Plasmapheresis Therapy Research Articles

Neuropsihijatrijski lupus - dva lica jedne bolesti (prikaz dva slučaja)

Introduction: Neuropsychiatric lupus (neuropsychiatric systemic lupus erythematosus - NPSLE) is a form of a systemic autoimmune disease - systemic lupus erythematosus (SLE), with dominant central and peripheral nervous system involvement. Nuclear magnetic resonance imaging (NMRI) of the brain is considered the gold standard for diagnosing patients with NPSLE. Treatment of NPSLE includes the following: high doses of corticosteroids, methylprednisolone pulse therapy, intravenous immunoglobulins, plasmapheresis, immunosuppressants (cyclophosphamide, azathioprine, mycophenolate mofetil), and biological therapy - rituximab. Case reports: In this paper, we present two patients diagnosed with neuropsychiatric lupus, with different clinical manifestations of the disease, as well as the diagnostics performed, and the good clinical outcomes of immunosuppressive therapy achieved in both patients. Conclusion: The pathogenesis of NPSLE involves neuroinflammatory (autoimmune) and ischemic mechanisms. Neuroimaging has shown good results in differentiating patients with SLE from controls. The goal of SLE treatment is to achieve remission or low disease activity and to prevent episodes of exacerbation. Treatment with intravenous cyclophosphamide as well as with systemic corticosteroids has shown significantly positive results in patients with NPSLE. There is great need, in the future, for new biomarkers in the serum and cerebrospinal fluid (CSF), as well as for more innovative radiological procedures. Additional clinical trials that would lead to new therapeutic options for the treatment of NPSLE are necessary.

The effect of plasmapheresis therapy on management of patients with snake bite

Objective: The aim of the study was to evaluate the effectiveness of plasmaferesis therapy in patients with snake bite who were admitted to intensive care unit in the setting of tertiary referral hospital. Materials and Methods: The retrospective study involved 114 adult patients with snake bite who were admitted to a referral hospital’s intensive care unit between January 2012 and December 2022. The patients were divided into four groups according to the treatments. Group AV performed antivenom (Group AV) alone. Group PP performed plasmapheresis alone. Group AV+PP performed antivenom and plasmapheresis. Group GST performed only general supportive therapy. Results: Fifty two of 114 were included in Group GST, 31 in Group AV, 18 in Group PP and 13 in Group AV+PP. APACHE score, SOFA score, GCS, stage of the bite, length of stay in the intensive care unit, acute kidney injury, and hematological disorders were higher while the platelet count at admission was lower in Group PP and Group AV+PP compared to Group AV and Group GST (p< 0.05). Conclusion: Antivenom and plasmapheresis are not alternatives to each other, antivenom should be performed to patients according to the severity of the bite, and plasmapheresis should be performed without delay in those with severe hematological effects.

Efficacy and adverse effects of insulin versus plasmapheresis in patients with hypertriglyceridemia-3-induced acute pancreatitis: a systematic review and meta-analysis.

Hypertriglyceridemia is a common cause of acute pancreatitis (AP). This literature review compared the effectiveness and adverse events of insulin therapy, with or without heparin, and plasmapheresis, in reducing triglyceride levels in patients with hypertriglyceridemia-induced AP. Systematic reviews, meta-analyses, evidence syntheses, editorials, commentaries, protocols, abstracts, theses and preprints were excluded. Review Manager was used to conduct the meta-analysis. The literature search yielded 2765 articles, but only 5 were included in the systematic review and meta-analysis and the total number of participants in the review was 269. From this study's analysis, insulin ± heparin was more successful in reducing triglyceride levels than plasmapheresis (standardized mean difference -0.37, 95% confidence interval [CI] 0.99 to 0.25; P=0.25). Insulin ± heparin therapy had a lower mortality rate than plasmapheresis (risk ratio [RR] 0.70, 95%CI 0.25-1.95). Hypotension, hypoglycemia, and acute renal failure were less common in the plasmapheresis therapy group than in insulin ± heparin therapy (RR 1.13, 95%CI 0.46-2.81, RR 3.90, 95%CI 0.45-33.78, and RR 0.48, 95%CI 0.02-13.98 for hypotension, hypoglycemia, and acute renal failure, respectively). This study found no significant difference in mortality between insulin ± heparin therapy and plasmapheresis used for the reduction in triglyceride levels. It is notable that no substantial differences were observed in the most common side-effects encountered during these therapies, thus indicating non-inferiority.

