19) has rapidly spread worldwide. Numerous studies have shown diverse findings on chest CT scan of the patients with COVID-19. The established well-known features of COVID-19 on chest imaging include bilateral multilobar ground-glass opacification (GGO) predominantly with peripheral distribution, mainly in the lower lobes. Atypical presentation of consolidative opacities superimposed on GGO may be found in a smaller number of cases, mainly in the elderly populations. Pleural and pericardial effusion, lymphadenopathy, cavitation, halo sign on CT scan, and pneumothorax are uncommon but may be seen with disease progression. We report a case of severe COVID-19 in an athlete man with development of bilateral pneumothorax, pneumomediastinum and subcutaneous emphysema during progression of the disease. The only risk factor for severe COVID-19 in our patient was suggested to be chronic use of dexamethasone as anabolic steroids. Our patient also received three sessions of plasmapheresis. Unfortunately, the patient expired due to recurrence of bilateral pneumothorax and pneumomediastinum.

Coexistence of thrombotic thrombocytopenic purpura and immune thrombocytopenic purpura in an Asian woman: a case report

A 33-year-old Chinese woman with a history of immune thrombocytopenic purpura presented with heavy menstrual bleeding. She was found to have thrombocytopenia, plasma ADAMTS13 activity of 0%, and positivity for the plasma ADAMTS13 inhibitor. She was diagnosed with the coexistence of thrombotic thrombocytopenic purpura and immune thrombocytopenic purpura. The patient was treated by plasmapheresis, a glucocorticoid, and rituximab. Her platelet level returned to normal, and she was discharged 28 days after admission. The number of plasmapheresis sessions and the timing of rituximab administration may be the key aspects of management of patients with thrombotic thrombocytopenic purpura who have underlying immune dysfunction caused by diseases such as immune thrombocytopenic purpura.

POS-139 AN ATYPICAL PRESENTATION OF DUAL-POSITIVE ANTI-MYELOPEROXIDASE AND ANTI-GLOMERULAR BASEMENT MEMBRANE ANTIBODIES VASCULITIS WITH PULMONARY-RENAL SYNDROME : A CASE REPORT

Double-positive vasculitis with anti-polynuclear cytoplasm (ANCA) and anti-glomerular basement membrane (GBM) antibodies is a rare entity of systemic vasculitis defined by the presence of ANCA and anti-GBM antibodies. We report a rare case of pulmonary-renal syndrome with atypical clinical presentation. A 52 year-old smoking man with a history of exposure to hydrocarbons and uretheral lithiasis, presented in April 2021 epigastralgia and vomiting. the investigations concluded to H.pylori gastritis and ulcer and he received a quadruple therapy. The kidney function was correct in April 2021. The evolution was marked by the persistence of symptoms and urine output had decreased for a few days. He was found to have renal dysfunction (serum creatinine: 2000 µmol/L). Abdominal CT scan without iodinated contrast injection showed severe hydronephrosis of the right pelvicalyceal system with cortical thinning and dilatation of the right ureter. The two kidneys had regular outlines seat multiple bilateral renal cysts with exophytic development. He had a nephrosomy with secondarily a right double-J stent with slight improvement of renal function. The patient presented then with acute respiratory distress.Testing for COVID-19: PCR and serology were negatives. Chest CT scan showed alveolar syndromeevoking pulmonary overload. No pneumopathy covid was shown. The evolution was marked by the non improvement by depletion and he developed hemoptic sputum and low-abundance epistaxis. The attitude was non-invasive ventilation and broad-spectrum antibiotics therapy. Control chest CT showed emphysematous lung with signs of fibrosis with bilateral subpleural nodules. A rereading of the scanner showed intraalveolar hemorrhage which has regressed on the imaging of the control. Based on these data, pneumo renal syndrome was suspected and a bronchoscopy was performed showing alveolar hemorrhage with 70% siderophageswith Gold score superior to 100. Anti-GBM and p-ANCA and antibodies were positive at a high titer. Electroneuromyogram was without anomaly. Kidney biopsy was not done because of the presence of multiple cysts. The patient received pulse methylprednisone for three days followed by oral prednisone and underwent eleven sessions of plasmapheresis. Intraveinous Cyclophosphamide has been started. He showed remarkable recovery as his lung fields cleared with negativity of GBM antibodies. Kidney function didn't improved and he remained dependent on dialysis. Our observation is exceptional since the clinical and radiological presentation of the patient was not that of a pulmonary-renal syndrome. The elements of this syndrome have in fact been masked by the obstacle on the urinary tract on one hand and the hypothesis of a covid19 pneumonia on the other hand in the face of the epidemiological context. Atypical feature of pulmonary renal syndrome should be kept in mind to avoid diagnostic and treatment delays.

