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https://www.readbyqxmd.com/read/28645643/management-of-thrombotic-thrombocytopenic-purpura
#1
P Coppo
Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of >80%. However, relapses occur in up to 40% of patients and refractory disease with fatal outcomes still occurs, typically within the first days of management. In this context, the introduction of rituximab has been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, and increasingly as frontline therapy, with high response rates in the following weeks...
June 20, 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/28644967/tramadol-may-increases-the-efficacy-of-therapeutic-plasma-exchange-in-anti-nmdar-encephalitis
#2
Bradley A Dengler, Deanna Kitchen, Ali Seifi
No abstract text is available yet for this article.
May 31, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28639937/a-multicentre-randomised-controlled-trial-of-intravenous-immunoglobulin-compared-with-standard-therapy-for-the-treatment-of-transverse-myelitis-in-adults-and-children-strive
#3
Michael Absoud, Peter Brex, Olga Ciccarelli, Onyinye Diribe, Gavin Giovannoni, Jennifer Hellier, Rosemary Howe, Rachel Holland, Joanna Kelly, Paul McCrone, Caroline Murphy, Jackie Palace, Andrew Pickles, Michael Pike, Neil Robertson, Anu Jacob, Ming Lim
BACKGROUND: Transverse myelitis (TM) is an immune-mediated disorder of the spinal cord that affects adults and children and that causes motor, sensory and autonomic dysfunction. There is a prolonged recovery phase, which may continue for many years. Neuromyelitis optica (NMO) is an uncommon relapsing inflammatory central nervous system condition in which TM can be the first presenting symptom. As TM and NMO affect many patients in the prime of their working life, the disorder can impose a significant demand on health resources...
May 2017: Health Technology Assessment: HTA
https://www.readbyqxmd.com/read/28639502/rituximab-as-first-line-treatment-for-acquired-thrombotic-thrombocytopenic-purpura
#4
Haifei Chen, Ailin Fu, Jing Wang, Tianqin Wu, Zhengyang Li, Jieqing Tang, Hongshi Shen, Jingjing Zhu, Jie Li, Qian Zhu, Longmei Qing
Objective To investigate the efficacy and safety of rituximab (RTX) as first-line treatment of acquired thrombotic thrombocytopenic purpura (aTTP). Methods Twenty-five patients with acute aTTP and/or severe a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) deficiency were admitted to our centre from April 2009 to March 2015. Fourteen patients received RTX plus standard therapy (plasma exchange and corticosteroids) at acute episodes. Haemoglobin, platelet count, schistocytes, lactate dehydrogenase levels, ADAMTS13 activity and its inhibitors, and the ratio of B lymphocytes in the peripheral blood, were monitored...
June 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28638625/early-eculizumab-use-in-atypical-haemolytic-uraemic-syndrome-in-a-jehovah-s-witness-refusing-blood-products
#5
May Al-Shaghana, Andrew Bentall, Mark D Jesky, William Lester, Graham Lipkin
Thrombotic microangiopathy (TMA) is characterized by microscopic angiopathic haemolytic anaemia, thrombocytopenia and organ injury. Supportive therapies include the use of blood products. Recently the terminal complement inhibitor eculizumab has been approved in atypical haemolytic uraemic syndrome (aHUS) in some countries. We report the case of a 23-year-old female Jehovah's Witness presenting with vaginal haemorrhage from thrombocytopaenia, severe anaemia (nadir Hb 28 g/L) and anuric acute kidney injury with TMA secondary to aHUS...
June 2017: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/28635356/simultaneous-abo-incompatible-living-donor-liver-transplantation-and-splenectomy-without-plasma-exchange-in-china-two-case-reports
#6
Guoyong Chen, Janjun Sun, Sidong Wei, Yongfeng Chen, Gaofeng Tang, Zhantao Xie, Huaen Xu, Janbin Chen, Huibo Zhao, Zhenhua Yuan, Weiwei Wang, Guangbo Liu, Bing Wang, Biao Niu
ABO-incompatible (ABO-i) living-donor liver transplantation (LDLT) is performed if an ABO-compatible graft cannot be obtained. However, a perfect desensitization protocol has not been established worldwide, especially for simultaneous ABO-i LDLT and splenectomy. We herein report two cases of ABO-i LDLT. To the best of our knowledge, this is the first case report of ABO-i LDLT in an adult patient in China. Splenectomy and T-cell-targeted immunosuppression (basiliximab) was used to overcome the blood group barrier in these recipients...
