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Objective To analyze the clinical outcomes of a patient with thrombotic thrombocytopenic purpura(TTP) as the starting performance who was considered as systemic lupus erythematosus(SLE) under multiple abnormal autoantibodies, and summarize the treatment experience through the clinical outcomes of plasma exchange combined with high-does steroid pulse therapy. Methods A middle-aged female patient presented with initial symptoms of fever, bleeding and hemolytic thrombocytopenia. Laboratory tests showed decreased activity of thrombospondin type 1 motif no.13(ADAMT13) and abnormal autoantibodies. The diagnosis was considered SLE with TTP, and the patient underwent continuous plasma exchange combined with high-dose steroid pulse therapy. The patient's platelet recovery was dynamically observed. Results The patient responded well to plasma exchange combined with high-does steroid pulse therapy, with platelet count raising to normal in a short period of time and hormone gradually decreasing. There was no recurrence during the follow-up period. Conclusion TTP combined with lupus is not common, but when both occur simultaneously, plasma exchange combined with high-does steroid pulse therapy may effectively alleviate symptoms. This treatment approach can serve as a valuable reference for similar clinical cases.

Autoimmune hepatitis (AIH) is a rare, immune-mediated inflammatory liver disorder, while its acute form has a spectrum of clinical manifestations including acute-icteric AIH, acute severe AIH (AS-AIH) and AS-AIH with acute liver failure (ALF) (1). A subset of patients with dermatomyositis (DM) presents with characteristic skin findings but without muscle involvement, termed clinically amyopathic dermatomyositis (CADM). This subgroup includes patients with amyopathic DM(AMD), who are devoid of all clinical and laboratory findings of muscle involvement (2). We report a case of a previously healthy 54-year-old woman who presented with acute icteric AIH and features of AMD, rapidly progressing to AS-AIH with ALF and the diagnostic and therapeutic challenges encountered while managing the patient.

Abstract Atypical haemolytic uremic syndrome (aHUS) is a rare disorder characterised by complement-mediated thrombotic microangiopathy (TMA). Despite clinical guidelines, the diagnosis and treatment of aHUS in its early stages remains challenging. This study examined the annual trends in aHUS clinical practices in Japan and explored factors influencing early diagnosis and treatment. Using data from the 2011–2020 Diagnosis Procedure Combination database, 3096 cases with the HUS disease code were identified, of which 217 were confirmed as aHUS and treated with eculizumab or plasma exchange. Early initiation, defined as starting eculizumab or plasma exchange within 7 days of admission, was the focus of the study. Our study revealed no significant changes over time in the number of aHUS diagnoses, cases treated with eculizumab, or early initiation cases. Early initiation cases underwent haemodialysis earlier and had ADAMTS13 activity measured earlier, shorter hospital stays, and lower hospitalisation costs than late initiation cases. In conclusion, we found no increase in the number of newly diagnosed aHUS cases or early treatment initiation over time. Early recognition of TMA and differentiation of the causative disease are crucial for identifying potential aHUS cases, which may lead to better patient prognoses.

Background: Therapeutic plasma exchange (TPE) is a highly effective rescue treatment for patients with acute exacerbation of neuroimmunological disease that removes circulating autoantibodies and inflammatory components from the bloodstream. The aims of this study are to explore the safety and the effectiveness of TPE in patients with autoimmune neurological disorders. Methods: We retrospectively evaluated the frequency of adverse events (AEs) and the effectiveness of TPE using the modified Ranking Scale (mRS) in patients with acute neurological flares who underwent TPE at the University Hospital of Palermo. Results: Of 59 patients, the majority underwent TPE due to multiple sclerosis (MS) relapse. In 23.7% of cases, TPE was performed before obtaining a definite diagnosis due to the severity of the clinical presentation. After TPE, the mRS score was globally reduced (p < 0.0001), and this effect was marked in patients with MS, Guillain–Barré syndrome, and myasthenia gravis crisis but not in those with paraneoplastic syndromes. Circulating pathogenetic antibodies, younger age, and the early use of TPE were factors strongly associated with TPE effectiveness. The overall safety profile of TPE was satisfactory with an AE frequency of 15%. Conclusions: These results highlight the early use of TPE in patients with circulating pathogenetic antibodies as well as its favorable safety profile.

Objective To compare the efficacy of double filtration plasmapheresis(DFPP), centrifugal therapeutic plasma exchange(cTPE) and centrifugation-filtration plasmapheresis(CFPP) in improving renal insufficiency after kidney transplantation, as well as the differences in inducing plasma exchange-related adverse reactions. Methods Clinical data from 46 patients who underwent plasma exchange after renal transplantation in our hospital were retrospectively collected, and patiens were divided into DFPP group(n=33), cTPE group(n=7) and CFPP group(n=6). Changes in peripheral blood creatinine, albumin, hemoglobin, platelets, fibrinogen levels and urine volume before and after TPE were compared and analyzed among the three groups. Results Among the DFPP group, cTPE group and CFPP group, the creatinine after TPE decreased by (31.40±25.38)%, (58.91±19.75)% and (39.44±28.64)%, respectively, with cTPE group significantly higher than the DFPP group(P0.05); the urine volume after TPE increased by (49.33±30.03)%, (54.62±39.32)% and (68.89±23.00)%, showing no significant differences(P>0.05); the hemoglobin after TPE decreased by (11.97±5.94)%, (20.17±5.75)% and (9.65±8.75)%, respectively, with the cTPE group significantly higher than the DFPP group and CFPP group(P0.05). The platelet count after TPE decreased by (37.88±18.39)%, (24.56±12.36)% and (21.40±12.51)%, respectively, with no significant differences between the three groups(P>0.05); the fibrinogen after TPE decreased by (0.57±0.20)%, (0.14±0.06)% and (0.26±0.22)%, respectively, with the DFPP group significantly higher than the cTPE group(P0.05); the albumin after TPE decreased by (11.41±5.97)%, (14.67±6.52)% and (25.18±5.10)%, respectively, with cTPE group and DFPP group significantly lower than the CFPP group(P0.05). Conclusion The effect of three plasma exchange methods varies on renal function, anemia and coagulation function of patients after kidney transplantation. It is necessary to consider the the patient’s disease characteristics and treatment needs, as well as the laboratory′s technical conditions and plasma supply when selecting TPE methods.