Comparison of the Effects of Plasmapheresis, Hemoperfusion, and Convalescent Plasma Therapy on Inflammatory Factors in COVID-19 Patients.

Background: Since the emergence of coronavirus disease 2019 (COVID-19), the treatment protocols are continuously updated, based on the evidence gathered all around the world and reported to the World Health Organization. Like many other emerging infectious diseases, using convalescent plasma from those recovered from the disease was a preliminary treatment approach that showed partial effectiveness for severe COVID-19 patients. Besides, blood filtration strategies, such as hemoperfusion and plasmapheresis, are employed to lessen the load of inflammatory molecules. However, few studies compared their effects to conclude which treatment might be more efficacious for COVID-19 patients. We compared the effects of plasmapheresis or plasma exchange, convalescent plasma therapy, and hemoperfusion on O2 saturation and inflammatory factors in COVID-19 patients. Methods: In this retrospective study, 50 COVID-19 patients received standard treatments based the international guidelines. Patients were divided into 4 groups: hemoperfusion, plasmapheresis, plasma therapy, and control. The control group received only the standard treatments. The mortality rate, O2 saturation, and laboratory factors were compared between the 4 groups. Results: We found a significant decrease in the C-reactive protein level following hemoperfusion (32.75 ± 23.76 vs 13 ± 7.54 mg/dL; p = 0.032) but not plasmapheresis and plasma therapy. Besides, serum levels of lactate dehydrogenase (p = 0.327, 0.136, 0.550, for hemoperfusion, plasmapheresis, and plasma therapy, respectively) and other inflammatory molecules did not significantly change following treatments. There is also no significant difference in the mortality rate between the treatment groups (p = 0.353). Conclusion: It seems that hemoperfusion, plasmapheresis, and plasma therapy did not have considerable effects on decreasing the inflammation and mortality rate compared with standard treatment.

397 HEART FAILURE WITH REDUCE VENTRICULAR EJECTION FRACTION FOLLOWING THERAPY WITH RITUXIMAB IN A PATIENT AFFECTED BY MPO ANCA RELATED MICROSCOPIC POLYANGITIIS

Abstract Introduction Rituximab is a chemotherapeutic drug widely used for the treatment of oncologic and rheumatologic diseases. Its cardiotoxic profile has not yet been defined in detail; only a few isolated case reports delineate cardiovascular adverse reactions following its use. In the cases described in literature, the drug was used to treat neoplastic diseases, often in combination with other chemotherapeutic drugs. We present a clinical case of heart failure with reduced ventricular ejection fraction (HFrEF), developed following therapy with Rituximab and a single administration of Cyclophosphamide, in a patient affected by MPO-ANCA-related Microscopic Polyangiitis. Case Report 54-year-old man arrived at the emergency department for weight loss, fever, asthenia, and lower limbs oedema; laboratory findings where diagnostic for acute kidney injury (AKI). His past history was unremarkable. He was admitted to the nephrology department where he was diagnosed with MPO-ANCA-related microscopic polyangiitis. Echocardiography on admission showed normal left systolic ventricular function. He underwent diuretic therapy, intermittent hemodialysis, plasmapheresis and immunosuppressive therapy with steroids, Rituximab (two administration of 375 mg/m2 i.v. one week apart) and Cyclophosphamide (one administration of 300 mg i.v.). Following the achievement of good clinical and hemodynamic compensation, the patient was discharged. However, 5 days later, the patient came back to the hospital, prior to the follow-up that was scheduled for the continuation of immunosuppressive therapy, with symptoms of worsening dyspnea related to acute pulmonary edema. A transthoracic echo showed severe left ventricular systolic dysfunction for global hypokinesia. Cardiac MRI showed hypokinetic dilated cardiomyopathy with severely depressed systolic function (EF 30%) in the absence of lesions compatible with edema and myocardial fibrosis/necrosis. No coronary artery disease was detected with CT coronary angiogram. Given the absence of signs of ischemic cardiopathy or inflammatory related cardiomyopathy, we considered this new onset of HFrEF likely due to cardiotoxicity from chemotherapy drugs, which, in agreement with nephrology colleagues, were discontinued. The patient was started on heart failure medication with Bisoprolol 2,5 mg once daily and Ramipril 2,5 b.i.d., mineralocorticoid receptor antagonist and glifozines were not administered due to the renal injury. Given the HFrEF and numerous features of non-sustained ventricular tachycardia found on EKG monitoring during hospitalization, the patient was discharged with a wearable ICD. He was discharged after 3 weeks in stable clinical conditions with a follow-up appointment in a month. Conclusion Our clinical case provides further evidence regarding the cardiotoxic effect of Rituximab. In contrast with the other cases in literature, Rituximab was used for the treatment of rheumatologic and non-neoplastic pathology, without being combined with several other chemotherapeutics but only with a low-dose administration of Cyclophosphamide. In light of the above, it is worth mentioning that when chemotherapeutics are used, not only in oncology but also in rheumatology, even when their cardiotoxicity profile has not been defined in detail, as in the case of Rituximab, it is essential to use the utmost caution in assessing cardiac function before and after drug administration, so that early detection of cardiotoxicity will be possible.