January 1, 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28626871/a-successful-living-donor-liver-re-transplantation-for-graft-failure-within-seven-days-due-to-acute-de-novo-donor-specific-anti-hla-antibody-mediated-rejection
#7
Yohei Yamada, Ken Hoshino, Teisaburo Mori, Miho Kawaida, Kiyotomo Abe, Hideo Ishihama, Takahiro Shimizu, Nobuhiro Takahashi, Kentaro Matsubara, Taizo Hibi, Yuta Abe, Hiroshi Yagi, Naoki Shimojima, Masahiro Shinoda, Minoru Kitago, Hideaki Obara, Yasushi Fuchimoto, Kaori Kameyama, Yuko Kitagawa, Tatsuo Kuroda
Growing evidence suggests a relationship between antibody-mediated rejection (AMR) and early graft failure due to a previously unknown etiology in liver transplantation (LTx). We herein report a three-year-old boy who developed rapid graft failure due to de novo donor-specific antibody (DSA)-driven AMR a week after living donor LTx, requiring a second transplant on the 10th day after the first LTx. The pathology of the first graft showed massive necrosis in zone 3 along with positive C4d and inflammatory cell infiltrates in portal areas...
June 19, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28619436/tumefactive-demyelinating-lesions-a-comprehensive-review
#8
REVIEW
Hussein Algahtani, Bader Shirah, Ali Alassiri
Tumefactive multiple sclerosis or tumefactive demyelinating lesion (TDL) is one of the rare variants of multiple sclerosis (MS) posing a diagnostic challenge and a therapeutic enigma since it is difficult to distinguish from a true central nervous system (CNS) neoplasm or other CNS lesions on magnetic resonance imaging (MRI). The prevalence of TDL is estimated to be 1-3/1000 cases of MS with an annual incidence of 0.3/100,000. This could be an underestimate due to unavailability of a global MS registry and under-reporting of this condition...
May 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28618127/regional-citrate-anticoagulation-in-membrane-based-plasma-exchange-safety-efficacy-and-effect-on-calcium-balance
#9
Daniel Christiadi, Chari Mercado, Richard Singer
AIM: To assess the efficacy, safety and calcium balance of a membrane based regional citrate anticoagulation plasma exchange protocol METHODS: This was an observational, prospective, single center study of membrane separation plasma exchange using regional citrate anticoagulation. It was performed using a fixed dose pre-filter citrate infusion that was based on the plasma flow rate. Patients received a post filter calcium infusion which was modified during treatment based on systemic ionized calcium monitoring...
June 15, 2017: Nephrology
https://www.readbyqxmd.com/read/28613436/the-incremental-cost-of-incompatible-living-donor-kidney-transplant-a-national-cohort-analysis
#10
David Axelrod, Krista L Lentine, Mark A Schnitzler, Xun Luo, Huiling Xiao, Babak J Orandi, Allan Massie, Jacqueline Garonzik-Wang, Mark D Stegall, Stanley C Jordan, Jose Oberholzer, Ty B Dunn, Lloyd E Ratner, Sandip Kapur, Ronald P Pelletier, John P Roberts, Marc L Melcher, Pooja Singh, Debra L Sudan, Marc P Posner, Jose M El-Amm, Ron Shapiro, Matthew Cooper, George S Lipkowitz, Michael A Rees, Christopher L Marsh, Bashir R Sankari, David A Gerber, Paul W Nelson, Jason Wellen, Adel Bozorgzadeh, A Osama Gaber, Robert A Montgomery, Dorry L Segev
Incompatible living donor kidney transplant (ILDKT) has been established as an effective option for end stage renal disease (ESRD) patients with willing but HLA incompatible live donors, reducing mortality and improving quality of life. Depending upon antibody titer, ILDKT can require highly resource intensive procedure including intravenous immunoglobulin, plasma exchange and/or cell depleting antibody treatment as well as protocol biopsies and DSA testing. This study sought to compare the cost and Medicare reimbursement, exclusive of organ acquisition payment, for ILDKT recipients (N=926) with varying antibody titers to matched compatible transplants (N=2762) performed between 2002-2011...
