Therapeutic plasma exchange as a steroid‐sparing therapy in a patient with limbic encephalitis due to antibodies to voltage‐gated potassium channels

Autoantibodies to the voltage‐gated potassium channel (VGKC) complex cause a spectrum of non‐paraneoplastic neurologic syndromes including limbic encephalitis (LE). We report a case of a man with LE who underwent a course of therapeutic plasma exchange (TPE) in addition to other immunomodulatory therapies and experienced sustained clinical resolution of his symptoms. This report adds to the existing literature supporting TPE in cases of LE due to VGKC complex autoantibodies. J. Clin. Apheresis 31:63–65, 2016. © 2015 Wiley Periodicals, Inc.     

Intravenous methylprednisolone versus therapeutic plasma exchange for treatment of anti‐n‐methyl‐d‐aspartate receptor antibody encephalitis: A retrospective review

Introduction : Anti‐N‐methyl‐d ‐aspartate (NMDA) receptor antibody encephalitis is an increasingly recognized form of autoimmune encephalitis. Conventional treatments include therapies such as corticosteroids, intravenous immunoglobulin (IVIg), and/or therapeutic plasma exchange (TPE). Although TPE is regularly used for treatment of anti‐NMDA receptor antibody encephalitis, the American Society for Apheresis has given it a category III recommendation only. Earlier administered immunotherapies in tumor‐negative patients may facilitate faster recoveries, but it remains unclear whether or not TPE is superior to steroids and/or IVIG. Methods : We retrospectively evaluated 10 of 14 patients that received steroids and TPE with modified Rankin scores and subjectively assessed the point of largest sustained improvement in all 14 patients. Results : In the patients that received both steroids and TPE at our institution during the same hospitalization (only 10 of 14 patients), 7/10 patients after TPE had improved with the modified Rankin score versus 3/10 patients after steroids. The average modified Rankin score improvement after steroids in this group was ?0.1 as compared with 0.4 after TPE. Based on subjective chart review analysis during which all 14 patients were assessed, the largest sustained improvement occurred immediately following the third–fifth exchange in 9/14 patients, whereas only 2/14 patients appeared to have had significant benefit immediately following steroids. Conclusions : This is compelling preliminary data that suggests that corticosteroids may not be as effective compared to steroids followed by TPE. Given the importance of time‐sensitive treatment, more formal studies may illuminate the ideal first‐line treatment for anti‐NMDA receptor antibody encephalitis. J. Clin. Apheresis 30:212–216, 2015. © 2015 Wiley Periodicals, Inc.     

Therapeutic plasma exchange in patients with neurologic diseases: retrospective multicenter study.

Therapeutic plasma exchange (TPE) is commonly used in many neurological disorders where an immune etiology was known or suspected. We report our experience with TPE performed for neuroimmunologic disorders at four university hospitals. The study was a retrospective review of the medical records of neurological patients (n=57) consecutively treated with TPE between April 2006 and May 2007. TPE indications in neurological diseases included Guillain-Barrè Syndrome (GBS) (n=41), myasthenia gravis (MG) (n=11), acute disseminated encephalomyelitis (ADEM) (n=3), chronic inflammatory demyelinating polyneuropathy (CIDP) (n=1) and multiple sclerosis (MS) (n=1). Patient median age was 49; there was a predominance of males. Twenty-two patients had a history of other therapy including intravenous immunoglobulin (IVIG), steroid, azothioprin, and pridostigmine prior to TPE. Another 35 patients had not received any treatment prior to TPE. All patients were classified according to the Hughes functional grading scores pre- and first day post-TPE for early clinical evaluation of patients. The TPE was carried out 1-1.5 times at the predicted plasma volume every other day. Two hundred and ninety-four procedures were performed on 57 patients. The median number of TPE sessions per patient was five, and the median processed plasma volume was 3075mL for each cycle. Although the pre-TPE median Hughes score of all patients was 4, it had decreased to grade 1 after TPE. While the pre-TPE median Hughes score for GBS and MG patients was 4, post-TPE scores were decreased to grade 1. Additionally, there was a statistically significant difference between post-TPE Hughes score for GBS patients with TPE as front line therapy and patients receiving IVIG as front line therapy (1 vs. 3.5; p=0.034). Although there was no post-TPE improvement in Hughes scores in patients with ADEM and CIDP, patients with MS had an improved Hughes score from 4 to 1. Mild and manageable complications such as hypotension and hypocalcemia were also observed. TPE may be preferable for controlling symptoms of neuroimmunological disorders in early stage of the disease, especially with GBS.     

