Guidelines on the use of therapeutic apheresis in clinical practice--evidence-based approach from the Apheresis Applications Committee of the American Society for Apheresis.
ABSTRACT The American Society for Apheresis (ASFA) Apheresis Applications Committee is charged with a review and categorization of indications for therapeutic apheresis. Beginning with the 2007 ASFA Special Issue (fourth edition), the subcommittee has incorporated systematic review and evidence-based approach in the grading and categorization of indications. This Fifth ASFA Special Issue has further improved the process of using evidence-based medicine in the recommendations by refining the category definitions and by adding a grade of recommendation based on widely accepted GRADE system. The concept of a fact sheet was introduced in the Fourth edition and is only slightly modified in this current edition. The fact sheet succinctly summarizes the evidence for the use of therapeutic apheresis. The article consists of 59 fact sheets devoted to each disease entity currently categorized by the ASFA as category I through III. Category IV indications are also listed.
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ABSTRACT: Patients with homozygous familial hypercholesterolemia (HoFH) represent the most severe patients within the spectrum of dyslipidemias. Untreated Low-Density Lipoprotein Cholesterol (LDL-C) levels in these patients are usually in the range 500 to 1200 mg/dL. Moreover, these patients exhibit a scarce responsiveness or even non responsiveness to oral lipid lowering agents. Patients with heterozygous familial hypercholesterolemia (HetFH) tend to have untreated LDL-C levels of 250-500 mg/dL. Many of these patients are responsive to 3-hydroxy-3-methylglutaryl-coenzyme A (HMGCoA-reductase) inhibitors (statins) and/or other specific drugs. Unfortunately, a significant subset of these patients (5-10%) have a severe and/or refractory form of HetFH and after current maximal oral therapy, they remain significantly far from treatment goals.(The National Cholesterol Education Program (NCEP) ATPIII guidelines).This would be defined as LDL-C levels of ≥ 190 mg/dL - prior Coronary Heart Disease (CHD) or CHD equivalent - or ≥ 250 mg/dL (no prior CHD or CHD risk-equivalent). The only current therapy option for these patients is Low Density Lipoproteins-apheresis (LDL_a). While LDL_a is very effective in reducing LDL-C, many patients do not receive this extracorporeal therapy because of costs and limited availability of LDL_a centers. Recently, new potent lipid-lowering drugs have been developed and are currently under investigation. Proprotein convertase subtilisin/kexin type 9 (PCSK9) plays a critical role controlling the levels of LDL-C. Studies have demonstrated that PCSK9 acts mainly by enhancing degradation of the Low-Density Lipoprotein receptor (LDLR) protein in the liver. Inactivation of PCSK9 in mice reduces plasma cholesterol levels. Since the loss of a functional PCSK9 in human is not associated with apparent deleterious effects, this protease is becoming an attractive target for lowering plasma LDL-C levels either alone or in combination with statins. Mipomersen, an apolipoprotein B (ApoB) synthesis inhibitor, for lowering of LDL-C showed to be an effective therapy to reduce LDL-C concentrations in patients with HoFH who are already receiving lipid-lowering drugs, including high-dose statins. Lomitapide is a potent inhibitor of microsomal triglyceride transfer protein and is highly efficacious in reducing LDL-C and triglycerides (TG). Lomitapide is currently being developed for patients with HoFH at doses up to 60 mg/d. These new powerful lipid-lowering drugs might be possibly superior than available hypolipidemic agents. Their mechanisms of action, effectiveness, safety, and indication in severe, genetically determined dyslipidemias, are reviewed.Current Medicinal Chemistry 09/2012; · 4.86 Impact Factor
Article: Medications in patients treated with therapeutic plasma exchange: prescription dosage, timing, and drug overdose.[show abstract] [hide abstract]
ABSTRACT: Therapeutic plasma exchange (TPE) is an extracorporeal process commonly used in clinical medicine for the treatment of a variety of neurological, renal, hematological, dermatological, and other diseases. Inherent to the procedure, patients' plasma removal may lead to the extraction of drugs they are concurrently receiving. This review discusses the published literature assessing TPE's influence on different drug classes' disposition and, when applicable, sets forth management recommendations in cases where the drugs are used at the usual doses and in cases of drug overdose.Seminars in Dialysis 02/2012; 25(2):176-89. · 2.27 Impact Factor
Chapter: Low Density Lipoprotein Apheresis[show abstract] [hide abstract]
ABSTRACT: Lipid-apheresis (LA) and LDL-apheresis (LDLa) are extracorporeal techniques which permit the unselective or specific removal of lipids and lipoproteins, namely Low Density Lipoproteins (LDL), as well as other apolipoprotein B100 (apoB)-containing lipoproteins from plasma -Intermediate Density Lipoproteins, Lipoprotein(a)-[IDL, Lp(a)]. LDLa represents a selective upgrading (with both clinical and metabolic advantages) from conventional forms of extracorporeal therapy such as plasma-exchange (PEX) used in the seventies to treat severe hypercholesterolaemia. The primary reason to use LDLa is the treatment of homo-, double-(compound), and heterozygous familial hypercholesterolaemia (Hoz-DHtz-Htz FH). This technique also been shown efficacious in the treatment of other severe forms of hyperlipoproteinemia such as Autosomal Recessive Familial Hypercholesterolaemia, HyperLp(a)lipoproteinemia, Familial Combined Hyperlipoproteinemia, and other lipid metabolism disturbances associated with an elevated cardiovascular risk (CVR) when used on patients who are poor-or non-responders to pharmacological treatment following specific guidelines for the reduction of cholesterol in plasma. Patients with these severe forms of dyslipidemia and, particularly, those affected by FH are subject to coronary ischemic events and thus require an intensive, efficacious, constant, continuous, and personalized form of therapy. A therapy based solely on pharmacological drugs does not achieve the desired results in the Hoz-and DHtz forms of FH as well as in approximately 15-20% of the Htz form. In the aforementioned clinical conditions LDLa treatment offers a necessary therapeutic Correspondence: Claudia Stefanutti, Extracorporeal Therapeutic Techniques Unit -Immunohematology and04/2013: pages 12; , ISBN: 978-1-62257-701-9