Prostate abscess with acute urinary retention as the initial presentation of granulomatosis with polyangitis

A 50-year-old male presented with acute urinary retention. He also had a history of cough, haemoptysis, exertional dyspnoea, loss of appetite, generalised weakness and left-ear discharge of 1-month duration. Prior episode of acute urinary retention had occurred 2 months ago; diagnostic evaluation revealed prostate abscess, and he had received treatment elsewhere for the same. He also had a history of hypertension. Physical examination revealed fever, pallor, bilateral parotid enlargement and left-ear serous discharge. Clinical examination, imaging of chest revealed consolidation with cavitation on the left side, bilateral pleural effusion. Laboratory testing revealed normocytic normochromic anaemia (haemoglobin 6.6 g/dL); neutrophilic leucocytosis (total leucocyte count 13,100 cells/mm3; polymorphs 87%); raised erythrocyte sedimentation rate (110 mm at the end of the first hour); elevated serum creatinine (2.69 mg/dL) and an active urine sediment. Flexible fibreoptic bronchoscopy showed alveolar haemorrhages. Bronchoalveolar lavage fluid Xpert MTB/RIF testing, cytopathology were negative. Computed tomography-guided biopsy from the lesion was suggestive of granulomatous vasculitis. Cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA) tested positive. The patient was diagnosed to have granulomatosis with polyangitis (GPA). As arterial hypoxaemia was evident, tracheal intubation and mechanical ventilatory support were initiated. Renal replacement therapy, intravenous (iv) methyl prednisolone and cyclophosphamide pulse therapy and five sessions of plasmapheresis were administered. As remission could not be achieved, iv rituximab was started. On the 36th day of admission, the patient developed septic shock and died. The present case documents the uncommon association of GPA with prostate abscess, acute urinary retention.

A CASE OF IVIG REFRACTORY GUILLAIN-BARRÉ SYNDROME IN THE SETTING OF SUBCLINICAL COVID-19 INFECTION

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: The novel coronavirus pandemic of 2019 (SARS COVID-19) has significant morbidity and mortality. The downstream complications resulting from this infection are not well characterized. There have been case reports of patients with COVID-19 infection presenting with Guillain-Barre Syndrome (GBS) Here, we present a case of one such patient, who failed initial management with intravenous immunoglobulin G (IVIG), requiring plasmapheresis as a treatment modality. CASE PRESENTATION: A 48-year-old male with a past medical history of hypertension and type 2 diabetes mellitus presented to the emergency department with a four day history of progressive paresthesias and weakness. This began in his bilateral lower extremities and ascended to the bilateral upper limb and face. Concurrent mild dysarthria and dysphagia were noted. On this encounter, he had a first time positive test for COVID-19 via nasopharyngeal polymerase chain reaction (PCR). On physical exam he had reduced dorsiflexion of the right ankle, with normal power throughout the rest of the body. He had graded sensory loss in the lower extremities, without a sensory level and diminished reflexes throughout. Lumbar puncture and MRI imaging were suggestive of demyelinating neuropathy. Due to the clinical history and albuminocytologic dissociation in the CSF, he was assessed with GBS and commenced on IVIG 0.4g/kg for 5 days. On day 4 of hospitalization, worsening dysarthria and dysphagia was noted and he was transferred to the intensive care unit (ICU). Subsequently, due to lack of adequate improvement in the patient's symptoms, plasmapheresis was initiated. The patient completed 7 additional sessions of plasmapheresis and had symptom resolution. DISCUSSION: There have been several case reports of GBS in the setting of COVID-19, which has been thought to be a para-infectious in origin. According to a systematic review, 96.6% of patients affected with COVID-19 GBS experienced typical COVID-19 symptoms prior to being diagnosed with GBS. This syndrome has been seen in other viral illnesses, such as West Nile virus and the Zika virus. Most of these cases have been treated successfully with IVIG, but in this report, the patient required plasmapheresis to achieve successful resolution of his symptoms. At this time, there have been approximately three published cases of GBS in the setting of COVID-19 in the USA which required plasmapheresis as a treatment modality. Additionally, all of these cases presented with typical clinical symptoms associated with COVID-19 infection. CONCLUSIONS: This patient's findings highlight one of the first cases of GBS in the setting of sub-clinical COVID-19 infection. This adds to the growing set of literature establishing a neurological component of the disease. It also demonstrates the use of plasmapheresis as an additional tool in the treatment of COVID-19 associated GBS. REFERENCE #1: Agosti, E., Giorgianni, A., D'Amore, F., Vinacci, G., Balbi, S., & Locatelli, D. (2021). Is Guillain-Barre syndrome triggered by SARS-CoV-2? Case report and literature review. Neurological Sciences, 42(2), 607-612. REFERENCE #2: Abrams, R. M., Kim, B. D., Markantone, D. M., Reilly, K., Paniz-Mondolfi, A. E., Gitman, M. R., … & Robinson-Papp, J. (2020). Severe rapidly progressive Guillain-Barre syndrome in the setting of acute COVID-19 disease. Journal of neurovirology, 26(5), 797-799. REFERENCE #3: Hasan, I., Saif-Ur-Rahman, K. M., Hayat, S., Papri, N., Jahan, I., Azam, R., … & Islam, Z. (2020). Guillain-Barre syndrome associated with SARS-CoV-2 infection: A systematic review and individual participant data meta-analysis. Journal of the Peripheral Nervous System, 25(4), 335-343. DISCLOSURES: No relevant relationships by Spencer Deleveaux, source=Web Response No relevant relationships by Sarah Usmani, source=Web Response

Plasmapheresis: a feasible choice for bullous pemphigoid patients infected with SARS-CoV-2.

