ECMO for the immunocompromised pediatric patient: Our experience

Objectives: To present a series of immunosuppressed patients (oncohematological disease, congenital immunosuppression, hematopoietic stem cell (HSCT), and solid organ transplant) assisted on ECMO. Method(s): Descriptive, retrospective study (2011-2020) of a cohort of 9 immunosuppressed patients, supported on ECMO. Medical records were reviewed and demographic, clinical, and analytical variables were collected. Result(s): In our series of 9 patients, 5 were male, the median age was 8 years [RIC 3-11 years]. Considering the underlying disease, 6 were oncologic, 1 liver transplant and 2 with congenital immunodeficiency after HSCT. 4 were under active chemotherapy (median 6 days after the last cycle [RIC 5-188]). 6 were admitted due to acute respiratory failure, 3 due to hemodynamic instability (3/9), (one septic shock). The median PEEP was 12 [RIC 9-15] and FiO2 100% (81-100%). 78% (6) required vasoactive drugs (median inotropic score 35 [RIC 0-75]. 40%. 5 had severe neutropenia and/or plateletopenia in the 24 hours prior to ECMO, and alterations in acid-base balance (median pH 7. 1 [RIC 6.9-7.15]. 5 were on multiorgan failure. TPrimary ECMO transport was performed in 4 patients (44%). Cannulation was peripheral in 80% (57% cervical, 43% femoral) and central in 20%;70% VA-ECMO. Median time of assistance was 15 days [RIC 3.5-31.5] in cardiac ECMO (4), and 29 days [RIC 13.5-42] and in pulmonary ECMO (n=5). The median total time of admission was 45 days [RIC 27-59]. 9 had an infection, 2 COVID after HSCT, and 8 bleeding complications, but only one required surgical revision. Renal replacement therapy was used in 5 (median 9 days [RIC 5-34.5]). Other therapies used were polymyxin hemadsorption(2), intratracheal surfactant(2), plasma exchange(1), infusion of mesenchymal cells(1) and specific memory T lymphocytes(2). 4 patients died, 5 survived decannulation, 2 died later, with an overall survival rate to hospital discharge of 33% (3/9). Conclusion(s): Despite having a worse prognosis, ECMO can increase survival in immunosuppressed patients, in situations that are challenging and require a multidisciplinary approach.

The Impact of Therapeutic Plasma Exchange on Inflammatory Markers and Acute Phase Reactants in Patients with Severe SARS-CoV-2 Infection.

Background and Objectives: Due to the poor prognosis and the very high mortality rate associated with severe SARS-CoV-2 infections, various regimens have been tried to stop the evolution of the inflammatory cascade, such as immunomodulatory therapy and plasma clearance of the acute phase reactants involved. Therefore, the objective of this review was to analyze the effects of using therapeutic plasma exchange (TPE), also known as plasmapheresis, on the inflammatory markers of critically ill COVID-19 patients admitted to the intensive care unit (ICU). Materials and Methods: A thorough scientific database search was performed, and it included a review of articles published on PubMed, Cochrane Database, Scopus, and Web of Science from the beginning of the COVID-19 pandemic in March 2020 until September 2022 that focused on the treatment of SARS-CoV-2 infections using plasma exchange for patients admitted to the ICU. The current study included original articles, reviews, editorials, and short or special communications regarding the topic of interest. Results: A total of 13 articles were selected after satisfying the inclusion criterion of three or more patients enrolled with clinically severe COVID-19 that were eligible for TPE. From the included articles, it was observed that TPE was used as a last-resort salvage therapy that can be regarded as an alternative treatment method when the standard management for these patients fails. TPE significantly decreased the inflammatory status as measured by Interleukin-6 (IL-6), C-reactive protein (CRP), lymphocyte count, and D-dimers, as well as improving the clinical status measured with PaO2/FiO2 and duration of hospitalization. The pooled mortality risk reduction after TPE was 20%. Conclusions: There are sufficient studies and evidence to show that TPE reduces inflammatory mediators and improves coagulation function and the clinical/paraclinical status. Nevertheless, although it was shown that TPE decreases the severe inflammatory status without significant complications, the improvement of survival rate remains unclear.

Research priorities for therapeutic plasma exchange in critically ill patients.