Therapeutic plasma exchange for the treatment of anti-NMDA receptor encephalitis

Anti-N-methyl-D-aspartate receptor (NMDA-R) encephalitis is thought to be one of the common paraneoplastic-associated encephalitides. Between February 2001 and February 2011, nine patients were diagnosed with this disorder at Columbia University Medical Center: eight females (mean age 23 years) and one male (3 years of age). Four female patients had ovarian teratomas, which were removed as part of their treatment. Therapeutic plasma exchange (TPE) was used as one of the treatment modalities in addition to immunosuppressive therapy, including corticosteroids, intravenous immunoglobulin (IVIG), and/or rituximab. A total of 56 TPE procedures were performed in these patients on alternate days (range, 5-14 procedures/patient). Approximately 1 plasma volume (PV) was processed for all patients; 5% albumin and 0.9% normal saline were used as replacement fluid. Complications occurred in 20% of TPE procedures; 9% were possibly due to underlying disease. The remaining 11% of complications were hypotensive episodes that rapidly responded to either a fluid bolus or a vasopressor treatment. One patient demonstrated immediate clinical improvement after three TPE treatments, and four patients had significant improvement at time of discharge from the hospital. Long-term follow-up showed that early initiation of TPE appears to be beneficial, and patients who received IVIG after TPE did better than those who received IVIG before TPE. However, the number of patients in this series is too small to provide statistically significant conclusions. Overall, TPE is a relatively safe treatment option in patients with anti-NMDA-R encephalitis. Further studies are needed to elucidate the benefit of TPE in this disease.     

Anti‐MDA‐5 antibody‐positive clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease treated with therapeutic plasma exchange: A case series

We present six cases of antimelanoma differentiation‐associated gene 5 antibody (anti‐MDA5‐Ab)‐positive clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP‐ILD), which is known to have a poor prognosis. The outcomes of these cases are described after treatment with therapeutic plasma exchange (TPE). Clinical and therapeutic data for patients with CADM with RP‐ILD were collected retrospectively from medical records. All six patients received early intensive care including high‐dose corticosteroids, intravenous cyclophosphamide, and a calcineurin inhibitor, but lung disease and hypoxia became more severe. TPE was performed over a median of 9.5 sessions (range 3‐14) per patient, and the median duration from admission to TPE was 23 days. Three patients received combined direct hemoperfusion using a polymyxin B‐immobilized fiber column (PMX‐DHP) therapy on successive days to manage acute respiratory failure. Four patients survived and two died due to respiratory failure. In the survival cases, ferritin decreased, and ferritin and KL‐6 were lower at diagnosis. The patients who died had a higher alveolar‐arterial oxygen difference and more severe lung lesions at the time of initiation of TPE. These findings indicate that a combination of conventional therapy and TPE may be useful for improvement of the prognosis of CADM with RP‐ILD at the early stage of onset.     

儿童抗MOG抗体合并抗NMDAR抗体双阳性自身免疫性脑炎2例并文献复习

目的 探讨儿童抗髓鞘少突胶质细胞糖蛋白(myelin oligodendrocyte glycoprotein, MOG)抗体合并抗N-甲基-D-门冬氨酸受体(N-methyl-D-aspartate receptor, NMDAR)抗体双阳性自身免疫性脑炎的临床特点。方法 回顾性分析邯郸市中心医院收治的2例抗MOG抗体合并抗NMDAR抗体双阳性自身免疫性脑炎患儿的病例资料,并复习相关文献。结果 2例脑脊液白细胞数目均增多,1例颅脑磁共振成像(magnetic resonance imaging, MRI)同时存在幕上及幕下病变,1例仅有幕上病变且其视频脑电图结果与抗NMDAR脑炎典型脑电图相似。经甲泼尼龙冲击、人免疫球蛋白治疗后,2例症状均得到改善,3个月后随访,均无复发。结论 该病多发于青少年,既有抗MOG抗体相关疾病的脑病表现,也有抗NMDAR脑炎的表现,且以抗NMDAR脑炎的表现更易被识别。颅脑MRI及视频脑电图等无创检查对此病诊断有一定提示意义,但确诊仍需神经抗体检查,诊疗方面一线治疗为激素、免疫球蛋白、血浆置换,多数患儿对此治疗反应良好,但发病机制仍需进一步研究。     