Bullous pemphigoid (BP) patients were vulnerable to severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) infection because they have similar risk factors, so we should pay attention to patients with BP during the epidemic of coronavirus disease‐19 (COVID‐19). As far as treatment is concerned, many strategies for BP were changed during the epidemic. Plasmapheresis not only has been included in the guidelines for BP but also has been used successfully to rescue COVID‐19 patients, especially in severe cases. Therefore, it is a feasible choice for BP patients, especially for refractory BP patients, infected with SARS‐CoV‐2. Apart from these, we have reviewed some points for attention during the plasmapheresis session.

Effect of modified neoadjuvant chemotherapy on levels of E6 oncoprotein in HPV-infected cervical tumor tissue

. Background. Human papillomavirus (HPV) has been established to be the etiological factor of cervical cancer (CC). HPV infection and CC progression involve the direct participation of the E6 oncoprotein. Aim. An analysis of the E6 oncoprotein levels in tissues of the tumor and its perifocal area in HPV-associated cervical squamous cell cancer as an objective indicator of the effect of treatment depending on preoperative chemotherapy. Material and methods. The study included clinical and laboratory data of 237 patients with high-risk HPV infection of the cervix. The patients were divided into 4 groups: two main groups (CC T2а2–2bN0–1M0) and two control groups. Patients in the main group 1 (n=84) received standard neoadjuvant chemotherapy (NACT), in the main group 2 (n=93) — modified NACT with prior plasmapheresis session and a parallel course of nonspecific immunotherapy with Allokin-alpha. Control group 1 (n=40) included patients with CC T1b2–2a1N0–1M0, surgical treatment; control group 2 (n=20) — HPV-positive patients without CC. Levels of E6 were measured in samples of the cervical tumor and perifocal tissues. Results. The lowest levels of the E6 oncoprotein were registered in the group of HPV-positive patients without CC. After modified NACT, E6 levels in tumor tissues remained 4.6 times higher than in intact tissues, and even so, these patients demonstrated minimal E6 levels compared to other CC patients. E6 in tumor tissues was significantly lower than in main group 1 (by 3.3 times) and 8 times lower than in control group 1. E6 levels in the perifocal tissues of patients in main group 2 were 1.9 times lower than in the corresponding tissues of patients in main group 1 and 2.2 times lower than in control group 1. Conclusions. Inclusion of plasmapheresis and inducers of endogenous interferonogenesis into neoadjuvant treatment for cervical cancer can be considered pathogenetically justified, since it affects the key unit of cervical carcinogenesis.

Lung transplant after 6 months on ECMO support for SARS-CoV-2-induced ARDS complicated by severe antibody-mediated rejection

There have been a few reports of successful lung transplantation (LTx) in patients with SARS-CoV-2-induced acute respiratory distress syndrome (ARDS); however, all reports were with rather short follow-up. Here we...

Risk of bleeding after plasmapheresis!

Background: Plasmapheresis is a growing up method used for treatment of many diseases. it is considered as first line therapy for different diseases. During this therapeutic modalities there is an undesirable l removal of coagulation proteins particularly fibrinogen. Aim The aim of the present study was to assess the presence or absence of bleeding risk following therapeutic plasmapheresis. This was done by assessment of fibrinogen level pre and post plasmpharesis in different indication including the renal and non-renal disorders.Methods: The present case control study was conducted on 40 persons from El-Demerdash Hospital in Ain Shams University from the period of May 2019 till November 2019. The study was approved by the hospital’s research ethics board. The 40 patients were divided into 2 groups according to presence or absence of renal disoredes as renal and non-renal indication for plasmapheresis, Fibrinogen level, aPTT, INR were estimated pre and post session of plasmapheresis. Also, all patients were observed during plasmapheresis session and for 72 hours later for clinical evidence of bleeding tendency.Results: Fibrinogen levels and aPTT were reduced significantly following plasmapharesis but without increased risk of bleeding.Conclusions: Fibrinogen level should be assessed in patients undergoing plasmapheresis especially those indicated for invasive maneuvers or surgery following plasmapheresis.

