Search results for "Plasma Exchange"
showing 10 items of 17 documents
Redefining outcomes in immune TTP: an international working group consensus report
2021
Abstract Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a potentially fatal thrombotic microangiopathy caused by autoantibody-mediated severe deficiency of ADAMTS13. Standardized definitions of response, exacerbation, remission, and relapse were initially proposed in 2003 and modified by the International Working Group for TTP in 2017. These definitions, which have been widely used in clinical practice and research, are based primarily on the platelet count and are benchmarked against the timing of discontinuation of therapeutic plasma exchange (TPE). They do not incorporate ADAMTS13 activity or the temporizing effects on the platelet count of caplacizumab, a novel anti–von W…
Albumin versus solvent/detergent-treated pooled plasma as replacement fluid for long-term plasma exchange therapy in a patient with primary hypertrig…
2015
Chylomicronemia syndrome is a metabolic condition characterized by severe fasting hypertrigliceridemia (≥ 1000 mg/dL) and other clinical features including chronic abdominal pain and recurrent acute pancreatitis. In patients with acute or recurrent pancreatitis, plasma exchange (PEx) is indicated for the treatment of acute disease and prevention of recurrence. The use of plasma instead of albumin as replacement fluid has been suggested for its putative ability to replace the deficient enzyme possibly leading to better clinical improvement.A 40-year-old man with chylomicronemia syndrome due to a newly identified loss-of-function mutation in the lipoprotein lipase (LPL) gene (IVS2, c.250-1G/C…
To do or not to do? plasma exchange and timing of steroid administration in progressive multifocal leukoencephalopathy
2016
OBJECTIVE: To retrospectively analyze the effect of plasma exchange (PLEX; yes = PLEX+ , no = PLEX- ) and steroids administration timing (prophylactically [proST] or therapeutically [therST]) on the longitudinal clinical course of patients with natalizumab-related progressive multifocal leukoencephalopathy (PML) and full-blown immune reconstitution inflammatory syndrome (PML-IRIS). METHODS: Clinical and radiological data of 42 Italian patients with PML were analyzed. Patient's data are available until 12 months after PML diagnosis. PLEX and steroids treatment as time-dependent covariates were entered in: (1) a Cox model to investigate their impact on full-blown PML-IRIS latency; (2) an anal…
Continuous intravenous infusion of dipyridamole as adjunctive therapy in the treatment of thrombotic thrombocytopenic purpura.
2003
Abstract Thrombotic thrombocytopenic purpura (TTP) is an uncommon hematologic thrombotic disorder characterized by fever, hemorrhagic and neurologic signs. The advent of plasma exchange has dramatically improved the prognosis of this disease, which was once inevitably fatal. However, mortality rates remain significant. Antiplatelet drugs have been widely used in combination with plasma exchange. In this pilot study we investigated the effects of an adjunctive therapy consisting of the continuous, intravenous infusion of dipyridamole, a modality of administration that has not been previously tested in this setting. Sixteen untreated TTP patients, diagnosed consecutively at our clinic, receiv…
Rare association of herpes simplex virus IgM-specific antibodies and Guillain-Barré syndrome successfully treated with plasma exchange and immunosupp…
1985
Herpes simplex virus (HSV) has been associated with various neurological disorders. In contrast, HSV infection is very rarely found in acute polyneuroradiculitis. In this report, a patient is described with a severe course of Guillain-Barr¿ syndrome (GBS). HSV IgM-specific antibodies and a rise of complement-fixation antibodies were detected. During the acute phase of neurologic syndrome, a nerve biopsy showed myelin damage and IgM deposits on the inner layer of the perineurium. Plasma exchange, in combination with immunosuppression, was successfully applied as a treatment in the relapsing course of GBS. Finally, after recovery, HSV-specific IgM antibodies disappeared.
Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura
2011
Thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder characterised by fever, microangiopathic haemolytic anaemia, renal insufficiency, and thrombocytopenia. Neurological involvement, a prominent component of TTP, is characterised by a variety of brain lesions which include reversible cerebral oedema or magnetic resonance imaging (MRI) features of reversible posterior leukoencephalopathy syndrome (RPLS). TTP is frequently associated with deficiency of the von Willebrand factor-cleaving protease, ADAMTS13.Here, we report a case of TTP with severe acute encephalopathy. Posterior leukoencephalopathy and brainstem oedema with triventricular hydrocephalus were observed on MRI. The …
Drug‐refractory myasthenia gravis: Clinical characteristics, treatments, and outcome
2022
[Objective] To describe the clinical characteristics and outcomes in patients with refractory myasthenia gravis (MG) and to determine the effectiveness and side effects of the drugs used for their treatment.
Tryptophan immunoadsorption during pregnancy and breastfeeding in patients with acute relapse of multiple sclerosis and neuromyelitis optica.
2017
Background: Up to every fourth woman with multiple sclerosis (MS) or neuromyelitis optica spectrum disorder (NMOSD) suffers a clinically relevant relapse during pregnancy. High doses of steroids bear some serious risks, especially within the first trimester of pregnancy. Immunoadsorption (IA) is an effective and more selective treatment option in disabling MS relapse than plasma exchange. Data on the use of IA during pregnancy and breastfeeding are scarce. Methods: In this retrospective multicenter study, we analyzed the safety and efficacy of IA treatment in acute relapses during pregnancy or breastfeeding. The primary outcome parameter - change of acute relapse-related disability after IA…
Guillain-Barré Syndrome
2022
Background: Guillain–Barré syndrome is a rare disorder in which our body’s immune system attacks nerves determining weakness and tingling of extremities as first symptoms. It can also be associated to respiratory failure and require mechanical ventilation during hospitalization (up to 30% of patients). Nowadays patient’s hyper-reactive immune responses benefits from immunotherapies such as intravenous immunoglobulin (IVIg), therapeutic plasma exchange (TPE) and new biological drugs. Case Report: We report our experience with the case of a 64-year-old woman who presented a symmetric progressive flaccid paralysis after a week of mild cold symptoms. The respiratory and neurological symptoms wo…