June 14, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28611164/fulminant-tumefactive-multiple-sclerosis-in-pregnancy
#11
Shabnam Pakneshan, Evanthia Bernitsas
A 35-year-old pregnant woman was admitted with acute onset of paresthesias and word finding difficulty. Initial MRI scan of the brain was suggestive of ischaemic event. During hospitalisation, she had a rapid course with worsening of her neurological deficit that prompted additional testing. Further investigations were consistent with tumefactive multiple sclerosis. After a poor initial response to intravenous steroids and plasma exchange, and discussion of all available therapeutic options, the patient decided to proceed with elective termination...
June 13, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28608569/membrane-based-therapeutic-plasma-exchange-mtpe-technical-and-clinical-experience
#12
Casey N Gashti, Daniel C Andreoli, Dipal Patel
Therapeutic plasma exchange (TPE) has long been utilized to manage a variety of immune-mediated diseases. The basic principle relies on removal of circulating pathogenic substances from the bloodstream. Methods of plasma separation include centrifuge (cTPE) and membrane (mTPE). Although mTPE has existed for a few decades, recent advances in developing highly permeable filters that are compatible with currently existing dialysis machines has opened a new frontier. Published data in the area of technical and clinical experience with mTPE is lacking...
June 13, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28608527/perfect-storm-therapeutic-plasma-exchange-for-a-patient-with-thyroid-storm
#13
Andrea M McGonigle, Aaron A R Tobian, Jennifer L Zink, Karen E King
Thyroid storm is a potentially lethal complication of hyperthyroidism with increased thyroid hormones and exaggerated symptoms of thyrotoxicosis. First-line therapy includes methimazole (MMI) or propylthiouracil (PTU) to block production of thyroid hormones as a bridge toward definitive surgical treatment. Untreated thyroid storm has a mortality rate of up to 30%; this is particularly alarming when patients cannot tolerate or fail pharmacotherapy, especially if they cannot undergo thyroidectomy. Therapeutic plasma exchange (TPE) is an ASFA category III indication for thyroid storm, meaning the optimum role of this therapy is not established, and there are a limited number of cases in the literature...
June 13, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28607779/thrombotic-microangiopathy-secondary-to-intravenous-abuse-of-opana%C3%A2-er
#14
Kamia Thakur, Vaibhav Agrawal, Ashley Kass, Lauren M Dimarino, R Patrick Dorion, Joseph Vadakara
Opana ER (oxymorphone) is an opioid drug available throughout the United States, and intravenous abuse of the crushed oral formulation has been associated with drug-induced thrombotic microangiopathy. In this abstract, we describe two young patients who lived together and used Opana ER intravenously. Both presented with microangiopathic hemolytic anemia that mimicked thrombotic thrombocytopenic purpura (TTP). Treating this condition poses a clinical challenge, as it is difficult to distinguish it from TTP. The role for plasma exchange is not clear but can be used while awaiting the results of the ADAMTS-13 activity, but ultimately supportive care with drug discontinuation is the recommended therapy of choice...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28605161/the-use-of-therapeutic-plasma-exchange-to-reduce-serum-bilirubin-in-a-dog-with-kernicterus
#15
Tricia Tovar, Sarah Deitschel, Christine Guenther
OBJECTIVE: To describe the use of a manual method of therapeutic plasma exchange to reduce total serum bilirubin, manage kernicterus, and halt progression of neurological dysfunction in a dog with immune-mediated hemolytic anemia (IMHA). CASE SUMMARY: A 5-year-old male neutered Lhasa Apso diagnosed with IMHA developed acute onset neurologic signs consistent with kernicterus. Manual therapeutic plasma exchange was performed in an attempt to reduce total serum bilirubin...