Case Report: Systemic lupus erythematous associated with thrombotic thrombocytopenic purpura, a diagnostic challenge

Thrombotic thrombocytopenic purpura (TTP) is an uncommon microangiopathic disease and often occurs as a complication of systemic lupus erythematous (SLE). However, this probable causal relationship has not been completely proven. The diagnostic differentiation of both diseases is difficult in the first instance because they share similar characteristics that may overlap. We present a case of a 32-year-old woman with antecedents of epilepsy since she was 12 years old. The patient was admitted to the emergency room with a clinical picture of headaches, fever, paleness in the skin and mucosa, confused state, paresthesia, and transient spasticity of the extremities. The laboratory results revealed Coombs negative direct autoimmune hemolytic anaemia, severe thrombocytopenia, significant elevation of the enzyme lactate dehydrogenase, and presence of schistocytes ++ in the peripheral film. In addition, positive antinuclear antibodies and positive anti-native DNA in titers of 1/320 and 1/160, respectively, were found. Renal function was conserved. We concluded that it was a case of TTP associated with SLE and indicated treatment with plasmapheresis and methylprednisolone pulses, obtaining a satisfactory response (normalization of biomarker levels, health condition) after the second session of plasmapheresis. Diagnosis of both SLE and TTP is often difficult to achieve; however, adequate correlation of clinical manifestations and laboratory tests, along with the help of partial therapeutic interventions, may lead to good clinical response.

EFFICACY OF PLASMAPHERESIS IN THE TREATMENT OF STEATOHEPATITIS ACCOMPANIED BY OBESITY AND HYPERSIDEREMIA

Aim of the study: To establish the effectiveness of discrete plasmapheresis in the treatmentof patients with nonalcoholic fatty liver disease accompanied by hyperferritinemia andconcomitant obesity.Material and methods. The study is based on the clinical observations of 18 patientswith non-alcoholic steatohepatitis accompanied by hyperferritinemia and concomitantobesity. Each patient was undergoing the basic therapy as well as the sessions of discreteplasmapheresis for 10-14 days, which led to the removal of 17-20% of the volume ofcirculating plasma during each procedure, the total of which amounts to about 1-1.5 ofthe circulating plasma volume.Results. As a result of the two-week treatment, the patients' well-being improvedsignificantly, their efficiency increased, and previously elevated levels of ferritin (1.29times in men and 1.21 in women) and serum iron (2.7 times) decreased. The initiallyelevated level of total cholesterol also decreased significantly (2.34 times), and due toVLDL (7.86 times) and triacylglycerols – 9.78 times. The atherogenic factor decreasedfrom 3.8 to 1.5. (2.5 times). The sizeable decrease was observed in the uric acid content– 1.77 times, ALT activity – 1.55 times, LDH – 1.77 times, LF – 1.42 times, GGTP – 4.0times (p <0.05).Conclusions. Inclusion of the discrete plasmapheresis sessions to the treatmentprogram of non-alcoholic steatohepatitis at comorbidity with hyperferritemia andobesity enables to reduce significantly the initially elevated blood levels of ferritin,serum iron, LPVLD, triacylglycerols, cholesterol, AAT, LDHG, AP, GGTF activity.

How to Stop a Thyroid Storm When the Liver Is Bad: A Case Report and Literature Review

Introduction: Patients with thyroid storm and resistance or contraindications to conventional medications may receive plasmapheresis until they have the definitive therapy.Case Presentation: A 42 years old lady with no past medical history was brought by the EMS with palpitations, shortness of breath, vomiting, and profuse diarrhea. She was found to have an atrial flutter with low blood pressure, received synchronized cardioversion, but unfortunately, she developed ventricular tachycardia, tonic-clonic seizure and went to pulseless electrical activity (PEA). Upon examination, the patient was intubated, heart rate of 200 beats/min, blood pressure of 80/60 on vasopressors. She had exophthalmos and icteric eyes. Neck examination revealed palpable goiter. There was bibasal fine cracked and mild lower limb edema. Laboratory showed FT4 39 (11.6-21.9 pmol/L), FT3 5 (3.7- 6.4 pmol/L), and TSH <0.01 (0.3-4.2 mIU/L). Burch- Wartofsky’s score was 55/140. Her presentation was suggestive of Graves’ disease with thyroid storm. Further labs showed high liver enzymes, high INR, ammonia as well as high creatinine.She was started on IV hydrocortisone and cholestyramine. Thionamides were contraindicated due to liver impairment.Extracorporeal membrane oxygenation (ECMO) was initiated for cardiopulmonary support and continued for 6 days. TSH receptor antibodies result was pending, thus a thyroid uptake scan was done while the patient connected to ECMO to confirm the diagnosis. Thyroid scan showed increased uptake suggestive of grave’s disease despite iodine contrast received for CT scan chest two days back. After 5 sessions of plasmapheresis, FT3 2.8, and FT4 30, Lugol’s iodine started and she underwent total thyroidectomy. She was successfully extubated and thyroxine replacement was started after normalization of thyroid hormonesDiscussion: The raised liver enzymes (shock liver) were a barrier to thioamides. With the contraindication to antithyroid medications, plasmapheresis was a rapid and safe option before thyroidectomy. The mechanism of plasmapheresis is to eliminate thyroid hormones, TSH-receptor antibodies, and cytokines. The current guidelines lack clear indications, the timing of initiation, and patient selection for plasmapheresis.Conclusion: Plasmapheresis should be considered as a stabilising measure, especially when patients cannot tolerate conventional medications. Plasmapheresis leads to rapid decline in thyroid hormone levels, providing a window to treat definitively with thyroidectomy.