Therapeutic plasma exchange rapidly improves cardiac allograft function in patients with presumed antibody‐mediated rejection

Background: Allograft dysfunction due to presumed antibody‐mediated rejection (pAMR) is one of the most serious complications of heart transplantation. Combination therapies of high‐dose steroids, intravenous immune globulin, and/or therapeutic plasma exchange (TPE) are often used in this setting. Methods: We performed a 9‐year retrospective review of all episodes of pAMR treated with TPE at our institution. pAMR diagnosis was based on clinical and pathologic findings. Left ventricular ejection fraction (LVEF) was measured at baseline, prior to initiation of TPE, and during the course of treatment. Results: There were 42 patients with 47 episodes of pAMR treated with TPE. The majority of episodes were treated with three TPE; however, eight required only two TPE and five episodes required >3 TPE. All episodes of pAMR had LVEF measured before and after the series of TPEs. The mean pre‐TPE LVEF was 38% compared with a post‐therapy mean LVEF of 50% (P < 0.0001). In 16 episodes of pAMR, for which LVEF was measured following each apheresis, there was significant improvement of allograft function after the first TPE (pre‐TPE mean LVEF of 31% and post‐first TPE mean LVEF of 37%; P = 0.02). Incremental and significant improvement in allograft function continued following each TPE. Changes in human leukocyte antigen‐donor specific antibodies and fibrinogen did not correlate with ejection fraction response. Conclusions: The rapid improvement in allograft function in our patients is most likely due to TPE as other pharmacologic interventions have longer onset. TPE should be considered a first‐line intervention in the setting of pAMR. J. Clin. Apheresis 29:316–321 2014. © 2014 Wiley Periodicals, Inc.     

Clinical improvement of a patient with severe Wilson's disease after a single session of therapeutic plasma exchange

Here, we report a case of a 17‐year old female with Wilson's disease presenting with progressive Coombs' negative hemolytic anemia and hepatic cirrhosis who was treated with one session of therapeutic plasma exchange (TPE) and clinically improved. In clinical situations where multiple sessions of TPE may not be possible, the use of a single session of TPE in conjunction with conventional therapy may be of benefit in preventing further clinical deterioration. J. Clin. Apheresis, 2009. © 2009 Wiley‐Liss, Inc.     

Thrombotic thrombocytopenic purpura in a patient with rheumatoid arthritis treated by plasmapheresis.

Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder characterized by consumptive thrombocytopenia, microangiopathic hemolytic anemia, and neurologic symptoms. TTP is associated with many diseases and several therapeutic drugs. We report the rare case of a patient with rheumatoid arthritis who developed TTP that was not associated with drug therapy, 18 months after the onset of rheumatoid arthritis. She recovered from the TTP following daily sessions of therapeutic plasma exchange (TPE) with fresh frozen plasma replacement and glucocorticoid therapy. Recent pathogenic mechanisms are reviewed as they relate to von Willebrand factor. In this report of the rare association of TTP with rheumatoid arthritis, an immediate relationship is likely because both are of an immune nature. Awareness of the possible development of TTP in rheumatoid arthritis is important for early diagnosis and treatment.     