A Rare Case of Acute Hemolytic Anemia in a Patient with Newly Diagnosed Multiple Myeloma: Maintaining a Fine Balance between Occam's Razor and Hickam's Dictum

AbstractAnemia is a common feature in multiple myeloma and is multifactorial. A 52-year-old lady was admitted to our hospital with complaints of fatigue, exertional dyspnea, paresthesia, and a recent-onset confusion state. Fundus examination revealed features of hyperviscosity. The patient received 2 units of packed red blood cell transfusion (PRBC) before the present hospital admission. Laboratory investigations revealed severe anemia and thrombocytopenia. The M-protein was 5.8 g/dL. Bone marrow showed sheets of plasma cells. Immunofixation electrophoresis confirmed the presence of an IgAλ band. FISH was positive for IgH-FGFR3 fusion. The investigations confirmed multiple myeloma R-ISS stage III. The patient was immediately started on CyBorD chemotherapy regimen. The patient had indirect hyperbilirubinemia and symptomatic anemia. Initial testing of the patient's sample showed blood grouping discrepancy with DCT, ICT, and auto control positive. The symptomatic anemia persisted requiring PRC transfusions. Further antibody study revealed the presence of anti-Jka antibody—a warm IgG antibody and cold antibody. Subsequently, the patient received Jka antigen-negative B-positive compatible PRBC transfusions and the hemoglobin normalized. Our patient had transfusion-associated alloimmunization along with hyperviscosity. The timely recognition and early institution of plasmapheresis and myeloma-directed therapy along with transfusion of compatible Jka antigen-negative PRBC lead to rapid improvement.

A Case Report of Three Guillain-Barré Syndrome During SARS-CoV-2 Pandemic: Promising Outcome in a Patient with History of Multiple Sclerosis

Neurologic complications in patients with coronavirus disease 19 (COVID-19) are common particularly in hospitalized patients. Guillain–Barré syndrome (GBS) is a rare complication of COVID-19. Most patients with GBS and COVID-19 presented with progressive, ascending limb weakness evolving over one to four days. There are some reported cases of GBS, mostly in form of case reports or case series, and also a comprehensive review on this topic. However, none of these cases experienced multiple sclerosis (MS) as an underlying disease. So, in this manuscript we reported three cases of COVID-19 induced GBS; one of them with history of MS and promising outcome after receiving five sessions of plasmapheresis and corticosteroid therapy.

Lack of Efficacy and Safety of Eculizumab for Treatment of Antibody-Mediated Rejection Following Renal Transplantation

We evaluated the efficacy and safety of eculizumab in comparison with plasmapheresis and intravenous immunoglobulin therapy in renal transplant recipients diagnosed with antibody-mediated rejection (AMR). This was a multicenter, open-label, prospective, randomized analysis. The patients were randomized by therapy type (eg, eculizumab infusions or standard of care [SOC]: plasmapheresis/intravenous immunoglobulin). The patients (ie, eculizumab arm: 7 patients, SOC arm: 4 patients) were evaluated for the continued presence of donor-specific antibodies (DSAs) and C4d (staining on biopsy), as well as histologic evidence, using repeat renal biopsy after treatment. The allograft biopsies revealed that eculizumab did not prevent the progression to transplant glomerulopathy. Only 2 patients in the SOC arm experienced rejection reversal, and no graft losses occurred in either group. After AMR treatment, the DSA titers generally decreased compared to titers taken at the time of AMR diagnosis. There were no serious adverse effects in the eculizumab arm. Eculizumab alone cannot treat AMR effectively and does not prevent acute AMR from progressing to chronic AMR or transplant glomerulopathy. However, it should be considered as a potential alternative therapy because it may be associated with decreased DSA levels.