Introduction. Myasthenia gravis (MG) or Erb-Goldflam syndrome is an autoimmune neuromuscular disease based on autoaggression of humoral immunity against elements of neuromuscular synapses and intracellular structures of muscle fibers (antibodies to acetylcholine receptors (AchR), muscle-specific tyrosine kinase (MuSK), protein 4 related to low-density lipoprotein receptors (LRP4), ryanodine receptors (RyR), titin, and skeletal muscle antigens). The exact causes of autoimmune processes in MG are unknown, but abnormalities of the thymus gland (hyperplasia and neoplasia), especially in patients with antibodies to AchR, as well as genetic predisposition are of indisputable importance [1]. Aim. To analyze the researches of pathogenesis, diagnosis and treatment for patients with severe MG from latest literature sources to optimize the treatment of this group of patients. To analyze the own experience of implementing a patient-oriented approach to the treatment and rehabilitation of MG. Materials and methods. Bibliosemantic, comparative and system analysis methods, and clinical and neurological examination of patients with MG. Results. The course of MG is variable and can be represented by episodic muscle weakness, stationary flow, slow or rapid progression [2]. Currently, the complex therapy of patients with MG has a clear tendency to move from symptomatic (acetylcholinesterase inhibitors, potassium preparations, potassium-sparing diuretics) treatment to pathogenetic, mainly etiopathogenetic concepts of therapy are being formed (immunosuppressive therapy, antigen-specific immunotherapy, monoclonal antibodies), and it is also being clarified effectiveness of early thymectomy [3]. Conclusions. Complex therapy of patients with MG is based on the use of a differential diagnostic algorithm for various pathogenetic variants of MG, which forms a mainly etiopathogenetic concept of therapy. Antigen-specific immunotherapy, aimed at restoring tolerance to the attacked autoantigen by targeting only the damaged part of the immune system, while leaving the rest intact, is considered more promising for the treatment of MG. MG treatment should be carried out in the conditions of a specialized neurological center, where a full examination and targeted pathogenetic therapy can be carried out.

Objective To statistically analyze the perioperative results of patients with ABO-incompatible kidney transplantation (ABOi-KT), in order to explore the changes in blood group antibody of type-A/B recipients. Methods A total of 33 cases of blood group A/B ABOi-KT recipients in our hospital from January 2021 to October 2023 were recruited and divided into two groups of group A(n=18) and group B(n=15) according to the different blood types of recipient. The effects of preoperative plasmapheresis on antibody titer, antibody rebound and renal function after operation(serum urea nitrogen, creatinine and estimated glomerular filtration rate on the 1st, 3rd, 7th and 14th day) were analyzed between the two groups. According to the postoperative rebound of blood type antibodies, 33 recipients were divided into antibody rebound group(n=7) and non rebound group(n=26), and the differences in initial blood type antibody titers between the two groups were analyzed. Results There was no significant difference in the clearance rate of IgM with preoperative plasma exchange between the two groups (Z=-0.26, P>0.05); Levels of serum urea nitrogen and creatinine on the 1st, 3rd, 7th and 14th day after operation between group A and group B were not statistically significant(P>0.05), the same as eGFR. Group B was more prone to rebound antibody compared with group A (P0.05) between the two groups was found. Conclusion The patients type B receiving type AB kidney donors are more prone to rebound antibody after ABOi-KT operation compared to the the patients type A receiving type AB.

Objective To analyze the characteristics of blood transfusion consultation cases and establish the consultation route, so as to provide reference for blood transfusion doctors to participate in blood transfusion consultation practice. Methods The cases involved in clinical transfusion consultation in the blood transfusion department of our hospital from 2020 to 2023 were collected from the hospital information system (HIS), and then classified by department and consultation type to summarize the main points of transfusion consultation, formulate transfusion consultation routes, and conduct typical cases analysis. Results There were 315 clinical transfusion consultations from 2020 to 2023, with an increasing trend year by year (26 in 2020, 67 in 2021, 81 in 2022, 141 in 2023). The consultations involved 24 departments, including cardiovascular medicine 14.0%(44/315), orthopedics 12.7%(40/315), intensive care medicine 8.9%(28/315), general medicine 8.3%(28/315), cardiopulmonary disease 6.0%(19/315), etc. There were 8 categories of consultations, including 35.6%(112/315) autologous ozonized blood transfusion, 23.8%(75/315) plasma exchange, 14.9%(47/315) perioperative mass blood preparation (transfusion), 11.4%(36/315) platelet- rich plasma therapy and 6.3%(20/315) autologous blood collection, etc. The clinical blood transfusion consultation route was formulated according to the consultation points. Six patients with various diseases were treated by blood transfusion department. With effective treatment measures taken, all of them improved and were discharged. Conclusion The summary of key points of clinical blood transfusion consultation and formulation of the blood transfusion consultation route by department of blood transfusion are conducive to the implementation of blood transfusion consultation and guarantee the safety of patients.

Abstract Background Despite the efficacy and safety of DIGIFab, it is relatively expensive and has limited availability. In addition, alternative interventions, such as therapeutic plasma exchange, may need to be considered in massive digoxin overdoses. Although few case reports describe its efficacy. Case presentation We report a case of a 17-year-old white male patient brought by family members to our emergency department in Riyadh, Saudi Arabia. After intentionally ingesting 48 mg of digoxin tablets to commit suicide, the patient’s initial digoxin serum level was 8.04 ng/mL. The patient was resuscitated in the emergency department. After admission to the intensive care unit, the patient underwent therapeutic plasma exchange, because of insufficient DIGIFab doses. Afterward, the serum digoxin levels drastically decreased, and his symptoms reverted. The patient was successfully managed and discharged 7 days after admission. Conclusion Despite insufficient evidence and a limited number of case reports describing the use of extracorporeal treatment in digoxin overdose, we noted the significant impact of therapeutic plasma exchange on our patient. However, therapeutic plasma exchange’s use in routine treatment requires stronger evidence to confirm its benefits.

Objective Rapidly progressive interstitial lung disease (RP-ILD) is a frequent and serious manifestation of idiopathic inflammatory myopathy (IIM) associated with poor outcomes. Plasma exchange (PE) can quickly remove pathogenic substances from the blood. Therefore, PE may be efficacious in IIM patients who have elevated levels of autoantibodies, cytokines and chemokines, fighting for time for immunosuppressive therapy. However, the value of adding PE to immunosuppressants remains unclear. The purpose of this study was to determine the short-term outcomes, including the survival rate at 6 months and change of the laboratory data, of PE in combination with immunosuppressants and/or biologics in the treatment of IIM-RP-ILD.Methods We searched PubMed, Embase and Cochrane Library to find reports of interest published from inception to March 4, 2024. STATA 15.1 was used for data analysis. A fixed or random-effects model with inverse-variance weighting was used to estimate the pooled risk ratio (RR) and 95% confidence interval (CI).Results Two hundred and thirty studies were identified. Eleven studies, including five retrospective cohort studies, four case-control studies and two case series, were included. PE was performed on 114 patients. The survival rate at 6 months was 80% (95%CI = 64%–92%), with moderate heterogeneity (I2=63.45%, p

Abstract* A 20’s primiparous woman, following spontaneous expulsion of intrauterine death of the fetus at 30 weeks of gestation, presented on post-partum day 8 with acute onset flaccid quadriparesis and breathing difficulty, which had rapidly progressed to involve the legs on day 3 up to her upper limbs on post-partum day 5. Following examination, Guillain Barre Syndrome (GBS) with ascending diaphragmatic involvement was diagnosed, and plasma exchange was initiated. She developed raised blood pressure, headache, sudden onset visual loss with 2 episodes of generalized seizures on post-partum day 14. Brain MRI and clinical suspicion helped diagnose Posterior Reversible Encephalopathy Syndrome (PRES). The patient was treated with anticonvulsants and antihypertensive agents. She regained her vision over the next two days, completed the treatment for GBS, and made a good recovery with independence for advanced activities of daily living on follow-up.