Therapeutic plasma exchange (TPE) is a therapeutic intervention that separates plasma from blood cells to remove pathological factors or to replenish deficient factors. The use of TPE is increasing over the last decades. However, despite a good theoretical rationale and biological plausibility for TPE as a therapy for numerous diseases or syndromes associated with critical illness, TPE in the intensive care unit (ICU) setting has not been studied extensively. A group of eighteen experts around the globe from different clinical backgrounds used a modified Delphi method to phrase key research questions related to "TPE in the critically ill patient". These questions focused on: (1) the pathophysiological role of the removal and replacement process, (2) optimal timing of treatment, (3) dosing and treatment regimes, (4) risk-benefit assumptions and (5) novel indications in need of exploration. For all five topics, the current understanding as well as gaps in knowledge and future directions were assessed. The content should stimulate future research in the field and novel clinical applications.

Therapeutic Plasma Exchange in Adult Patients with COVID-19 and Severe Pneumonia: Single Center Experience of Eighty Patients

Aim: Therapeutic plasma exchange (TPE) is a frequently discussed treatment modality in severe coronavirus disease 2019 (COVID-19) patients. It requires an apheresis device and experienced personnel for the application. In this study, we aimed to reveal the characteristics and clinical outcomes of adult patients with COVID-19 who experienced TPE. Material(s) and Method(s): Adult patients who had undergone TPE in our apheresis unit were retrospectively analyzed and COVID-19-positive cases were included in the study. All the medical information about the cases was obtained from the electronic database and technical details of the procedures were gathered from apheresis unit records. Result(s): A total of 80 patients with a median age of 60 (19-85) years were included in the study. Severe pneumonia was present in 98.8% (n=79) of the cases. More than three-quarters of the patients had lymphopenia, critically elevated C-reactive protein (CRP), and D-dimer, and 41.0% (n=32) had high ferritin. The median length of stay in the intensive care unit was 26 (5-124) days. The mortality rate observed on the 14th and 28th days following the TPE procedure was 51.3% (n=41) and 75.0% (n=60), respectively. High ferritin level, multiple organ failure (MOF), and intubation were parameters found to be associated with mortality in the multivariate analysis. Conclusion(s): The mortality rate observed in patients with COVID-19 who underwent TPE in our study was similar to the cases in the literature without the procedure, while it has been shown that high ferritin levels, intubation, and the presence of MOF increase the risk of mortality.Copyright © 2023, Duzce University Medical School. All rights reserved.

The efficacy of therapeutic plasma exchange in COVID-19 patients on endothelial tightness in vitro is hindered by platelet activation.

Coronavirus disease (COVID)-19 is characterised in particular by vascular inflammation with platelet activation and endothelial dysfunction. During the pandemic, therapeutic plasma exchange (TPE) was used to reduce the cytokine storm in the circulation and delay or prevent ICU admissions. This procedure consists in replacing the inflammatory plasma by fresh frozen plasma from healthy donors and is often used to remove pathogenic molecules from plasma (autoantibodies, immune complexes, toxins, etc.). This study uses an in vitro model of platelet-endothelial cell interactions to assess changes in these interactions by plasma from COVID-19 patients and to determine the extent to which TPE reduces such changes. We noted that exposure of an endothelial monolayer to plasmas from COVID-19 patients post-TPE induced less endothelial permeability compared to COVID-19 control plasmas. Yet, when endothelial cells were co-cultured with healthy platelets and exposed to the plasma, the beneficial effect of TPE on endothelial permeability was somewhat reduced. This was linked to platelet and endothelial phenotypical activation but not with inflammatory molecule secretion. Our work shows that, in parallel to the beneficial removal of inflammatory factors from the circulation, TPE triggers cellular activation which may partly explain the reduction in efficacy in terms of endothelial dysfunction. These findings provide new insights for improving the efficacy of TPE using supporting treatments targeting platelet activation, for instance.

The Role of Monoclonal Antibodies in Contrasting Cases of Norse

1. A 42-year-old female developed status ten days following admission for alcohol-related liver disease. MRI brain showed symmetrical medial temporal high signal. No cause was identified. Though the convulsive seizures settled, focal seizures persisted. A second MRI showed extensive multi-lobar signal change, presumed inflammatory in nature. Pulsed methylprednisolone and plasma exchange were ineffective. Tocilizumab was administered ten weeks following onset of seizures. Sequential MRIs showed resolution of inflammatory changes. The patient was discharged to rehabilitation-Modified Rankin Score 3. 2. A 79-year-old female presented with convulsive NORSE, 24hours after first dose of Pfizer COVID vaccine. She had a background of vascular dementia. The patient never recovered her GCS. Convulsive seizures were replaced by epilepsy partialis continua. Sequential MRIs showed diffuse left parietal cortical high signal. An inflammatory aetiology was presumed, pulsed methylprednisolone and the ketogenic diet (3 months) were ineffective. Anakinra was administered on week twelve. Subsequent MRIs showed progression of confluent white matter disease, now bi-hemispheric. She remains symptomatic, a year post presentation. We hypothesise that both patients had NORSE arising from an unidentified inflammatory aetiology. Age and premorbid function are known to influence recovery. Early use of monoclonal antibodies may be beneficial, including in those with systemic disease.