ACUTE DISSEMINATED ENCEPHALOMYELITIS POST SARS-COV-2 VACCINATION

Many of the central and peripheral nervous system complications related to severe acute respiratory syndrome coronavirus (SARS-CoV-2) infection have been recently described. An efficient mass vaccination program is required in order to effectively reduce the spreading of the infection and, therefore, minimize long-term sequelae, including neurological ones. Nonetheless, as more patients are accessing the 2019 coronavirus (COVID-19) vaccines, it is important to report potential adverse events. For this reason, we report here, the case of a patient with no remarkable medical history, having developed acute disseminated encephalomyelitis (ADEM) 3 weeks after being vaccinated against COVID-19 Diagnostic criteria for possible autoimmune encephalitis were fulfilled. Our patient showed a good response to plasmapheresis therapy after a failure of corticosteroid one. The Complications of post-vaccination encephalitis following SINOPHARM vaccination still seem to be very rare, but should be diagnosed and treated appropriately.

Neuromyelitis Optica Spectrum Disorder (Nmosd): A Rare Report

Neuromyelitis Optica spectrum disorder (NMOSD), also known as Devic disease, is a chronic disorder of the brain and spinal cord dominated by inflammation of the optic nerve (optic neuritis) and inflammation of the spinal cord (myelitis). We present the case of a 25-year-old female patient who presented with complaints of lower back ache, decreased vision and double vision, generalized body pain, neck pain and vomiting. On examination, Extraocular Movement (EOM) showed right gaze restriction with nystagmus; her serum Neuromyelitis Optica (NMO) result was positive; and her MRI showed lesions in the brain. Insight of her presenting illness and investigations, she was successfully treated with IV steroids, plasmapheresis, and immunosuppressive therapy and was neurologically stable. We present this rare case to clinically enlighten the diagnosis and treatment of NMOSD.

Incidence and clinical characteristics of hypertriglyceridemic acute pancreatitis: A retrospective single-center study.

BACKGROUNDThe incidence of hypertriglyceridemic acute pancreatitis (HTG-AP) has increased yearly, but updated population-based estimates on the incidence of HTG-AP are lacking. Reducing serum triglyceride (TG) levels quickly is crucial in the early treatment of HTG-AP. Decreased serum TG levels are treated by non-invasive methods, which include anti-lipidemic agents, heparin, low-molecular weight heparin, and insulin, and invasive methods, such as blood purification including hemoperfusion (HP), plasmapheresis, and continuous renal replacement therapy. However, authoritative guidelines have not been established. Early selection of appropriate treatment is important and beneficial in controlling the development of HTG-AP.AIMTo evaluate the effect between patients treated with intravenous insulin (INS) and HP to guide clinical treatment.METHODSWe retrospectively reviewed 371 patients with HTG-AP enrolled in the Department of Fujian Provincial Hospital form April 2012 to March 2021. The inpatient medical and radiologic records were reviewed to determine clinical features, severity, complications, mortality, recurrence rate, and treatment. Multivariate logistic regression analyses were used to analyze risk factors for severe HTG-AP. Propensity score matching was used to compare the clinical outcomes of INS and HP.RESULTSA total of 371 patients met the HTG-AP criteria. The incidence of HTG-AP was increased by approximately 2.6 times during the 10 years (8.4% in April 2012-March 2013 and 22.3% in April 2020-March 2021). The highest incidence rate of acute pancreatitis was observed for men in the age group of 30-39 years. The amylase level was elevated in 80.1% of patients but was only three times the normal value in 46.9% of patients. The frequency of severe acute pancreatitis (26.9%), organ failure (31.5%), rate of recurrence (32.9%), and mortality (3.0%) of HTG-AP was high. Improved Marshall score, modified computed tomography severity index score, baseline TG, baseline amylase, C-reactive protein (CRP), albumin, aspartate aminotransferase, low-density lipoprotein cholesterol, urea nitrogen, creatinine, calcium, hemoglobin, free triiodothyronine, admission to intensive care unit, and mortality were significantly different between patients with different grades of severity (P < 0.050). Multivariate logistic regression analysis confirmed that high CRP [P = 0.005, odds ratio (OR) = 1.011, 95%CI: 1.003-1.019], low calcium (P = 0.003, OR = 0.016, 95%CI: 0.001-0.239), and low albumin (P = 0.023, OR = 0.821, 95%CI: 0.693-0.973) were risk factors of severe HTG-AP. After propensity score matching adjusted by sex, age, severity of HTG-AP, and baseline TG, the serum TG significantly decreased in patients treated with INS (P < 0.000) and HP (P < 0.000) within 48 h. However, the clearance rate of TG (57.24 ± 33.70% vs 56.38 ± 33.61%, P = 0.927) and length of stay (13.04 ± 7.92 d vs 12.35 ± 6.40 d, P = 0.730) did not differ between the two groups.CONCLUSIONThe incidence of HTG-AP exhibited a significant increase, remarkable severity, and recurrent trend. Patients with mild and moderately severe acute pancreatitis can be treated effectively with INS safely and effectively without HP.