Metabolic Improvements After Gastric Sleeve Surgery in a Patient With Familial Partial Lipodystrophy

Familial Partial Lipodystrophy (FPLD) is a rare genetic disorder characterized by loss of subcutaneous adipose tissue mainly from peripheral areas but preservation, or increase, of fat in the face, neck, and trunk. This abnormal fat redistribution leads to a characteristic phenotype and severe metabolic derangements that are difficult to manage. FPLD often present with severe insulin resistance causing type 2 diabetes mellitus (DM2), acanthosis nigricans, hypertriglyceridemia (HTG), and non-alcoholic steatohepatitis (NASH). We present a case of FPLD with severe HTG and HTG induced pancreatitis requiring plasmapheresis, with dramatic metabolic improvements after gastric sleeve surgery. Case presentation: Our patient is a 40-year-old Caucasian male who was diagnosed with DM2 and HTG at age 18 when he presented with pancreatitis. He reported eruptive xanthomas with triglyceride (TG) >3000 mg/dl on the initial presentation. He has central obesity with disproportionately thin extremities and NASH. He has a strong family history of HTG and premature coronary artery disease. He was in a leptin trial; however, he was not included in an extended arm due to deterioration of his metabolic profile, specifically NASH. Despite aggressive therapy with dietary changes, fenofibrate, statin, omega-3, and niacin, he had multiple episodes of pancreatitis with TG levels >5000 mg/dl on many occasions. As a result, he was started on biweekly plasmapheresis that was later changed to weekly. His insulin requirement increased to 450 units daily on U-500. A decision was made for him to proceed with bariatric surgery with his history of insulin-resistant DM2 and morbid obesity. He lost 54 lbs in one year with sleeve gastrectomy and his insulin requirement decreased to 120 units daily. Above all, he had only a single incomplete session of plasmapheresis since his bariatric surgery. He has not required plasmapheresis for over a year so far and his TG levels are consistently <500 mg/dl while only on rosuvastatin 40 mg, with the most recent TG level of 182 mg/dl. Discussion: Bariatric surgery has shown tremendous results in terms of reversal of diabetes and other metabolic derangements. These metabolic benefits are attributed mainly to weight loss in restrictive surgeries and proposed increased GLP-1 levels with Roux-en-Y Gastric Bypass Surgery (RYGB). There are a few case reports of FPLD patients with positive outcomes in terms of metabolic profile with RYGB. In our patient, bariatric surgery was decided due to his DM2 and morbid obesity. He had an unexpected dramatic improvement in the metabolic control of his lipodystrophy. To our knowledge, this is the first case of a FPLD patient with severe HTG requiring plasmapheresis with striking metabolic improvements after sleeve gastrectomy. Gastric sleeve surgery may be an important adjunct or alternative treatment option to the current standard of therapy in patients with FPLD.

Guillain-Barre syndrome after coronary artery bypass graft surgery: a case report and literature review

BackgroundGuillain-Barre syndrome (GBS) is an acute polyradiculoneuropathy, often preceded by an infection. Rarely, it is preceded by a surgical procedure. Most of the postsurgical GBS cases are reported with the neurosurgical, gastrointestinal, orthopedic, and cardiac procedures. GBS is rarely reported after a coronary artery bypass grafting (CABG). To date, only 12 cases have been reported in the literature. Our case is 13th in number and the first case from Pakistan.Case presentationWe report a case of a 54-year-old man presented with acute flaccid quadriparesis and areflexia after a CABG procedure. He was subsequently diagnosed with an acute inflammatory demyelinating polyneuropathy (AIDP) variant of GBS and underwent five sessions of plasmapheresis. His weakness improved and could ambulate unassisted on follow-up visits.ConclusionGBS is a potentially treatable condition if timely diagnosed and managed. It should be considered in every patient presenting with an acute-onset weakness after surgery.