Leptospirosis is an infection with multiorgan involvement and a high mortality rate. Extreme hyperbilirubinemia in leptospirosis has been reported to exert multiple cellular toxic effects on kidney and liver cells. We describe a young man with leptospirosis who had extreme hyperbilirubinemia, acute liver injury, coagulopathy, hepatic encephalopathy, and pulmonary haemorrhages. Early initiation of therapeutic plasma exchange (PEX) dramatically improved patient’s clinical and biochemical parameters. We conclude that, in addition to the mortality benefit provided by PEX in severe pulmonary hemorrhage syndrome in leptospirosis, PEX is also beneficial in reducing systemic toxic insults of hyperbilirubinemia.

Plasma exchange (PE) is effective in a part of autoimmune diseases, and its main action mechanisms include removing pathogenic factors from the patient's blood, regulating immune function, replenishing normal plasma components. The use of PE in the treatment of severe immune-related skin diseases, such as pemphigus vulgaris, toxic epidermal necrolysis and clinically amyopathic dermatomyositis, has become more widespread. This review provides an overview of the progress of PE application in severe immune-related skin diseases at home and abroad in recent years to provide new ideas for the treatment of clinical severe skin diseases.

Objective To investigate the effect of double plasma molecular adsorption system and sequential half-dose plasma exchange (DPMAS+HPE) on the short-term survival rate of patients with hepatitis B associated acute-on-chronic liver failure (HBV-ACLF). Methods Data on HBV-ACLF cases hospitalized in our hospital from January 1, 2015 to December 31, 2022 were retrospectively collected, and were divided into standard comprehensive medical treatment group and DPMAS+HPE group according to different treatment methods. Propensity score matching (PSM) was used to eliminate inter group confounding bias. The baseline data and improvement of laboratory indicators after treatment between two groups were compared. Death related risk factors in HBV-ACLF patients were screened by logistic regression analysis, and cumulative survival rates at 30 and 90 days between the two groups were compared by Kaplan-Meier survival analysis. Results A total of 373 cases of HBV-ACLF were included in this study. Among them, 136 cases in the treatment group received DPMAS+HPE once on the basis of comprehensive internal medicine treatment, and 237 cases only received comprehensive internal medicine treatment. After PSM, 136 patients were included as the control group. The decrease in alanine aminotransferase (ALT), aspartate aminotransferase (AST), and total protein (TP) in the treatment group before and after treatment was significantly greater than that in the control group (446.5 vs 159.0, 317.0 vs 92.0,5.2 vs 0.3), with statistically significant difference (P

Vasculitis occurs frequently in systemic lupus erythematosus (SLE) patients with an active disease and poor prognosis. Nervous system vasculitis – specifically, multiple mononeuropathy, seizures, and transverse myelitis – is among the most severe complications in SLE that need timely aggressive therapy. Unfortunately, no robust literature is available to guide their management, and therapeutic recommendations are commonly based upon other autoimmune conditions or case reports, case series, and expert opinions. We hereby present a case of a 27-year-old female patient with SLE that sequentially developed an asymmetric distal axonal sensorimotor neuropathy in her limbs and digital ischemic lesions of the hands, that progressed despite maximal treatment with standard therapies, thus requiring several sessions of plasmapheresis.

Background: Yellow phosphorous (YP) is a protoplasmic poison that is mainly used as a rodenticide. Aims and Objectives: The aim of the study is to describe the clinical profile of YP poisoning in children in a tertiary care hospital. Materials and Methods: This is a cross-sectional study done in a tertiary care hospital in Tamil Nadu. A convenience sample of all the cases admitted during the year 2022 was done. Demographic and clinical data were collected and analyzed. Results: The male: female ratio was 1.16:1 (n=13). The mean age of the children was 4.0769±1.373 years. About 84.61% of the children reported to the hospital within 6 h. On admission, fever (30.76%), vomiting (30.76%), abdominal pain (30.76%), bleeding (15.38%), and altered sensorium (15.38%) were the common symptoms. All the children ingested YP accidentally. Leukocytosis was observed in about 30.76%. The mean peak serum aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels were 516.9231±685.976 IU/L and 161.9231±215.107 IU/L, respectively. There were no alterations in the Electrocardiographic tracings, levels of serum electrolytes, blood glucose, and serum creatinine in children with YP poisoning. Significant differences were observed between children who survived and children who died in the levels of mean peak AST, mean peak ALT, INR, and in the presence of encephalopathy and bleeding manifestations. Conclusion: YP is an uncommon but lethal poison in children. Creating awareness among parents about the dangers of YP and nationwide prohibition of the sale of YP will be an important step in eliminating deaths in children who are accidental victims.

Coronavirus disease 2019 (COVID-19) is associated with a variety of neurologic manifestations. Acute necrotizing encephalopathy (ANE) is a rare, life-threatening complication characterized by rapid deterioration of neurologic status following viral infection, such as influenza or human herpesvirus 6. Since the COVID-19 pandemic, a rise in ANE cases associated with the infectious disease has been reported in adult patients. We present a case of COVID-19-associated ANE in a 9-year-old boy. The patient experienced 3 days of fever and mild respiratory symptoms, followed by lethargy. Magnetic resonance imaging on day 4 showed hyperintensity in the bilateral thalami, midbrain, pons, hypothalamus, and cerebellum, along with some areas of hemorrhage. From the imaging findings, ANE was strongly suspected, leading to the initiation treatment involving a 5-day course of remdesivir and multiple immunomodulator therapies, including high-dose corticosteroids, intravenous immunoglobulin, tocilizumab, and 10 cycles of therapeutic plasma exchange. Subsequently, the patient gradually improved, experiencing only minor neurological sequelae and showing favorable radiologic improvement. In COVID-19-infected patients presenting neurologic symptoms, it is crucial to promptly suspect and investigate unexplained encephalopathy using neuroimaging. Early administration of immunomodulator therapy is vital for the diagnosis and optimizing clinical outcomes.