Two PerPLEXing Cases of Hashimoto’s Encephalopathy Unresponsive to Steroid and Intravenous Immunoglobulin Therapy

Hashimoto’s encephalopathy is a nebulous entity over which much controversy exists. Often referred to as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), it describes a myriad of neurological sequelae that typically are observed to occur in patients with the presence of thyroid antibodies. We aim to raise clinical awareness of this seldom diagnosed entity as a potential etiology for altered mental status in patients who present with supporting clinical features and elevated thyroid antibodies. While steroid responsiveness is deemed a typical presenting feature of this medical condition, our cases aim to describe two cases that required escalation of therapy to intravenous immunoglobulins, and ultimately, plasmapheresis therapy for improvement in their clinical status. Our patients had a dramatic improvement in their mentation within three to four sessions of plasmapheresis, improving rapidly toward their baseline. Such a dramatic improvement, coupled with the corresponding reduction in their thyroid antibody titer supports the diagnosis of Hashimoto's encephalopathy and highlights the importance of having a low clinical threshold for the diagnosis of this entity in patients who, despite extensive evaluation, reveal no apparent cause for their altered mental status.

Immunomodulatory Treatment of Lyell's Syndrome: A Simultaneous Plasmapheresis and Intravenous Immunoglobulins Therapy.

Lyell's syndrome, or toxic epidermal necrolysis (TEN) is a rare but life-threatening condition. It manifests with blistering of skin and mucous due to subepidermal bullae and keratinocyte necrosis. In most cases, it is an immune response to drugs or their metabolites. The mortality in TEN is high despite optimal infection and wound control. There are no unequivocal treatment guidelines in TEN. Immunosuppressive treatment may increase the wound infection risk and mortality. The aim of the study was to evaluate a 10-year experience with immunomodulatory therapy in TEN. We perform a combination of plasmapheresis and intravenous immunoglobulins to control the disease. There were 35 patients in the group and we performed a post hoc evaluation. Twenty-eight patients received the full protocol and there were seven patients who did not complete the treatment (single therapy group). The mortality in the test group was 14.29%, and the difference reached statistical significance in comparison with the single therapy group (P &lt; .05). Our protocol reduced the mortality risk five times. Our study proved that simultaneous plasmaphereses with intravenous immunoglobulins administration were safe and improved patients' outcome in TEN.

Robotic-assisted minimally invasive thymectomy for myasthenia gravis with thymoma.

Robotic-assisted minimally invasive thymectomy for myasthenia gravis with thymoma.

Aluminum Phosphide Toxicity

Background: Aluminum phosphides (ALPs) are still being used in developing countries as a pesticide despite the fact that ingestion and/or inhalation can cause significant morbidity and mortality. The generation of phosphine when ALP comes in contact with water leads to the blockade of electron transfer, which inhibits oxidative phosphorylation and in turn, cellular respiration in multiple organ systems. Clinical Description: A 3-year-old Turkish girl and her family were inadvertently exposed to overnight inhalation intoxication after a tablet of ALP was dissolved in water and used as a pesticide for Isot pepper, a local spice used in their household. The child presented with rapidly progressive respiratory distress and coma. The other members displayed mild respiratory symptoms. Management: At the time of admission, she was in altered sensorium, tachycardic, tachypneic, hypoxic, and in shock. She was immediately intubated and intermittent positive pressure ventilation started. Shock was managed as per the standard protocol. The child emanated garlic odor breath, had sluggishly reactive bilateral mitotic pupils. She had cardiorespiratory arrest within 30 min of intubation and could be revived. Despite supportive treatment, multiorgan dysfunction syndrome developed cardiac, pulmonary, renal, hepatic, coagulopathy, hyperglycemia, and metabolic acidosis. She succumbed to another cardiorespiratory arrest before plasmapheresis and continuous renal replacement therapy could be started in an attempt to eliminate the toxins. Consent for autopsy was not given. Conclusions: ALPs poisoning still occurs in developing countries and is a leading cause of mortality. The most common cause of death within the first 24 h is cardiovascular collapse.