Therapeutic plasma exchange and immunosuppressive therapy in a patient with anti‐GAD antibody‐related epilepsy: Quantification of the antibody response

Antibodies to glutamic acid decarboxylase (GAD) have been associated with a host of neurological disorders including stiff person syndrome, cerebellar ataxia, limbic encephalitis, and epilepsy. Whether anti‐GAD antibodies have an etiological role in these neurological disorders or simply serve as disease markers is unclear. Here, we report a case of a patient with recurrent seizures, poorly responsive to conventional treatment, associated with anti‐GAD antibodies. The patient was experiencing near daily seizures at the time of presentation and had marked improvement while receiving immunosuppressive therapy and therapeutic plasma exchange (TPE). We go on to show that the patient had a substantial reduction of her GAD autoantibody burden following this therapy. Using immunostaining, we further demonstrate a progressive loss of GAD reactivity in the patient's sera to neurons and GAD‐expressing HELA cells with successive TPEs. Hence, these data support the concept of an immune‐mediated pathogenic component to these autoantibody‐associated neurological syndromes. J. Clin. Apheresis 30:8–14, 2015. © 2014 Wiley Periodicals, Inc.     

The effect of therapeutic plasma exchange on management of HELLP Syndrome: The report of 47 patients

Background and objectivesHELLP syndrome is a life-threatening condition that may potentially cause complications during pregnancy. If not diagnosed and treated quickly, HELLP syndrome may lead to serious complications both for the mother and the baby. The aim of this study was to determin the effectiveness of therapeutic plasma exchange (TPE) for treatment of Class-I HELLP syndrome.Materials and MethodsLaboratory results from 47 patients with Class-I HELLP syndrome patients who underwent TPE between 2011 and 2020 were recorded before and after the procedure. A central venous catheter was inserted, and TPE was performed in patients who had not responded to delivery, steroid, and supportive therapy (blood products, anti-hypertensive therapy, intravenous fluid administration, and antibiotics) within 24 hours after the diagnosis of Class I HELLP syndrome according to the Mississippi Criteria.ResultsThe average age of patients was 33 ± 4.7 years (range; 21-39 years). A mean of 5 (range; 4 to 6) TPE sessions were performed. There was a statistically significant decrease in total bilirubin, lactic dehydrogenase, aspartate aminotransferase, and alanine aminotransferase levels in all patients, whereas a significant increase in platelet count was observed (p < 0.05). Furthermore, clinical and laboratory improvement was achieved.ConclusionIn all patients with HELLP syndrome, a dramatically clinical and laboratory improvement occurred after TPE. Our study suggests that postpartum use of TPE within 24 hours is an efficient treatment option for Class-I HELLP syndrome.     

Therapeutic plasma exchange for control of thyroid storm

Therapeutic plasma exchange (TPE) for thyroid storm has recently been upgraded to a category II indication after decades though its recommendation level still remains at Grade 2C according to the American Society for Apheresis (ASFA). In the absence of prospective randomized controlled trials due to the rarity of thyroid storm, retrospective data from case series continue to elevate the clinical evidence supporting TPE as a life‐saving modality for complicated thyroid storm patients. We report three cases of life‐threatening thyroid storm from Graves' disease rescued by TPE via rapid reduction in circulating thyroid hormones. Each patient underwent TPE when it was judged that other thyroid storm treatment options were futile or unsafe. The first patient received 4 cycles of TPE while the second patient received 9 cycles of TPE, and the third patient received 2 cycles of TPE with satisfactory clinical improvement. Plasma FT4 and TSH receptor antibody levels of the first case declined by 41.3% and >50% respectively right after the first round of TPE; plasma FT4 of the second patient dropped by up to 31.6% during the course of TPE; plasma FT4 and TSH receptor antibody of the third patient declined by 66% and 56.2% respectively after the first cycle of TPE. This demonstrates the safety, efficacy, and feasibility of TPE in thyroid storm especially when other therapeutic interventions are contraindicated. TPE operates via the elimination of serum proteins‐bound thyroid hormones, thyroid autoantibodies, cytokines, and catecholamines in addition to increasing unsaturated binding sites for thyroid hormones.     