Abstract Background The incidence of acute liver failure from herpes simplex virus is rare. Case presentation A 71-year-old Japanese man was diagnosed with acute liver failure and was transferred to our hospital. Steroid therapy, plasma exchange, and hemodiafiltration were started for liver failure, and antimicrobial therapy was initiated for pneumonia. Staphylococcus epidermidis was detected in blood culture. Skin rash appeared; a positive anti-herpes simplex virus result led to the diagnosis of acute liver failure from herpes simplex virus. Hence, acyclovir was started. After blood tests improved, treatments for acute liver failure were discontinued. Antimicrobial therapy was continued; however, he died. In this case, persistent bacteremia and drug-induced liver damage due to acyclovir may have contributed to his death. Conclusions Acute liver failure can lead to complications and death. Thus, careful observation is crucial, even if the patient has shown some improvements.

AbstractObjectives To appraise whether plasma exchange (PLEX) effectively improves visual function for acute optic neuritis (ON) in neuromyelitis optica (NMO) or neuromyelitis optica spectrum disorder (NMOSD).Methods and analysis We searched Medline, Embase, Cochrane Library, ProQuest Central, and Web of Science to identify relevant articles published between 2006 and 2020.Eligible studies were in English and evaluated visual outcomes for people with acute ON in NMO or NMOSD treated with PLEX. They also had adequate pre- and posttreatment data. Excluded were studies with 1 or 2 case reports, or incomplete data.Results Twelve studies were qualitatively synthesized (1 RCT; 1 controlled NRSI; 10 observational studies). Five before-and-after observational studies were used for quantitative synthesis. The PLEX in the 5 studies (3 to 7 cycles over 2 to 3 weeks) was performed as second-line or adjunctive therapy for acute ON in NMO/NMOSD.The qualitative synthesis revealed that visual-acuity recovery occurred between one day and 6 months after the first PLEX cycle completion. Thirty-two of 48 participants in the 5 quantitative-synthesis studies received PLEX. Relative to pre-PLEX values, visual-acuity improvements were nonsignificant at these post-PLEX time points: 1 day (SMD 0.611; 95% CI −0.620 to 1.842); 2 weeks (SMD 0.0214; 95% CI −1.250 to 1.293); 3 months (SMD 1.014; 95% CI −0.954 to 2.982); and 6 months (SMD 0.450; 95% CI −2.643 to 3.543).Conclusions There were inadequate data to determine whether PLEX effectively treats acute ON in NMO/NMOSD.

Key Clinical Message Thrombotic microangiopathies are a side effect of anti‐VEGF therapies, which are often limited to the kidneys but can also occur systemically and be life‐threatening. Screening for increasing proteinuria is essential. Abstract We present the case of a 65‐year‐old male patient with a multifocal HCC, Barcelona clinic liver cancer (BCLC) classification B at the time of diagnosis. The HCC was treated with nine sessions of transarterial chemoembolization (TACE), and after a progress, the therapy was switched to a combination of atezolizumab and bevacizumab. Five months after therapy change, he presented with an acute kidney injury. The histopathology of the renal biopsy showed findings of a thrombotic microangiopathy (TMA), which we treated with 12 sessions of therapeutic plasma exchange in combination with steroids, resulting in a decreased TMA activity and later in a remission of the TMA. This case suggests the importance of monitoring the kidney function and proteinuria in patients under anti‐vascular endothelial growth factor (VEGF) therapy and shows a rare differential diagnosis for a worsening of kidney function in these patients. Furthermore, it shows that therapeutic plasma exchange might be a valuable therapeutic option for patients with TMA due to anti‐VEGF therapy.

Sepsis is a potentially lethal condition that occurs when the body's response to infection damages tissue and organs. The production of inflammatory mediators typically assists in defending the body against infection; however, an overreaction to inflammation can cause coagulation problems, vascular endothelial damage, and organ hypoperfusion. Blood purification methods, such as plasmapheresis, can effectively remove inflammatory mediators from plasma. The purpose of this meta‐analysis was to explore the efficacy of plasma exchange for sepsis treatment as noted in recent studies. The authors searched the Pubmed (Medline), Cochrane Central Register of Controlled Trials (The Cochrane Library), Embase (Ovid), and Scopus databases and included controlled clinical studies that compared plasmapheresis or plasma filtration with conventional treatment in patients with severe sepsis. The Newcastle–Ottawa Scale literature quality assessment tool was used to assess the risk of bias. The primary study outcome was all‐cause mortality. The random effects model was adopted for conducting the meta‐analysis. Among the 1013 records found, the study included 5 trials, all of which carried a low risk of bias. The use of plasmapheresis was associated with a longer stay in the intensive care unit (odds ratio [OR], 0.85, 95% confidence interval [CI], 0.39–1.32, heterogeneity [I2] = 0%), a significant reduction in all‐cause mortality (OR, 0.54, 95% CI, 0.33–0.89, I2 = 70%), and reduced mortality (OR, 0.29, 95% CI, 0.13–0.67, I2 = 0%) in adults; the results for children differed from this (OR, 0.79, 95% CI, 0.36–1.72, I2 = 89%). Four trials reported no adverse events; one trial reported an adverse event related to plasma exchange, including an instance of hypotension in one patient. Plasmapheresis appeared to be an effective treatment for patients suffering from sepsis. A large number of additional randomised controlled trials are needed to confirm this finding. [ABSTRACT FROM AUTHOR]

Abstract Background Blood derivatives therapy is a conventional clinical treatment, while the treatment for Alzheimer’s disease (AD) is relatively novel. To provide clinical references for treating AD, this meta-analysis was performed to evaluate the efficacy and safety of blood derivatives therapy on the patients with AD. Methods A systematic articles search was performed for eligible studies published up to December 6, 2021 through the PubMed, Embase, Cochrane library, ClinicalTrials.gov , Chinese National Knowledge Infrastructure database, and Wanfang databases. The included articles were screened by using rigorous inclusion and exclusion criteria. Study selection and data-extraction were performed by two authors independently. Random effects model or fixed effects model was used. Quality of studies and risk of bias were evaluated according to the Cochrane risk of bias tool. All analyses were conducted using Review Manager 5.4. The study was designed and conducted according to the Preferring Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline. Results A total of three plasma administrations (two plasma exchange and one young plasma infusion) and five intravenous immunoglobulin (IVIG) randomized controlled trials with a sample size of 1148 subjects diagnosed with AD were included. There was no significant difference in cognitive improvement and all-cause discontinuation between intervention and placebo groups (RR 1.10, 95% CI 0.79–1.54). And Intervention groups showed not a statistically significant improvement in cognition of included subjects measured by the ADAS-Cog (MD 0.36, 95% CI 0.87–1.59), ADCS-ADL (MD −1.34, 95% CI − 5.01–2.32) and NPI (MD 2.20, 95% CI 0.07–4.32) score compared to the control groups. IVIG is well tolerated for AD patients even under the maximum dose (0.4 g/kg), but it is inferior to placebo in Neuropsychiatric Inventory scale in AD patients (MD 2.19, 95% CI 0.02–4.37). Conclusions The benefits of blood derivatives therapy for AD are limited. It is necessary to perform well-designed randomized controlled trials with large sample sizes focusing on the appropriate blood derivatives for the specific AD sub-populations in the future. Systematic review registration PROSPERO CRD42021233886