Successful sequential extracorporeal multiple organ support therapy in COVID-19 pneumonia: A case report

Patients with severe COVID-19 pneumonia can develop acute respiratory distress syndrome, which triggers respiratory failure. Multiple Organ Support Therapies (MOST), such as Extra Corporeal Membrane Oxygenation (ECMO), Continuous Renal Replacement Therapy (CRRT) or plasmapheresis, can be used as part of adjunctive management in patients with refractory hypoxemia even with the appropriate use of optimal mechanical ventilation and pharmacological measures. We describe a case of a 31-year-old man who, upon admission, is managed with mechanical ventilation and due to poor evolution, it is decided to start ECMO therapy within the first 24 hours of admission; subsequently, management with CRRT with oXiris filter and plasmapheresis is initiated in the first three days of hospitalization. The patient presents a rapid clinical improvement and a significant decrease in inflammatory markers. CRRT with oXiris filter has been associated with a significant reduction in cytokine and endotoxin levels; while plasmapheresis therapy also eliminates inflammatory mediators. Management with ECMO has shown benefits as salvage therapy in patients with hypoxemia. The success of these cases depends largely on the early onset of ECOS in the critically ill patient.

CLINICAL CASE OF COVID-ASSOCIATED GUILLAIN-BARRE SYNDROME

The aim of our article is to shed light on the problem of the development of Guillain-Barre syndrome after COVID-19 infection&#x0D; Materials and methods&#x0D; The main research methods we used were scientific research and the actual description of our own clinical case. Laboratory research methods, magnetic resonance imaging, and electromyography were used to confirm the diagnosis and perform differential diagnostics.&#x0D; Results.&#x0D; In 2021, the Center for Acute Neurology and Pain Treatment treated 12 patients with Guillain-Barre syndrome. Guillain-Barre syndrome (GBS) is an acute autoimmune inflammatory polyradiculoneuropathy. The clinical picture of GBS includes progressive motor and sensory disorders in the extremities and pain.&#x0D; We bring to your attention a clinical case of GBS in patient B., who was treated at the clinical hospital "Feofania". Patient B., 32 years old, was hospitalized in the Feofania Clinical Hospital with complaints of weakness in her arms and legs, inability to walk, foreign body sensation in the throat, difficulty swallowing, chest discomfort, and difficulty breathing. A few weeks before the disease, the patient was treated for a mild SARS-CoV-2 infection. The patient's condition gradually deteriorated by day 25 of the disease, despite plasmapheresis, intravenous immunoglobulin and glucocorticoid therapy. However, from day 29, the condition gradually improved and on re-examination after 2 months, the patient remained mild distal paresis of the leg muscles (up to 4 points), decreased tendon reflexes from the lower extremities. Disorders of sensitivity and function of the pelvic organs were not observed.&#x0D; Conclusion.&#x0D; In our article, we present a case of COVID-19-associated GBS with a classic clinical picture, increased peripheral paresis, stabilization, and gradual recovery of all functions (respiration, pelvic function, motor, sensory, and reflex) through pathogenetic treatment and follow-up rehabilitation measures.

Optic neuritis secondary to the Pfizer-BioNTech-162b2 COVID-19 vaccine managed with plasmapheresis: A case report and review

The COVID‑19 pandemic has had a significant public health impact globally, with inoculation now paramount in limiting the spread of the disease. Although the safety and efficacy profiles of COVID‑19 vaccines are well documented, it is upon the medical community to be aware of adverse effects to appropriately advise and treat patients. We report the case of a 39‑year‑old female who was hospitalized with optic neuritis (ON) 1 week after her second dose of the Pfizer‑BioNTech (BNT162b2) COVID‑19 vaccine. The patient did not have any significant medical history, including personal or familial history of multiple sclerosis or any other demyelinating disease, other than prior COVID‑19 infection. She had since made a full recovery and tested negative at the time of hospitalization for ON. Over the course of her admission, the patient's visual acuity (VA) deteriorated from 20/400 to no light perception. After methylprednisolone was ineffective, she was started on plasmapheresis (PLEX) therapy which resulted in mild improvement of VA. To our knowledge and after a thorough PubMed literature search, this is the first reported case of isolated ON associated with COVID‑19 vaccination.