Efficacy of therapeutic plasma exchange in a patient with coagulation inhibitors (acquired haemophilia A) – A case report

Acquired haemophilia A (AHA) is a rare disorder with mostly idiopathic aetiology that leads to factor VIII (FVIII) deficiency due to coagulation inhibitors formation. Treatment protocol includes immunosuppression and Factor VIII bypassing agents including activated Prothrombin Complex Concentrates (PCC). Nevertheless, the role of plasma exchange is not clear in the treatment of AHA. We report a case of 73 year old male who presented with haematuria, prolonged activated partial thromboplastin time (APTT) and a very high titres of Factor VIII inhibitors of 98 Bethesda units (BU) and was diagnosed with acquired haemophilia A. He failed to respond to multiple immunosuppressive therapies including rituximab. Therefore, therapeutic plasma exchange (TPE) therapy was planned due to persistence of haematuria despite immunosuppressive therapies. After five cycles of plasma exchange, APTT became normal, haematuria subsided and Factor VIII inhibitors became negative. Patient was discharged without any bleeding and in a stable condition. In this index case, plasma exchange played a very crucial role, resulting in recovery of the patient. These results advocate that therapeutic plasma exchange is an effective therapy for acquired haemophilia A.     

Electroconvulsive therapy and/or plasmapheresis in autoimmune encephalitis?

Autoimmune encephalitis is a poorly understood condition that can present with a combination of neurological and psychiatric symptoms, either of which may predominate. There are many autoantibodies associated with a variety of clinical syndromes - anti-N-Methyl-D-Aspartate receptor (NMDAR) is the commonest. Currently, the most widely used therapy is prompt plasmapheresis and steroid treatment (and tumour resection if indicated), followed by second line immunosuppression if this fails. Given the growing awareness of autoimmune encephalitis as an entity, it is increasingly important that we consider it as a potential diagnosis in order to provide timely, effective treatment. We discuss several previously published case reports and one new case. These reports examined the effects of electroconvulsive therapy (ECT) on patients with autoimmune encephalitis, particularly those in whom psychiatric symptoms are especially debilitating and refractory to standard treatment. We also discuss factors predicting good outcome and possible mechanisms by which ECT may be effective. Numerous cases, such as those presented by Wingfield, Tsutsui, Florance, Sansing, Braakman and Matsumoto, demonstrate effective use of ECT in anti-NMDAR encephalitis patients with severe psychiatric symptoms such as catatonia, psychosis, narcolepsy and stupor who had failed to respond to standard treatments alone. We also present a new case of a 71-year-old female who presented to a psychiatric unit initially with depression, which escalated to catatonia, delusions, nihilism and auditory hallucinations. After anti-NMDAR antibodies were isolated, she was treated by the neurology team with plasmapheresis and steroids, with a partial response. She received multiple sessions of ECT and her psychiatric symptoms completely resolved and she returned to her premorbid state. For this reason, we suggest that ECT should be considered, particularly in those patients who are non-responders to standard therapies.     

A case of acute optic neuritis during pregnancy treated by membrane-based therapeutic plasma exchanges without systemic anticoagulation

IntroductionIn acute optic neuritis, high dose steroid therapy as first - line treatment is contraindicated in early pregnancy, therapeutic plasma exchanges (TPE) represent an alternative. We report a case of a pregnant woman with progressive, acute optic neuritis subjected to membrane-based therapeutic plasma exchange with extracorporal citrate-based anticoagulation.Case presentationA 35 year-old second-time pregnant woman (4th week of gravidity) of Caucasian ethnicity complained of visual impairment of the right eye. She was hospitalized for suspected optic neuritis. In the eye exam central and peripheral scotoma of the right side were found. T2 weighted Magnetic-Resonance Imaging revealed an isolated, prechiasmal lesion of the right optic nerve, and the patient had a delayed p100 latency of visually evoked potentials of the right eye. Cerebrospinal-fluid investigation was unrevealing. The diagnosis of right sided optic neuritis was established. Due to early pregnancy, steroids were contraindicated. Visual disturbances further deteriorated by day 2 in hospital. For therapy, 5 sessions of membrane-based therapeutic plasma exchange with albumin solution were performed. An extracorporal anticoagulation using citrate with calcium substitution was applied. After the second session, there was a subjective improvement of symptoms. At discharge on day 14, visual acuity was no longer impaired, sensitivity to bright light remained. In eye exam at 3.5 months after discharge, the patient ha d a complete recovery. Follow-up gynecological exams were unrevealing.ConclusionThis case of unilateral acute optic neuritis supports the view that membrane-based therpautic plasma exchange without systemic anticoagulation represents a safe intervention in pregnancy.     