A Case of Sars-Cov-2 Omicron Variant Associated Acute Encephalomyelitis

With the ongoing pandemic of SARS-CoV-2 many neurological complications in relation to COVID-19 infection as well as immune-mediated and vaccine-associated phenomena have been described. To our knowledge, there has been no publication of a case of SARS-CoV-2 Omicron variant associated acute encephalomyelitis. We present a case of a 73-year-old woman with no relevant Background history who is otherwise fit and well and fully vaccinated. She suffered from mild COVID symptoms and had a positive PCR test with presumptive Omicron variant on day 2. Five days into her respiratory illness she developed in quick suc- cession sensory disturbances of hands and feet, bilateral asymmetric flaccid leg weakness, and mild arm weakness. She had absent deep tendon reflexes in the legs and diminished deep tendon reflexes in the right arm. MRI of brain and spine showed signal changes in the brainstem, cervical and low thoracic cord in keeping with acute encephalomyelitis. Her CSF showed an inflammatory picture with raised protein of 1.27g/L and no cells. At the time of submission, the patient received treatment with five days of intravenous steroids followed by ongoing plasma exchange and no comment on treatment response can be made at this stage.

Renal Thrombotic Microangiopathy: A Review.

Thrombotic microangiopathy (TMA), a pathological lesion observed in a wide spectrum of diseases, is triggered by endothelial injury and/or dysfunction. Although TMA lesions are often accompanied by clinical features of microangiopathic hemolytic anemia, thrombocytopenia, and ischemic end-organ injury, renal-limited forms of TMA are not infrequently encountered in clinical practice. The presence of renal-limited manifestations can be diagnostically challenging, often delaying the initiation of targeted therapy. Prompt investigation and empirical treatment of TMA is warranted to reduce associated morbidity and mortality. Major advances have been made with respect to the pathophysiology of primary TMA entities, with the subsequent development of novel diagnostic tools and lifesaving therapies for diseases like thrombotic thrombocytopenic purpura and complement-mediated TMA. This article will review the clinical presentation and pathologic hallmarks of TMA involving the kidney, and the disease-specific mechanisms that contribute to the endothelial injury that characterizes TMA lesions. Diagnostic approach and both empirical and disease-specific treatment strategies will be discussed, along with the potential role for emerging targeted disease-specific therapies.

Triple Positive Antiphospholipid Antibody Syndrome in Pregnancy with High Frequency Plasma Exchange: A Case Report.

Introduction: Triple antibody positive antiphospholipid syndrome during pregnancy carries a poor prognosis. The placental vasculature is particularly vulnerable to these antibodies resulting in a marked increased risk of fetal growth restriction, placental infarction, abruption, stillbirth, and preterm severe preeclampsia. Case Presentation: We report a case of a primigravida with triple antibody positive antiphospholipid syndrome that demonstrated placental insufficiency and fetal compromise at a previable gestation. The patient underwent plasma exchange every 48 h for 11 weeks resulting in delivery of a viable infant. Placental blood flow was improved after complete absence of end-diastolic flow in the fetal umbilical artery. Conclusion: Scheduled plasmapheresis every 48 h can be considered in select cases of antiphospholipid antibody syndrome.

Comparison of outcomes and costs of extracorporeal blood purification therapies in critically ill COVID-19 patients.

INTRODUCTION: Plasmapheresis and hemoperfusion are used against cytokine release syndrome in COVID-19. This study aims to compare their outcomes, costs, and side-effects. METHODS: Survival, costs and side-effects were compared in intensive care unit (ICU) patients receiving plasmapheresis (n = 49), hemoperfusion (n = 20), or none (n = 107), followed until death or discharge. RESULTS: Plasmapheresis survival time was higher than hemoperfusion or controls (HR = 0.764, p = 0.397 and HR = 0.483, p = 0.002, respectively), although the latter diminished after controlling for age and disease severity (p = 0.979). There was no significant difference in ICU costs for plasmapheresis and hemoperfusion (p = 0.738) while both costed more than controls (both p < 0.001). Hypocalcemia and thrombocytopenia incidence did not differ between two groups (p = 0.124 and p = 0.389, respectively) while being higher than controls in plasmapheresis (both p < 0.001) and hemoperfusion (p < 0.001 and p = 0.056, respectively). CONCLUSION: Plasmapheresis and hemoperfusion do not differ significantly in patient survival, ICU costs and side-effects with a higher incidence of hypocalcemia and thrombocytopenia compared witcontrols.