Stevens-Johnson syndrome and toxic epidermal necrolysis: a systematic review and meta-analysis.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and severe skin and mucosal reactions that are associated with high mortality. Despite the severity, an evidence-based treatment protocol for SJS/TEN is still lacking. In this systematic review and meta-analysis, the PubMed database was searched using the following terms: [Stevens-Johnson syndrome] OR [toxic epidermal necrolysis] AND [therapy] OR [treatment] over a 20-year period (1999-2019) in the German and English language. All clinical studies reporting on the treatment of SJS/TEN were included, and epidemiological and diagnostic aspects of treatment were analysed. A meta-analysis was conducted on all comparative clinical studies that met the inclusion criteria. A total of 88 studies met the inclusion criteria, reporting outcomes in 2647 patients. Treatment was either supportive or used systemic corticosteroid, intravenous immunoglobulin, plasmapheresis, cyclosporine, thalidomide or cyclophosphamide therapy. The meta-analysis included 16 (18%) studies, reporting outcomes in 976 (37%) patients. Systemic glucocorticoids showed a survival benefit for SJS/TEN patients in all analyses compared with other forms of treatment. Cyclosporine treatment also showed promising results, despite being used in a small cohort of patients. No beneficial effects on mortality could be demonstrated for intravenous immunoglobulins. Glucocorticoids and cyclosporine may be tentatively recommended as the most promising immunomodulatory therapies for SJS/TEN, but these results should be investigated in future prospective controlled trials.

First diagnosis of thrombotic thrombocytopenic purpura after SARS-CoV-2 vaccine \u2013 case report

BackgroundWe report a case of a 25-year-old male patient, who developed acquired thrombotic thrombocytopenic purpura (aTTP) after receiving a first dose of mRNA-based SARS-CoV-2 vaccine Spikevax (mRNA-1273, Moderna Biotech, USA). While this is the first case in literature describing a case of aTTP after receiving the Spikevax vaccine, there are two other cases after mRNA-based Covid-19 vaccine and two after adenoviral SARS-CoV-2 vaccine.Case presentationThe patient presented with persisting malaise, fever, headache, word-finding difficulties, nausea, vomiting, petechial bleeding, and hematuria 13 days after receiving a first dose of vaccination. Laboratory testing showed low platelet count, Coombs-negative hemolytic anemia, and mild acute kidney injury. We excluded vaccine induced immune thrombotic thrombocytopenia (VITT) as another important differential diagnosis and the final diagnosis was established after ADAMTS-13 (A Disintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13) activity was found to be < 1% (reference range > 40%) and ADAMTS-13 antibodies being 72.2 IU/L (reference range < 12 IU/L).We initiated empiric therapy of plasmapheresis and corticosteroids on admission and started caplacizumab the day after. The patient’s thrombocyte count normalized 3 days after admission, hemolysis and acute kidney injury resolved after 2 weeks. The patient received 2 doses of rituximab (1 g each) after the diagnosis of immune TTP was established. One month after the initial presentation, the patient is in good overall condition, but still receives daily caplacizumab due to ADAMTS-13 activity of < 1%.ConclusionsLow platelet count after vaccination against SARS-CoV-2 has gained attraction after vaccine-induced immune thrombotic thrombocytopenia (VITT) has been described as a rare but severe complication of adenoviral-based vaccines. Thrombotic thrombocytopenic purpura (TTP) is an important differential diagnosis, but there are only few reports of TTP following SARS-CoV-2 vaccination. Despite pathophysiological and clinical differences of both entities, diagnostic uncertainty can result in the acute setting, since they share main symptoms such as headache and neurological alterations in addition to thrombocytopenia. In difference to other cases reported, this patient developed first symptoms of TTP as early as 4 days after vaccination, which suggests that vaccination merely acted as trigger for occult TTP, instead of truly inducing an autoimmunological process.