Therapeutic plasma exchange in patients with neurological diseases: Multicenter retrospective analysis

Therapeutic plasma exchange (TPE), is a procedure, changing pathologic substances in the plasma of patients with replacement fluid. TPE has an increasing list of indications in recent years such as neurological, connective tissue, hematological, nephrological, endocrinological and metabolic disorders. We report our multicenter data about therapeutic plasma exchange in patients with neurological diseases. Six University Hospitals’ aphaeresis units medical records about neurologic diseases were reviewed retrospectively. Hundred and fifteen patients and 771 TPE sessions from six aphaeresis units’ were included to this study. Of the 115 patients, 53 (46%) were men and 62 (54%) were women. The median age was 50 (range: 5–85) years. Of these patients 58.3% were Guillain–Barre syndrome (GBS), 17.4% were acute disseminated encephalomyelitis (ADEM), 10.4% were chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), 7% were multiple sclerosis, 6.1% were myasthenia gravis (MG) and 0.9% were Wilson disease (WD). The median number of TPE sessions per patient was 5 (range 1–72). Human albumin was used as a replacement fluid in 66% and fresh frozen plasma was used in 34% of cases. TPE was done through central venous catheters in 66%, and peripheral venous access in 34% of patients. Some complications were seen in patients (18.3%) during TPE sessions. These complications were, complications related to catheter placement procedure (8.7%), hypotension (3.5%), hypocalcaemia (3.5%) and allergic reactions (1.7%). The complication ratios were 2.7% in total 771 TPE procedures. TPE procedure was terminated in 6% of sessions depending on these complications. Overall responses to TPE were noted in 89.5% of patients.In conclusion; Therapeutic plasma exchange is an effective treatment option in several neurologic diseases.     

Hyperchloremic metabolic acidosis after plasma exchange in a patient with renal transplant rejection: A case report

Therapeutic plasma exchange (TPE) is an effective treatment for several renal disorders, including renal transplant rejection. However, repeated plasma exchanges can result in various metabolic disturbances and complications. We present a 61-year old male with a medical history of type 2 diabetes, hypertension, successfully treated multiple myeloma, and a post-mortem kidney transplantation 7 months prior to presentation. The patient was hospitalized with an antibody-mediated transplant rejection for which treatment with methylprednisolone, TPE with a 40 g/L albumin solution as a replacement fluid, and intravenous immunoglobulins was initiated. After four TPE treatments, the patient developed gastrointestinal complaints and muscle weakness. Despite daily oral bicarbonate supplementation, laboratory tests revealed a hyperchloremic metabolic acidosis: bicarbonate 11.7 mmol/L, chloride 111 mmol/L, and sodium 138 mmol/L. Metabolic acidosis due to citrate accumulation was ruled out with a normal total-to-ionized calcium ratio. After treatment with intravenous bicarbonate supplementation, the symptoms disappeared. Analysis of the albumin solution showed a chloride concentration of 132 mmol/L. This is the first case that describes severe metabolic acidosis after multiple sessions of TPE with an albumin solution in a patient with impaired renal function. The hyperchloremic metabolic acidosis is the result of administration of large volumes of an albumin solution with high chloride concentrations. Special attention should be paid to the acid–base balance during TPE in patients with impaired renal function. Future research should investigate the incidence of hyperchloremic metabolic acidosis during TPE in patients with impaired renal function.     

Emergency ABO‐incompatible liver transplant secondary to fulminant hepatic failure: Outcome,role of TPE and review of the literature