Acute Kidney Injury after Covid 19 Infection in Chronic Kidney Disease Patients of Bangladesh: Retrospective Study

Introduction: COVID 19 pandemic has caused unprecedented devastation worldwide. Spectrum of Covid 19 illness is wide and variable. Risk of mortality is increased in chronic kidney disease patients, during coronavirus disease. CKD is an independent risk factor for poor outcome. AKI is also common in COVID-19 patients who are hospitalized. This study was undertaken to see the outcome of Covid-19 infection in CKD patients. Method(s): This retrospective observational study was carried out in the Kidney Foundation Hospital and Research Institute, Bangladesh from January 2021 to July 2022. One hundred CKD patients who were on regular follow up in the outpatient department and developed COVID-19 as confirmed by reverse transcription polymerase chain reaction (RT-PCR) test underwent chart review after they consented to be part of the study. Their clinical parameters, treatment regiments and laboratory investigations were noted in a data collection sheet. Data was analyzed by Statistical Analysis Software. Result(s): The mean age of the patients was 55.2 years. Of them 43% were female. Diabetes mellitus was the most common comorbidity, seen in 65% of the patients. 24% were CKD stage 4 or 5 prior to the onset of COVID-19, rest were of earlier stage. Hospitalization was required in 65.3% patients;41.1% required oxygen, steroid given in 19.8% patients,8.4% required ICU transfer. 7 patients died, all of respiratory failure. Treatment with antiviral, biologics like Tocilizumab and plasma exchange was not commonly done. AKI developed in 28% of the patients during the course of the illness. Males were more prone to develop AKI (p = 0.23). People with longer duration of symptoms had higher incidence of AKI (p < 0.0001). AKI incidence did not vary according to baseline eGFR (p = 0.16). Among those who developed AKI, 17.9% required temporary dialysis and 7.1% went on to develop end stage kidney disease. Interim outcomes such as hospitalization, oxygen requirement, ICU transfer and death did not vary according to development of AKI. Conclusion(s): People with chronic kidney disease and other comorbid conditions are at higher risk for more serious COVID-19 illness. In our study it has been shown that a significant proportion of CKD patients developed AKI after COVID 19 infection of which a number of patients develop end stage kidney disease and required renal replacement therapy. No conflict of interestCopyright © 2023

Guillain-Barre syndrome following SARS-CoV-2 vaccination: Is there a real association?

Background: Guillain-Barre Syndrome (GBS) is the most common cause of acute flaccid paralysis, with an incidence of 0.81-1.89 cases per 100,000. With the SARS-CoV-2 virus pandemic, major international vaccination campaigns continue to be carried out to minimize the total burden of the disease. This study aims to report a case series of consecutive GBS patients after SARS-CoV-2 vaccination during the massive campaign in Mexico in 2021. Method(s): A single-center, observational study of consecutive GBS subjects diagnosed by Asbury criteria from January 1 to August 31, 2021. Including GBS-related symptoms on or after six weeks of vaccination record, both first and second doses. Result(s): From a total of 53 GBS patients, eight had a history of SARS-CoV-2 vaccination, 87.5% male, the median vaccination-symptom onset and symptom-to-admission time were 15 (IQR 12.75-23.25), and 3.5 (IQR 1.5-8.25), all of them had GBS Disability Scale >=3 at admission. Acute inflammatory demyelinating polyneuropathy (AIDP) was the most common electrophysiological variant encountered in this population. All patients received treatment Intravenous Immunoglobulin (IVIG) or Plasma Exchange (PE), 62.5% recovered independent walk at three months follow up. Conclusion(s): The annual incidence of GBS cases associated with vaccination remains lower (0.81 - 1.89 cases / 100,000 persons) than non-vaccinated patients;this should encourage health authorities to continue promoting massive vaccination as benefits outweigh the risks.Copyright © 2021

New-onset neuromyelitis optica spectrum disorder in a patient with COVID-19 and chronic Hepatitis B co-infection