Abstract Background Leptospirosis is a common zoonotic infection caused by the spirochete Leptospira. The disease is more prevalent in the tropics, causing subclinical to severe illness leading to high morbidity and mortality. Case presentation A 77-year-old healthy Sri Lankan man presented to the Teaching Hospital Peradeniya with severe leptospirosis complicated with acute kidney injury, pulmonary hemorrhages, myocarditis, and severe thrombocytopenia. He was deteriorating despite treatment with intravenous antibiotics and methylprednisolone boluses. He made a dramatic improvement with two cycles of plasma exchange. Conclusion Therapeutic plasma exchange is a life-saving treatment modality in severe leptospirosis with multiorgan failure.

Kawasaki disease (KD) is a systemic vasculitis and is the most frequent pediatric acquired heart disease in developed countries. The diagnosis of KD is typically made by the ≧ 5 of 6 principal signs. However, approximately 20% of KD patients present with less than 5 of these diagnostic signs but may be suffering from coronary artery aneurysms and have been diagnosed with incomplete KD. In this case report, we describe a 2-month-old infant who showed just fever without any other signs of KD but was suffered from multiple arterial aneurysms, including coronary, pulmonary, and carotid arteries. Because she did not respond to intravenous immunoglobulin infusion, we placed her on plasma exchange that has successfully brought defervescence without any significant complications. This case may represent the end spectrum of incomplete KD in very young infants.

Thrombotic Thrombocytopenic Purpura (TTP) is an acute, rare, potentially life-threatening disorder presenting with thrombocytopenia, hemolytic anemia, and clinical consequences of microvascular thrombosis caused by a deficiency of ADAMS13 [1, 2]. The incidence of acquired TTP is approximately three cases per one million adults per year, based on the Oklahoma TTP-Hemolytic uremic syndrome (HUS) Registry [3]. Here, we present a case of TTP with persistent severely deficient ADAMS13 activity and recurrent relapses. She had a refractory relapse in the third trimester of pregnancy, complicated with superimposed severe preeclampsia and sepsis, and finally expired due to intracranial hemorrhage (ICH).

Abstract Acute fatty liver disease of pregnancy AFLP is an obstetrical emergency, with severe complications that may include death. Management of AFLP is challenging and include plasma exchange which helps to improve the prognosis for both mother and fetus and delay liver transplantation.

Abstract Background Thymoma is an uncommon cancer often associated with myasthenia gravis, an autoimmune disorder of the neuromuscular junction characterized by muscular fatigability. In patients with advanced nonmetastatic thymoma, primary chemotherapy may be required to induce tumor shrinkage and to achieve radical resection. Cancer chemotherapy has been anecdotally reported as a trigger factor for worsening of myasthenia gravis in thymic epithelial cancers. The study of uncommon cases of chemotherapy-related myasthenic crisis is warranted to gain knowledge of clinical situations requiring intensive care support in the case of life-threatening respiratory failure. Case presentation We report a case of an 18-year-old Caucasian woman with advanced Masaoka-Koga stage III type B2 thymoma and myasthenia gravis on treatment with pyridostigmine, steroids and intravenous immunoglobulins, who developed a myasthenic crisis 2 hours after initiation of cyclophosphamide/doxorubicin/cisplatin primary chemotherapy. Because of severe acute respiratory failure, emergency tracheal intubation, mechanical ventilation, and temporary (2 hours) discontinuation of chemotherapy were needed. Considering the curative intent of the multimodal therapeutic program, we elected to resume primary chemotherapy administration while the patient remained on mechanical ventilation. After 24 hours, the recovery of adequate respiratory function allowed successful weaning from respiratory support, and no further adverse events occurred. After 3 weeks, upon plasma exchange initiation with amelioration of myasthenic symptoms, a second course of chemotherapy was given, and in week 6, having documented partial tumor remission, the patient underwent radical surgery (R0) and then consolidation radiation therapy with 50.4 Gy in 28 fractions in weeks 15–20. Conclusions This case report, together with the only four available in a review of the literature, highlights that chemotherapy may carry the risk of myasthenic crisis in patients affected by thymoma and myasthenia gravis. To our knowledge, this is the first reported case of chemotherapy continuation on mechanical ventilation in a patient with chemotherapy-induced myasthenic crisis requiring tracheal intubation. The lesson learned from the present case is that, in selected cases of advanced thymoma, the paradoxical worsening of myasthenia gravis during chemotherapy should not be considered an absolute contraindication for the continuation of primary chemotherapy with curative intent.

COVID-19, formerly coronavirus infection 2019-nCoV, is a potentially severe acute respiratory infection caused by the SARS-CoV-2 coronavirus (2019-nCoV). It is a dangerous disease that can occur both in the form of a mild acute respiratory viral infection and in a severe form with the development of bilateral polysegmental viral pneumonia, specific complications of which may include acute respiratory distress syndrome, resulting in the respiratory failure with a high risk of death. Due to the absence of etiotropic therapy for the coronavirus infection, pathogentic treatment becomes of primary importance. We present a clinical case of a severe form of COVID-19 in a 33-year-old man to whom we administered a new method of pathogenetic treatment of this disease based on immunosuppresiive therapy followed by therapeutic plasma exchange and infusion of non-specific human immunoglobulin.

Aim: Multiple myeloma (MM) patients might require haemodialysis (HD) and/or plasma exchange (PE) in cases of acute kidney injury (AKI) and/or chronic kidney disease (CKD) and/or other indications. The study analysed the survival of MM patients who required HD and/or PE. Subjects and methods: All 144 patients treated for MM at the University Hospital Centre Osijek between 1994 and 2018 (of whom 47.9 % were men) were included in the study. Data were collected from medical records. MedCalc Statistical Software version 17.8.2 was used for the statistical analysis, with significance set at α = 0.05. Results: Forty-three of 144 MM patients (29.9 %) were treated with HD and/or PE. Male patients required HD or PE more often than female patients (62.8 % vs 37.2 %, P = 0.02). Patients who did not require HD or PE were significantly older at the time of their death than the patients treated with HD or PE [75 (interquartile range, IQR, 72 – 77) vs 72 (IQR 66 – 75) years; P = 0.009, Mann-Whitney test]. Among all patients who required acute or chronic HD, PE or a combination of the treatments, the longest life span was found in 17 patients who were treated with chronic HD (median 12 months, IQR 8 – 58). Conclusion: Kidney failure requiring HD or PE in MM was associated with a significantly shorter life span in comparison with other MM patients. Chronic HD patients had the longest survival among patients who required acute or chronic HD, PE or a combination of the treatments. In general, MM patients in need for HD and/or PE had poor survival.