The increasing demand for solid organ transplants has brought to light the need to utilize organs in critical situations despite ABO‐incompatibility. However, these transplantations are complicated by pre‐existing ABO antibodies which may be potentially dangerous and makes the transplantation prone to failure due to rejection with resulting necrosis or intrahepatic biliary complications. We report the clinical outcome of an emergency ABO‐incompatible liver transplant (due to fulminant hepatic failure with sudden and rapidly deteriorating mental status) using a modified therapeutic plasma exchange (TPE) protocol. The recipient was O‐positive with an initial anti‐B titer of 64 and the cadaveric organ was from a B‐positive donor. The patient underwent initial TPE during the peri‐operative period, followed by a series of postoperative daily TPE, and later a third series of TPE for presumptive antibody‐mediated rejection. The latter two were performed in conjunction with the use of IVIg and rituximab. The recipient's anti‐B titer was reduced and maintained at 8 or less 8 months post‐op. However, an elevation of transaminases 3 months post‐transplant triggered a biopsy which was consistent with cellular rejection and with weak C4d positive staining suggestive of antibody mediated rejection. Additional plasma exchange procedures were performed. The patient improved rapidly after modification of her immunosuppression regimen and treatment with plasma exchange. This case illustrates that prompt and aggressive plasma exchange, in conjunction with immunosuppression, is a viable approach to prevent and treat antibody mediated transplant rejection in emergency ABO‐incompatible liver transplant. J. Clin. Apheresis, 2012. © 2012 Wiley Periodicals, Inc.     

Therapeutic approaches in hypertriglyceridemia-induced acute pancreatitis: A literature review of available therapies and case series

Hypertriglyceridemia-induced acute pancreatitis (HGAP) is the third most common etiology of acute pancreatitis. HGAP can be attributed to genetic disturbances in triglyceride metabolism or multiple secondary causes. Here, we presented three cases for HGAP and explored different therapeutic approaches for treating HGAP. A case series of three patients who presented with HGAP and underwent different therapeutic approaches was conducted. The first patient was a 37-year-old male who presented with nonsevere HGAP; he was treated with conservative therapy with insulin and heparin infusion, which resulted in clinical and laboratory improvement. The second patient was a 64-year-old male with human immunodeficiency virus on multiple highly active antiretroviral therapy. He presented with severe HGAP and multiorgan failure. After initiation of therapeutic plasma exchange, his HGAP resolved. The third patient was a 28-year-old male who presented with recurrent episodes of HGAP; his conservative therapy failed and was eventually escalated to therapeutic plasma exchange (TPE). HGAP can be attributed to genetic disturbances of lipid or secondary etiologies. A nonsevere form of HGAP can be managed with conventional therapy including insulin and heparin; however, severe HGAP may require TPE.     

Therapeutic plasma exchange in acute disseminated encephalomyelitis in children

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system that is probably due to an autoimmune mechanism with an acute presentation and a monophasic course. The management of patients with ADEM is based on supportive therapy, corticosteroids, and intravenous immunoglobulin, and in selected cases, with therapeutic plasma exchange (TPE). The aim of our study is to evaluate the efficacy of TPE, as adjuvant therapy in pediatric patients with ADEM. We retrospectively reviewed the medical records of children with the diagnosis of ADEM between 2009 and 2011 to which TPE was indicated and were admitted in the ICU of Hospital Sant Joan de Deu (Spain). The diagnosis of ADEM was made by clinical and laboratory criteria and by the presence of compatible lesions on cranio‐spinal Magnetic Resonance Imaging (MRI). For signaling TPE, we followed the guidelines established by the American Association of Apheresis (ASFA) in 2010. Five cases were identified. The predominant neurological symptoms in our patients were: altered level of consciousness, seizures, motor deficits, cranial nerve disorders, and aphasia. Most important demyelinating lesions were located in cortical and subcortical white matter of the brain and highlighted brainstream. Patients performed between 4 and 5 sessions, with no reported side effects. Progressive clinical improvement was evident in all patients, with good neurosensory response to stimulation, cessation of seizures, and recovery of limb mobility. Nowadays, one patient's right paresis persists and another suffers epileptic seizures. None of the cases in our series presented new episodes of demyelination. Due to the suggested immune‐mediated pathogenesis of ADEM, treatment is based on immunomodulatory agents, being glucocorticoids the most important ones. The treatment can be complemented with intravenous immunoglobulin and plasmapheresis. Available data suggests that plasma exchange is beneficial in children with ADEM who fail these treatments. The good tolerance of the procedure without adverse reactions and the progressive neurological improvement detected in the reviewed cases suggest that the use of TPE in pediatric patients is a good therapeutic option when performed in an experienced center. J. Clin. Apheresis 30:335–339, 2015. © 2015 Wiley Periodicals, Inc.