POS-026 INAUGURAL ACUTE AND SEVERE RENAL FAILURE OF THROMBOTIC THROMBOCYTOPENIC PURPURA

Thrombotic Thrombocytopenic Purpura (TTP) is a rare disease characterized by generalized microvascular thrombotic lesions. associating hemolytic anemia and thrombocytopenia associated. The most frequent other signs are neurological, cardiac and gastrointestinal damage. Associated Kidney damage is rare and poses the problem of differential diagnosis with hemolytic and uremic syndrome. We report a case of TTP with atypical presentation but a favorable outcome. Observational case report A 43-year-old patient was admitted for investigation of severe acute renal failure (creatinine 1642 μmol/l). The history revealed generalized tonicoclonic seizures in childhood. He has no hypertension nor neurological or gastrointestinal signs. Biologicals finding were: microscopic hematuria, proteinuria: 400mg/g, hemoglobin 6g/dl, schizocyte 2%, haptoglobin<0.1g/l, platelets 35. 109/l, direct coombs test was negative. Antinuclear antibody were positive (1/200) and speckled, low C3, normal C4. Factor H was normal without factorH antibodies. Ultrasound showed normal kidney size. The diagnosis of lupus nephritis was retained, renal biopsy was contraindicated because of deep thrombocytopenia. Several dialysis sessions were performed. The patient was treated by high-dose intravenous methylprednisolone (0.5 g/d for three days) then oral prednisone up to 1 mg/kg and by monthly intravenous pulses of cyclophosphamide dosed at 1g/m2 for 6 months (according to the NIH regimen) with improvement of renal function (creatinine 240 μmol/l) and hematological disturbances. Kidney biopsy was performed three months later, after correction of thrombocytopenia, renal histology showed eight normal glomeruli with interstitial fibrosis and negative immunofluorescence. Six months later, while the patient was on prednisolone 20 mg/day, he presented with hemiparesis and dysarthria revealing a stroke in the left middle cerebral arterial territory with hypertensive emergency (blood pressure: 200/100mmHg) and acute renal failure (creatinine 959μmol /l), hemolytic anemia and thrombocytopenia. The patient received high-dose intravenous corticosteroids and 3 sessions of plasmapheresis using fresh frozen plasma with clinico-biological improvement. ADAMTS-13 activity level was 1% without autoantibodies (<12U/ml). Two years after the inaugural episode, he maintains stable renal function (creatinine 200 μmol/l) using regularly fresh frozen plasma perfusion. This is a constitutional and severe ADAMTS-13 deficiency, with atypical renal involvement and absence of hypertension at presentation. The neurological manifestations, appeared nine months after the onset of the disease, rectified the diagnosis. Initial improvement with corticosteroid therapy and plasmapheresis suggest that autoantibodies may also be present. Regular supplementation of deficient protein maintained remission.

POS-506 ROLE OF PLASMAPHERESIS IN RAT KILLER PASTE POISONING

Yellow phosphorus containing rat killer paste causes toxic hepatitis and coagulopathy in humans. It is a common mode of self-harm in a developing country like India. Till date, only liver transplant is the definitive treatment of fulminant liver failure caused by rat killer paste poison. Plasmapheresis replaces the main functions of the failing liver and removes proinflammatory cytokines that are responsible for multi-organ failure. The replacement fluid replenishes the coagulation factors, albumin and immunoglobulins. The complete process improves the microenvironment of the liver, which in turn accelerates the regeneration and helps in the functional recovery. In this study, we have tried plasmapheresis as an innovative approach for the management of liver failure cases due to rat killer paste poisoning. This prospective study was conducted in Madurai Medical College. The dialysis machine used was 4008 S Fresenius and the filter used was Plasma Flux P2 filter. 76 cases (Male-47, Female-29) with a definite history of rat killer paste poisoning who had INR more than 3, hepatic encephalopathy and 10-fold elevation in the liver enzymes were taken up for plasmapheresis and the outcomes compared. Pre and post plasmapheresis liver function tests (LFTs) and coagulation profile were compared, and Model for End stage Liver Disease (MELD) score was calculated. Delta MELD was calculated as the difference between pre and post plasmapheresis MELD score. The population was subjected to a minimum of 1 session and a maximum of 3 sessions of plasmapheresis. The replacement fluid was Fresh Frozen Plasma (FFP) according to the body weight. All the patients were also treated with iv glutathione and N-Acetyl cysteine. The median age was 23 years (19-32) (Median with Interquartile Range). The overall survival rate was 63.2 %. 88.2 % of the population had hyponatremia (survivors- 131.5 mEq/L, non-survivors- 129.57 mEq/L), which was found to be statistically significant with regards to outcome (p <0.001). Incidence of hypokalemia, leucopenia and thrombocytopenia were 51.3%, 52.6% and 47.4% respectively, though not statistically significant. Comparison of the pre and post plasmapheresis LFTs were found to be significant to outcome (p-0.001) [Total Bilirubin (12.4mg/dl, 6.8mg/dl), SGOT (1126 IU/L, 108 IU/L), SGPT (876 IU/L, 116 IU/L), PT (51.1s, 18.4s), INR (4.6, 1.5), APTT (48s, 30.2s)]. Median Delta MELD was found to be 12.08 in survivors, whereas it was 8.7 in non survivors, and was found to be statistically significant with regards to outcome (p- 0.001). The total number of plasmapheresis sessions conducted also correlated with outcome (p-0.001). The Median Survival Time in non-survivors was 199.585 hours (95% Confidence Interval). Plasmapheresis can be a therapeutic option for the treatment for acute liver failure due to rat killer paste poisoning. This is supported by the fact that there is a significant improvement in the liver functions and the coagulation parameters post plasmapheresis. Delta MELD may be used as a prognostic indicator of outcome.