Theophylline is an old drug of modern medicine. Theophylline toxicity may develop both with acute or chronic intakes due to its narrow therapeutic index. We present a case of theophylline toxicity because of its severe and lethal cardiac and metabolic toxic effects and good response to extracorporeal and medical therapies. A 15-year-old girl with a history of multiple drug intake was admitted to our pediatric intensive care unit due to paracetamol overdose. Theophylline poisoning was suspected because of clinical signs of tachycardia, tachypnea, agitation and metabolic signs of hyperglycemia, hypophosphatemia, hypokalemia,and hypomagnesemia in the early course of hospitalization and blood sample was taken for the measurement of theophylline blood level. The patient was considered as having theophylline toxicity due to high theophylline blood level (112 μg/mL) and normal paracetamol blood level. The patient was intubated because of clinical deterioration, and midazolam for sedation and fentanyl for analgesia were started. Ventricular extrasystoles were detected and after a bolus injection, lidocain infusion was started. Dexmedetomidine and esmolol for tachycardia and levetiracetam for seizure prophylaxis and electrolyte replacement for metabolic disturbances were started as medical treatments. Continuous venovenous hemodialysis was administered for three days and therapeutic plasma exchange was performed for three times with 4% albumin solution (1.5 plasma volume in the first session) and fresh frozen plasma solutions (second and third sessions with one plasma volume). The patient was extubated on the third day and medical therapies were terminated on the fourth day of hospitalization. The patient was discharged on fifth day of hospitalization to the pediatric ward.

A 62-year-old male who was receiving prednisolone and methotrexate for scleroderma and rheumatoid arthritis complained of diarrhea and vomiting, and was transferred to our hospital for detailed examination and treatment of renal dysfunction and thrombocytopenia. Hemolytic anemia and crushed erythrocytes were found during the patient’s course; therefore, we suspected thrombotic microangiopathy (TMA). His ADAMTS13 activity was 60.3% and his ADAMTS13 inhibitor was under 0.5. In addition, his blood culture was positive for Streptococcus pneumoniae, and we finally diagnosed Streptococcus pneumoniae-associated TMA (pTMA). The patient was treated with antibiotics and hemodialysis. The patient recovered and was discharged on the 45th hospital day. Adult pTMA cases are remarkably rare. We herein report a successfully treated adult case of pTMA.

Kawasaki disease (KD) is a form of acute multisystem vasculitis that presents with various complications, including coronary artery aneurysm. Heart failure and brain damage are rare, but life-threatening complications are associated with KD. Here, we describe a 4-year-old girl who developed intravenous immunoglobulin-resistant KD with both left ventricular failure and acute encephalopathy. On day 8 of the illness, the low left ventricular ejection fraction, mitral regurgitation, and low blood pressure, which required continuous administration of dobutamine, were observed during the treatments for KD, including intravenous immunoglobulin. She also appeared unconscious, where the electroencephalogram showed slow waves of activity in all regions of the brain. The cardiac performance improved after she received plasma exchange for three days. However, her unconsciousness with slow waves of activity on electroencephalogram and fever continued after the plasma exchange. Therefore, she was treated with methylprednisolone pulse, followed by prednisolone, as well as intravenous immunoglobulin. Finally, she recovered without any cardiac or neurological sequelae not only at the time she was discharged, but also throughout the follow-up period. The combination therapy using plasma exchange and methylprednisolone pulse may be a treatment option for severe KD with left ventricular failure and acute encephalopathy complications.

Objective : To compare the outcome of plasma exchange versus intravenous immunoglobulin among children with Guillain Barre Syndrome. Methods : A randomized controlled trial was conducted at department of Pediatrics, National Institute of Child Health, Karachi from August 2016 to February 2017. All patients of age ranged from 2-15 years of either gender having duration of Guillain Barre Syndrome not more than 14 days were included. The children were divided into plasma exchange or intravenous immunoglobulin group. Intravenous immunoglobulin was administered for five days in a dose of 0.4 g/kg/day/ while a daily one-volume plasma exchange was given to patients in the plasma exchange group for five days. Duration of mechanical ventilation and the pediatric intensive care unit stay in days were noted. Results Of 78 children, the mean age was 6.64 ±3.06 years. There were 47 (60.3%) males and 31 (39.7%) females. A significantly higher length of intensive care unit stay was noted among children who received plasma exchange (9.45 ±4.59 days) as compared to the children who received intravenous immunoglobin (4.97 ±2.84 days) (p-value

Abstract Transverse myelitis has been reported as a complication of COVID‐19 in recent studies. Here, we report two cases of transverse myelitis related to COVID‐19. Both patients underwent plasma exchange after being treated with antibiotics and corticosteroids which lead to the recovery of one of them.

Key Clinical Message Acquired thrombotic thrombocytopenic purpura is a life‐threatening condition that rarely presents during pregnancy. Early diagnosis and treatment with plasma exchange is needed to achieve a good pregnancy outcome.

Abstract Utilization of therapeutic plasma exchange in select patients with COVID‐19 microangiopathy may provide useful treatment by modulation of inflammatory cytokines and coagulation cascade to maintain homeostasis.

Introduction: Myasthenia gravis is an autoimmune disease of the postsynaptic part of the neuromuscular junction. The disease is chronic, manifesting itself in the fatigue of various muscle groups, and the first manifestation of the disease often affects the muscles that move the eyeball and eyelid, although MG can lead to weakness of any muscle group. In turn, myasthenic crisis is a severe worsening of muscle weakness that often results in the urgent need of mechanical ventilation of the patient, due to the respiratory failure. Case reports: The first patient, 64 years old, female was admitted to the Neurology Department due to breathing difficulties and weakness of the limbs, which suddenly worsened within a dozen of hours before admission. During the stay, steroid therapy was used, as well as six times plasma exchange took place at the Nephrology Department with good treatment tolerance. The dosage of pyridostigmine was also modified, with no adverse effects. During the stay, the patient's condition improved - respiratory disorders resolved, ptosis was significantly withdrawn, and limb muscle tension increased globally. The second patient, aged 67, also female, with a history of myasthenia gravis as well, was admitted to the Neurology Department due to increased fatigue, ptosis, dysphagia, and difficulties in speaking and breathing. Steroid therapy was used, but due to high blood glucose, the steroid dose was reduced and a 2-day cladribine treatment was initiated, with good tolerance. Discussion: Myasthenic crisis is often a life-threatening condition that requires immediate treatment. Steroid therapy, pyridostigmine, IVIG and plasma exchange are standard approaches in myasthenic crisis. Cladribine is not an ordinary approach, but can be beneficial in some patients.