Background: Neurological autoimmune disorders are often triggered by bacterial and viral infections, with growing evidence supporting coronavirus disease 2019 (COVID-19) infection precipitation of these disorders. COVID-19 is already implicated in causing discrete para-infectious neurological syndromes: acute disseminated encephalomyelitis (ADEM), transverse myelitis, neuromyelitis optica spectrum disorders (NMOSD), Guillain-Barre syndrome (GBS), and is also associated with encephalopathy, acute cerebrovascular disease, neuromuscular disorders, and seizures. Case Presentation: We describe a case of a 43-year-old Asian woman with chronic Hepatitis B (HBV) co-infected acutely with COVID-19, presenting with urinary retention, bilateral blindness, thoracic sensory level, and quadriparesis. Extensive workup narrowed down her diagnosis as seronegative NMOSD. She had complete resolution of symptoms after treatment with concurrent plasma exchange (PLEX), high dose corticosteroids, and emtricitabine-tenofovir. Follow-up visit showed no seroconversion at 6 months and no relapses. Conclusion(s): Our literature review highlights the likely link between COVID-19 infection and the development of neurologic autoimmune diseases. Our literature review supports a virus-triggered immune-mediated process rather than neuro-invasion. Many viral illnesses have been linked to the development of NMOSD and anti-AQP4 antibody-related myelitis. Additionally, there is limited literature linking chronic HBV infection with the development of optic neuritis and speculation thatcross-reactivity between HBsAg and myelin antigens may lead to the development of demyelinating diseases in the CNS and PNS. We observed remarkable clinical improvement after treatment with alternating days of IV methylprednisolone and therapeutic PLEX.Copyright © 2022

Serum negative neuromyelitis optica spectrum disorder after vaxzevria vaccination: A case report

Background: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease, targeting the central nervous system, rarely associated with vaccination. Case report: We report a case of a 47 year-old healthy woman who presented, ten days after the first dose of SARS-CoV-2 Vaccine AstraZeneca (Vaxzevria), with back pain, tetraplegia, urinary retention, dysarthria and dysphagia. The patient was diagnosed NMOSD. She underwent intravenous-corticosteroids, vein-Immunoglobulin and plasma-exchange without significant improvement . Conclusion(s): The absence of any possible related conditions, the temporal relation with anti-SARS-CoV-2 vaccination, suggest that, in our case, NMOSD may be due to the cross reaction from Vaxzevria.Copyright © 2021 The Author(s)

The Importance of CMV Reactivation in COVID-19-Related ARDS Patients

Objectives: Cytomegalovirus (CMV) reactivation is a significant cause of morbidity and mortality in critically ill patients. Existing or newly developed immunosuppression appears to be the main factor for reactivation. COVID-19 patients with acute respiratory distress syndrome can be affected by a variety of conditions that cause immunosuppression. Clarifying CMV reactivation and notably its predictive features became important during the epidemic. Method(s): This is a retrospective, observational, and cohort study. All COVID-19 patients admitted to the ICU between March 11, 2020 and March 11, 2021 were analyzed. All of the information was gathered from the hospital's electronic records. CMV reactivation was defined as CMV DNA >=1000 copies/ml in tracheal samples. The patient population was analyzed in two groups, namely, patients with CMV reactivation and patients without reactivation. Result(s): During the study period, 99 of all COVID-19 ARDS patients fulfilled the inclusion criteria, and CMV reactivation was detected in 55 (55.6%) of them. Age, BMI, APACHE-II score, hypertension, chronic respiratory disease, the usage of interleukin blockers, the duration of steroid usage, procalcitonin (PCT), and CD-8 T-cell levels differed significantly from the patients without CMV reactivation. Furthermore, the reactivation group had longer ICU stays, longer durations of mechanical ventilation, and higher mortality. Conclusion(s): CMV can be reactivated in critically ill COVID-19 ARDS patients, which appears to correlate with worse outcomes. Obesity, the usage of IL-blockers and steroids >12 days, high PCT, and low CD-8 T-cell levels appear to be risk factors. Critically ill COVID-19 patients should be closely monitored with regard to immunosuppression and CMV status.Copyright © 2022 by The Cardiovascular Thoracic Anaesthesia and Intensive Care.