PREDICTORS OF RESPONSE TO PLASMAPHERESIS IN AUTOIMMUNE NEUROPATHIES

Background: Immune-mediated neuropathies represent a significant portion of cases encountered by neurologists. The scope of responsive neuropathies, the extent of response to plasmapheresis and its predictors varies widely. Objective: To recognize predictors of response to plasmapheresis in autoimmune-neuropathies. Patients and Methods: Seventy-six subjects; 61 Guillain-Barre syndrome (GBS) patients and 15 chronic inflammatory demyelinating polyneuropathy (CIDP) patients were recruited and evaluated using Medical Research Council sum score (MRCSS) and Modified Neuropathy Disability Score (NDS); on admission, 2 weeks and 3 months after six session of plasmapheresis. Different clinical, laboratory and neurophysiological variables were evaluated as possible predictors of response. Results: Of 61GBS patients, after 3 months, 41% (n=25) of them were poor-responders to plasmapheresis (<50% increase in MRCSS), and 59% (n=36) were good-responders. Older age, higher MRCSS on admission, lower NDS on admission, cyto-albuminous dissociation, electro-physiological evidence of axonal nerve affection and low NLR were predictors for poor response to plasmapheresis in GBS patients. Of 15 CIDP patients, after 3 months, 53.3% (n=8) of them were poor-responders to plasmapheresis (< 30% increase in MRCSS), and 46.7% (n=7) were good-responders. Higher MRCSS on admission, low CMAP amplitude, low MCV and long time between onset of the health problem and start of plasmapheresis were predictors for poor response to plasmapheresis in CIDP patients. Conclusion: Response to plasmapheresis in autoimmune neuropathies is variables and depends on several factors that can predict it.

Heparin-Induced Thrombocytopenia and Thrombosis with Axillary Impella 5.5 as Bridge-to-Transplant

IntroductionA patient presenting with biventricular failure was managed with Impella 5.5 as BTT. His course was complicated by HITT causing device failure, and he successfully underwent heart transplant (HTx) after plasmapheresis.Case ReportA 44-year-old man with DM, HTN, and HLD was transferred to our center in cardiogenic shock. Coronary angiography revealed slow flow without obstruction. TTE showed biventricular dysfunction with LVEF 10%. Impella CP was placed, and on transfer the device was exchanged for a surgically placed axillary Impella 5.5. Filling pressures and RV function improved with diuresis. Platelets decreased from 230 (1000/uL) to 96 by hospital day 10, and a PF4 antibody was sent (Fig 1A). The next day, Impella purge pressure doubled and purge flow decreased, concerning for device thrombosis. The PF4 antibody returned positive, suggesting HITT. Heparin was stopped and argatroban administered systemically and through the purge. Pump power and hemodynamics were maintained. While awaiting results of a serotonin release assay, a suitable donor was identified. The risks and benefits of HTx included risk of cardiopulmonary bypass with heparin-PF4 antibodies, risk of Impella exchange, and risk of imminent device failure and stroke. Preliminary SRA was positive. The patient underwent two sessions of plasmapheresis and was transplanted on day 13. In the OR, there was extensive thrombus encasing the device (B). The driveline was transected proximally, and the distal catheter was removed with the native heart to decrease risk of embolization. After HTx, the patient was placed on argatroban and transitioned to apixaban.SummaryWe describe using Impella 5.5 as BTT in severe biventricular failure, where RV function improved with LV unloading. The case is notable for TCS failure due to HITT despite only modest thrombocytopenia (nadir 86,000), and for proceeding with HTx after plasmapheresis rather than Impella exchange. The patient was anticoagulated for a month post-HTx and is doing well. A patient presenting with biventricular failure was managed with Impella 5.5 as BTT. His course was complicated by HITT causing device failure, and he successfully underwent heart transplant (HTx) after plasmapheresis. A 44-year-old man with DM, HTN, and HLD was transferred to our center in cardiogenic shock. Coronary angiography revealed slow flow without obstruction. TTE showed biventricular dysfunction with LVEF 10%. Impella CP was placed, and on transfer the device was exchanged for a surgically placed axillary Impella 5.5. Filling pressures and RV function improved with diuresis. Platelets decreased from 230 (1000/uL) to 96 by hospital day 10, and a PF4 antibody was sent (Fig 1A). The next day, Impella purge pressure doubled and purge flow decreased, concerning for device thrombosis. The PF4 antibody returned positive, suggesting HITT. Heparin was stopped and argatroban administered systemically and through the purge. Pump power and hemodynamics were maintained. While awaiting results of a serotonin release assay, a suitable donor was identified. The risks and benefits of HTx included risk of cardiopulmonary bypass with heparin-PF4 antibodies, risk of Impella exchange, and risk of imminent device failure and stroke. Preliminary SRA was positive. The patient underwent two sessions of plasmapheresis and was transplanted on day 13. In the OR, there was extensive thrombus encasing the device (B). The driveline was transected proximally, and the distal catheter was removed with the native heart to decrease risk of embolization. After HTx, the patient was placed on argatroban and transitioned to apixaban. We describe using Impella 5.5 as BTT in severe biventricular failure, where RV function improved with LV unloading. The case is notable for TCS failure due to HITT despite only modest thrombocytopenia (nadir 86,000), and for proceeding with HTx after plasmapheresis rather than Impella exchange. The patient was anticoagulated for a month post-HTx and is doing well.