Background/Aims Severe fever with thrombocytopenia syndrome (SFTS) is a viral hemorrhagic fever with a high fatality rate. However, effective treatments for SFTS cases not responded to supportive therapy have not been established. Herein, we introduced the therapeutic plasma exchange (TPE) in SFTS patients in a tertiary hospital between 2013 and 2015. Methods TPE was performed in patients with rapidly progressing SFTS. Clinical, laboratory, and virological parameters were compared before and after TPE. Results Among 27 confirmed SFTS patients, two patients were treated with TPE and ribavirin combination in May 2013, then, 14 patients with rapidly progressing SFTS patients were treated with only TPE from June 2013 to September 2015: their median age was 58 years (interquartile range, 50 to 70) and eight (57.1%) were male. Body temperature, pressure-adjusted heart rate, white blood cell and platelet counts, coagulation profile, serum creatinine, and multiple organ dysfunction score improved immediately after TPE. In addition, the mean cyclic threshold value of real-time reverse transcriptase polymerase chain reaction for SFTS virus after TPE (mean ± standard deviation, 31.3 ± 2.9) was significantly higher than that before TPE (26.5 ± 2.9; p < 0.001), indicating that serum viral loads decreased after TPE. Finally, 13 of 14 TPE-treated patients (92.8%) recovered from rapidly progressing SFTS without sequelae. Conclusions SFTS patients treated with TPE showed improvements in clinical, laboratory, and virological parameters. These results suggest that TPE would be a therapeutic modality as rescue therapy in patients with rapidly progressing SFTS.

Abstract Background Therapeutic plasma exchange (PE) or plasmapheresis is an extracorporeal procedure employed to treat immunological disorders. Exosomes, nanosized vesicles of endosomal origin, mediate intercellular communication by transferring cargo proteins and nucleic acids and regulate many pathophysiological processes. Exosomal miRNAs are potential biomarkers due to their stability and dysregulation in diseases including complex regional pain syndrome (CRPS), a chronic pain disorder with persistent inflammation. A previous study showed that a subset of CRPS patients responded to PE. Methods As a proof-of-concept, we investigated the PE-induced exosomal miRNA changes in six CRPS patients. Plasma cytokine levels were measured by HPLC and correlated with miRNA expression. Luciferase assay following co-transfection of HEK293 cells with target 3′UTR constructs and miRNA mimics was used to evaluate miRNA mediated gene regulation of target mRNA. Transient transfection of THP-1 cells with miRNA mimics followed by estimation of target gene and protein expression was used to validate the findings. Results Comparison of miRNAs in exosomes from the serum of three responders and three poor-responders showed that 17 miRNAs differed significantly before and after therapy. Of these, poor responders had lower exosomal hsa-miR-338-5p. We show that miR-338-5p can bind to the interleukin 6 (IL-6) 3′ untranslated region and can regulate IL-6 mRNA and protein levels in vitro. PE resulted in a significant reduction of IL-6 in CRPS patients. Conclusions We propose that lower pretreatment levels of miR-338-5p in poor responders are linked to IL-6 levels and inflammation in CRPS. Our data suggests the feasibility of exploring exosomal miRNAs as a strategy in patient stratification for maximizing therapeutic outcome of PE.

Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy. Progressive limb weakness, diminished/absent reflexes, sensory disturbance, and variable autonomic dysfunction are its core clinical manifestations. Bifacial weakness with paraesthesias (BFP) is a rare regional variant of GBS and is characterized by simultaneous facial diplegia, distal paraesthesias and minimal or no motor weakness. The association of headache with classic GBS has been rarely reported in the literature, and has not yet been described in the BFP variant. Here we report a misleading case of BFP variant associated with severe headache and mild pleocytosis. The repetition of nerve conduction studies (NCS) was extremely beneficial in this confusing case.

Background: Thrombotic thrombocytopenic purpura (TTP) is an uncommon haematological disease which can occur at any age and may present with COVID-19. This case describes a COVID-19 complication associated with a presentation resembling TTP. Case description: A 51-year-old man who had received a kidney transplant and was on immunosuppressant medication, was admitted to a critical care unit with severe COVID-19 pneumonia/acute respiratory distress syndrome (ARDS) which required intubation, mechanical ventilation and inotropic support. The course was complicated by the classic pentad of thrombocytopenia, intravascular haemolysis, acute kidney injury, neurological symptoms and fever, which prompted the diagnosis of probable TTP. After five sessions of therapeutic plasma exchange, the patient’s general status improved, he was weaned off mechanical ventilation and his renal panel and haemolytic markers normalized. Conclusion: TTP is a life-threatening condition which requires urgent management with therapeutic plasma exchange. This case highlights some possible complications of COVID-19 generally and in immunocompromised patients specifically. The potential role of plasma exchange in COVID-19 patients without a positive diagnosis of TTP (the so-called ‘TTP resembling presentation’) is an area of further research.

Background: Thrombotic thrombocytopenic purpura is a relatively rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end-organ damage. The early recognition is critical because it is an emergency condition requiring urgent therapeutic plasma exchange, which is a life-saving procedure and the cornerstone of the management. Methods: A retrospective descriptive study was conducted at the Department of Medicine-Hematology at King Hussein Medical Center, Amman, Jordan, between June 2018 and July 2019. All patients from all departments who were diagnosed with Thrombotic thrombocytopenic purpura during the study period were enrolled. We obtained the information from all patients concerning demographic data, signs, and symptoms of presentation, and the results of investigations at admission and discharge. The number of plasma exchange sessions, days of hospitalization, usage of Rituximab, and outcome. Results: 21 patients were enrolled, predominantly female (67%), the median age was 36.67 years. At diagnosis, 81% of patients showed neurological involvement. The median platelet count at presentation was 20.000/μL. All patients were anemic (mean HCT,25.42). Schistocytes were present in the peripheral blood film of all patients. The median number of plasma exchange sessions was 19 (range, 2–42), and the mean stay in hospital was 25 ± 11.6 days. Rituximab was administered to 11 patients (52%). A complete response was achieved in 62% (13/21) of patients, The mortality rate was 38% (8/21 patients). None of the patients’ characteristics or laboratory results at presentation were significantly associated with mortality. Conclusions: Although our study included a limited number of TTP patients, our findings revealed that our patients had poor prognostic factors and were less responsive to PEX than TTP patients in other countries. Our data also confirm a better survival rate in younger patients than in the elderly.