Repeat Doses of Sotrovimab for Recurrence of COVID-19 in a High-Risk Pregnancy

Background: Myasthenia gravis (MG) is an autoimmune disorder leading to variable degrees of skeletal muscle weakness. During pregnancy, infections can trigger exacerbations and should be treated promptly and aggressively.(1) Sotrovimab is a monoclonal antibody used as monotherapy in high-risk, symptomatic non-hospitalized patients at risk of developing COVID-19 disease. (2) It is thought to have retained activity against SARS-CoV-2 omicron variant. (3) Limited data are available on its use in pregnancy. Case: A 39-year-old woman with severe generalized MG, was referred to our joint neuro-obstetric multidisciplinary service. Her two previous pregnancies were complicated by severe exacerbations of MG necessitating intensive care admissions, and preterm labour. Her long-term therapy included high dose steroids, intravenous immune globulin (IVIG) and plasma exchanges. In this pregnancy, she additionally received rituximab in the first-trimester, allowing her prednisolone to be weaned to 20 mg daily, with ongoing 3-weekly IVIG. She received 3 doses of the Pfizer COVID-19 vaccine. At 19 weeks she developed mild coryzal symptoms, sore throat and myalgia. Lateral flow and polymerase chain reaction tests in the community confirmed infection with SARS-CoV-2. She was treated with sotrovimab with uneventful recovery at home. At 31 weeks, she again tested positive for SARS-CoV-2, after reporting mild COVID-19 symptoms. She received a second dose of sotrovimab and had a quick recovery. Subsequent SARS-CoV-2 genotyping indicated she had contracted the Omicron-BA.2 variant. Fetal surveillance for growth (SARS-CoV-2) and arthrogryposis (MG) did not raise concerns. At 35+3 weeks, she went into spontaneous labour and was delivered by caesarean section for evolving chorioamnionitis, with uneventful recovery for mother and baby. Discussion(s): We report a case of repeated treatment with sotrovimab (in second and third trimesters) of a high-risk, non-hospitalized pregnant woman, who was re-infected with SARS-CoV-2. We identified no immediate maternal, fetal or neonatal complications following two doses of sotrovimab for mild COVID-19.

Guillain-Barre syndrome associated with SARS-CoV-2 infection: A case series from 4 Colombian cities during the pandemic

SARS-CoV-2 infection has been associated with multiple neurological manifestations. One such manifestation, which has been described since the early stages of the COVID-19 pandemic and is relevant for current neurological practice, is Guillain-Barre syndrome (GBS). The literature describes neurotoxic mechanisms of the virus itself and the possible pathways by which it may affect the peripheral nerves in experimental studies;however, we still lack information on the mechanisms causing the immune response that gives rise to GBS in the context of SARS-CoV-2 infection. Colombia is one of the Latin American countries worst affected by the pandemic, with the third-highest number of cases in the region;thus, it is essential to recognise GBS, as this potential postinfectious complication may severely compromise the patient's functional status in the absence of timely diagnosis and treatment. We present a series of 12 cases of GBS associated with SARS-CoV-2 infection from hospitals in 4 different Colombian cities and describe the clinical presentation, laboratory and electrophysiological study findings, and treatment.Copyright © 2022 Sociedad Espanola de Neurologia

Clinical Analysis of 15 Adult Patients with Thrombotic Thrombocytopenic Purpura

Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy characterized by severe thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia ascribable to platelet-rich microthrombi. TTP is related to a severe deficiency in ADAMTS13, the specific von Willebrand factor-cleaving metalloprotease. The aim of our study is to analyze the clinical characteristics, treatment and outcome of patients with TTP. Method(s): We retrospectively analyzed 15 patients with TTP treated Farhat Hached hospital, Sousse, Tunisia, from2004 to 2021. Result(s): Among the 15 patients, there were 7 males and 8 females, with a median age of 45,5 (30~72) years. Two of them had evolutive cancer and 1 had covid-19 vaccination 3 months earlier. Twelve patients had neurological presentations (80%), 4 had mucocutaneous bleeding and 2 had fever. Biology showed thrombopenia, mechanical hemolytic anemia in all patients and renal failure in 2 patients. No abnormalities in coagulation tests were detected. The ADAMTS13 activity was performed in only 3 patients (Due to test unavailability) showing a level <=10% in all 3 patients. All patients were treated with plasma exchange/ plasma infusion + glucocorticoid, combined with rituximab in 2 cases. Ten patients showed complete remission, 1 relapsed within the first year, 2 died and 2 others were lost to follow-up. Discussion/Conclusion: Most TTP patients presented with the triad of microangiopathic anemia, thrombocytopenia, and neurologic abnormalities and improved with plasma therapy.