Treatment of Anti-Body Mediated Rejection Complicated by Cardiogenic Shock with a Temporary Left Ventricular Assist Device as a Bridge to Recovery

<h3>Introduction</h3> Anti-body mediated rejection (AMR) after cardiac transplantation is a difficult entity to diagnose and treat. Mortality rates in AMR remain high and treatment strategies vary but consist of therapies that aim to remove circulating antibodies, decrease antibody production, suppress T and B-cell responses, and inhibit complement. We report a case of AMR associated cardiogenic shock supported on Impella 5.0. <h3>Case Report</h3> : A 51-year-old man who received a cardiac transplant in 2018 was admitted for chest pain and dyspnea. Six weeks prior, he had similar symptoms with a right heart catherization (RHC) showing normal hemodynamics. Endomyocardial biopsy did not show cellular or AMR. On admission, he was found to be in cardiogenic shock SCAI stage D with severe bi-ventricular failure. RHC confirmed elevated right and left-sided filling pressures and low cardiac output (cardiac power output 0.7). Biopsy showed grade 1R cellular rejection and infiltrative quilty lesions with focal endocardial inflammation and C3d positive capillaries. He was intubated for hypoxemic respiratory failure and an Impella 5.0 was placed for left ventricular unloading and tissue perfusion in addition to milrinone and epinephrine drips. Labs were significant for new class II DSA (DQ7, DR53 immunodominant antibodies). He was treated with intravenous solumedrol, plasmapheresis, and antithymocyte globulin-rabbit (rATG, Thymoglobulin). He received multiple sessions of bortezomib infusion with plasmapheresis while supported with Impella 5.0. He improved over the following three weeks and was extubated with explant of the Impella 5.0 on day 16. His DSA's continued to decrease and graft function improved with ejection fraction up to 50% (Figure 1). He was discharged with plans for further bortezomib and plasmapheresis sessions. <h3>Summary</h3> This case demonstrates the successful treatment of cardiogenic shock precipitated by AMR with an Impella 5.0 as a bridge to recovery.

BDUMP: A Rare Case of Vision Loss in a Lung Transplant Recipient

Introduction Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a potentially devastating rare paraneoplastic syndrome often associated with severe vision loss occurring in patients with underlying carcinoma. In approximately 50% of cases this may be the first sign of an underlying primary malignancy. First described in 1966 there are currently less then 100 cases described in the literature. Treatment of this condition includes treatment of the primary malignancy and plasmapheresis. We describe what we believe to be the first case reported in a lung transplant recipient. Case Report A 72 year old woman status post bilateral lung transplant for COPD was referred to ophthalmology due to increased vision loss for four months, one month prior to presentation she had undergone a left YAG capsulotomy. Maintenance immunosuppression included tacrolimus, mycophenolate mofetil, and prednisone. Fundus exam was remarkable for bilateral retinal pigment epithelium mottling and leopard spot fundus. Exam on the left showed edema. BDUMP was diagnosed, she was admitted to the hospital for plasmapheresis and an extensive evaluation for underlying malignancy. Remarkable laboratory studies showed a new acute kidney injury in the setting of chronic kidney disease with nephrotic range proteinuria. All tumor markers, ANA, ANCA, SPEP, and PLA2R were negative. CT of the head, chest, abdomen, and pelvis were unrevealing, PET scan showed a hypermetabolic breast nodule which was biopsied. The biopsy was consistent with ductal carcinoma in situ, and her mycophenolate was stopped. She completed 14 sessions of plasmapheresis and had improvement in her vision. Summary BDUMP, a rare condition which left untreated can lead to rapid vision loss, is associated with carcinomas of the cervix, uterus, ovary, lung, kidney, pancreas, breast, gastrointestinal tract, and Hodgkin lymphoma. While the exact pathogenesis for BDUMP has not yet been described it has been shown that these patients have a factor in the IgG fraction of serum that selectively causes melanocyte proliferation and thus plasmapheresis is an effective treatment. Patients that are immune suppressed are at an elevated risk for cancer, therefore it's highly important to keep up with health maintenance examinations in this population. Visual changes after transplant are also common, and a complete ophthalmologic examination should be completed with new onset of symptoms.