Background: Thrombotic thrombocytopenic purpura is a potentially life-threatening condition. Although the introduction of therapeutic plasma exchange has reduced mortality rates from over 90% to 10%-20%, approximately 40% of patients relapse, and outcomes may be fatal in refractory patients. There is clearly a need for additional therapeutic approaches. Aims: To describe the outcomes of relapsed/refractory thrombotic thrombocytopenic purpura patients treated with vincristine as an adjunct to therapeutic plasma exchange. Study Design: Cross-sectional study. Methods: The medical records of all relapsed/refractory patients with thrombotic thrombocytopenic purpura treated with vincristine adjunct to therapeutic plasma exchange between October 2000 and December 2016 were retrospectively reviewed. Diagnosis of thrombotic thrombocytopenic purpura was based on clinical history, physical examination, and laboratory examinations. Patient demographics, laboratory findings, initial date and duration of therapeutic plasma exchange, dosage and time of administration of vincristine, and outcomes were recorded. Results: The study included 15 patients [median age: 37 years (range: 26-65); 7 women and 8 men] with either relapsed or refractory thrombotic thrombocytopenic purpura who were treated with vincristine as an adjunct to therapeutic plasma exchange for a total of 22 episodes. Eighty-seven percent of patients achieved remissions in 20 of 22 episodes, with a median duration of remission of 29.5 months (range: 3-105). After a median follow-up of 55 months, 11 patients were alive. Vincristine was well tolerated with no safety concerns. Conclusion: Vincristine offers a reasonable option for the treatment of patients with relapsed/refractory thrombotic thrombocytopenic purpura. Further studies evaluating vincristine in the front-line setting and in the relapsed/refractory setting are needed to validate the role of vincristine in thrombotic thrombocytopenic purpura patients.

Introduction: The aim of this study was to identify demographic and clinical characteristics of patients who were subjected to plasma exchange (PE) at our unit, and to investigate the effect of these factors on treatment outcome and patient prognosis. Methods: Demographic, clinical and laboratory data of patients who were subjected to PE between January 2012 and August 2015 were obtained from the hospital information system, medical records and the records of apheresis unit. Results: Plasma exchange was performed in 40 patients for 168 times throughout the study. The median age of the patients was 9.4 (range: 1.5-17.3) years, with a male/female ratio of 1.35. Of the patients, 47.5% had an underlying disease. The most common comorbidity was malignancy. The most common indication for PE was sepsis-related multiple organ failure (n=19, 47.5%). The mortality rate was higher in patients with an underlying chronic disease, compared to those without (25% and 7.5%, respectively). No life-threatening complication associated with the apheresis procedure was observed. Conclusion: Our results suggest that PE can be safely performed in children. It seems that indication for PE and the presence of underlying diseases are affecting the mortality rate.

Introduction: The aim of this study was to evaluate treatment approaches towards Guillain-Barré syndrome and treatment response in patients with Guillain-Barré syndrome admitted to the pediatric intensive care unit. Methods: We retrospectively evaluated patients aged between 1 month and 18 years who were admitted to our pediatric intensive care unit between January 2006 and January 2016 with the diagnosis of Guillain-Barré syndrome. Results: A total of 27 patients (10 girls; 37%) were included in this study. The mean age of the patients was 6.24 years (IQR: 4.07-10.03). Nine patients (33.3%) had a Hughes functional grading scale score of 3, 16 (59.3%) had 3 and 2 (7.4%) had 5. Electrophysiological studies were performed in 22 cases (81.4%) and acute inflammatory demyelinating polyneuropathy was detected in 10 cases (45.5%), acute motor axonal neuropathy in 9 cases (40.9%), and acute motor and sensory axonal neuropathy in 1 case (4.5%). Two (9.1%) patients were evaluated as normal. All the patients were administered intravenous immunoglobulin (IVIG). In 12 (52.17%) patients, plasmapheresis was performed for a median of 8 sessions (5-9) before IVIG. Mechanical ventilation was required in 6 patients (22.2%) and was performed for a median of 24 days (5-41). The mean period of time of the first supported sitting was 6 (3-10) days and the mean period of time of the first walking with aid was 9 (7-15) days. Conclusion: Although there have been studies claiming that plasmapheresis was more successful, it has been generally accepted that plasmapheresis and IVIG have the same efficacy. Under the conditions of our country, we believe that both plasmapheresis and IVIG can be safely used at experienced units in patients with Guillain-Barré syndrome who need intensive care treatment.

The article reveals medical and social relevance of extreme familial hypercholesterolemia. It presents the main principles, innovative technologies and an algorithm of diagnosis, diagnostic verification, risk stratification of patients and optimization of the use of aggressive combined cholesterol-lowering drug treatment and precision of indications for plasma exchange and the lack of options.

Intrahepatic cholestasis, a rare complication of sickle cell anemia, is characterized by marked hyperbilirubinemia, acute hepatic failure, and an often fatal course. In this report, we present patients with homozygous hemoglobin S and one patient with sickle-beta thalassemia disease who have intrahepatic cholestasis. Despite automated red blood cell exchange transfusion, which successfully decreased the hemoglobin S level to less than 30% of total hemoglobin level in the peripheral blood, basic biochemical laboratory examination revealed signs of cholestasis with a serum bilirubin level of up to 50 mg/dL in the first case, 30 mg/dL in the second, and 10 mg/dL in the third. The patients underwent plasma exchange, which improved their clinical condition except one. These cases suggest that plasma exchange may have a role in improving the clinical condition of patients with sickle cell disease complicated with intrahepatic cholestasis (SCIC) that fails to respond to automated red blood cell exchange therapy [Cukurova Med J 2016; 41(4.000): 799-803]

Thrombotic thrombocytopenic purpura (TTP) is a rare disease associated with microangiopathic hemolytic anemia, thrombocytopenia, fever, neurological disorders, and renal insufficiency pentad. It is a fatal hematologic emergency if left untreated. If plasma exchange is feasible, treatment is easy and comfortable. It should be kept in mind that such as in our patient may be acquired at all congenital illnesses.

Q fever is a zoonosis produced by Coxiella burnetii, with potential neurological complications such as aseptic meningitis, myelitis, encephalitis and peripheral neuropathy. We report the case of a 68-year-old male with recent history of acute Q fever, who developed progressive numbness of the hands, lower limbs and abdomen. Clinical exam revealed bilateral crural loss of proprioception and vibration senses, positive Romberg, bilateral Babinski and intact DTRs. Initial electroneurography was normal, however, when repeated, revealed axonal sensory polyneuropathy. Brain and cord MRI, including assessment of lumbo-sacral plexus, was also normal. All laboratory findings were within normal range, except for slightly elevated glycemia. Plasma exchange was initially tried producing limited improvement. Repeated C. burnetii serology and CSF analysis revealed elevated levels of IgM and IgG, still suggestive of acute infection. For this reason, the patient continued treatment with Doxycycline and upon discharge from hospital, finally reported improvement of symptoms, proving retrospectively that the neuropathy was secondary to C. burnetii infection.