June 12, 2017: Journal of Veterinary Emergency and Critical Care
https://www.readbyqxmd.com/read/28604555/immune-related-neurological-symptoms-in-an-adolescent-patient-receiving-the-checkpoint-inhibitor-nivolumab
#16
Dmitry Tchapyjnikov, Alexandra J Borst
Immune checkpoint inhibitors are a novel group of immunotherapeutic agents for the treatment of cancer. Immune-related adverse events, including neurological symptoms, have been associated with these agents. We present the case of an adolescent with refractory Hodgkin lymphoma treated with nivolumab, a PD1 inhibitor, who developed Hashimoto thyroiditis, posterior reversible encephalopathy syndrome causing seizures, as well as urinary retention, truncal/appendicular spasticity, dysphagia, and a progressive encephalopathy that was clinically consistent with a diagnosis of progressive encephalopathy with rigidity and myoclonus, a stiff-person-syndrome spectrum disorder...
June 9, 2017: Journal of Immunotherapy
https://www.readbyqxmd.com/read/28604090/anesthetic-management-of-a-patient-with-multiple-sclerosis
#17
Naohiro Ohshita, Shoko Gamoh, Masahiko Kanazumi, Masahiro Nakajima, Yoshihiro Momota, Yasuo M Tsutsumi
A 54-year-old woman diagnosed with multiple sclerosis (MS) at the age of 19 years was scheduled to undergo temporomandibular joint mobilization. She was currently in a remission phase from her MS but with persistent sequelae, including impaired eyesight and muscle weakness of the limbs. In addition, the blood vessels in her upper limbs were compromised by the formation of internal shunts secondary to vascular prosthesis replacements for plasma exchange therapy in MS. After a previous joint mobilization surgery, her temporomandibular joint developed adhesions with resultant trismus...
2017: Anesthesia Progress
https://www.readbyqxmd.com/read/28599619/a-devastating-case-of-diarrhea-associated-hemolytic-uremic-syndrome-associated-with-extensive-cerebral-infarction-why-we-need-to-do-better
#18
Werner Keenswijk, Evelyn Dhont, Ann Raes, An Bael, Johan Vande Walle
A 4-year-old girl with diarrhea-associated hemolytic uremic syndrome (D+HUS) was transferred to the PICU of our center due to deteriorating renal function and neurological involvement. On admission, a comatous child was seen with hypoventilation and she was placed on mechanical ventilation. Hemodialysis was commenced but plasma exchange was discontinued due to repeated hypersensitivity reactions. A trial of eculizumab was given in light of the worsening of her neurologic condition with development of a pyramidal syndrome and deepening of the coma...
June 9, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28597710/comparing-treatment-options-for-chronic-inflammatory-neuropathies-and-choosing-the-right-treatment-plan
#19
Eduardo Nobile-Orazio, Francesca Gallia, Fabrizia Terenghi, Mariangela Bianco
Chronic inflammatory neuropathies are disorders caused by an immune response to peripheral nerve. They include chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy (MMN) and neuropathy associated with anti-MAG IgM monoclonal gammopathy and other less frequent neuropathies. Several immune therapies have been proven to be effective in these neuropathies even if the best therapeutic option is still unsettled. Areas covered: The authors reviewed the literature to compare the efficacy and safety of currently used immune therapies in these neuropathies...
June 9, 2017: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/28597317/erratum-to-plasma-exchange-response-in-34-patients-with-severe-optic-neuritis
#20
Romain Deschamps, Antoine Gueguen, Nathalie Parquet, Samir Saheb, Francoise Driss, Malcie Mesnil, Catherine Vignal, Jennifer Aboab, Raphael Depaz, Olivier Gout
No abstract text is available yet for this article.
June 8, 2017: Journal of Neurology
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