Since 1986, the American Society for Apheresis (ASFA) has published practice guidelines on the use of therapeutic apheresis in the Journal of Clinical Apheresis. Since 2007, the guidelines are published in regular intervals to reflect current evidence-based apheresis practice with the most recent edition published in 2019. The ASFA guidelines are written in a user-friendly fact sheet format and represent a concise yet comprehensive review of the English language literature on the use of apheresis to treat disease. The role of the guidelines is to provide the most current information available to apheresis practitioners. The PEXIVAS study is an international, randomized controlled trial comparing therapeutic plasma exchange (TPE) versus no TPE and steroid dosing regimen on the primary composite outcome of end stage renal disease or death in patients with ANCA-associated vasculitis. The study was published in early 2020 in the NEJM. This is the largest study on the role of therapeutic apheresis in ANCA-associated vasculitis published to date. The study showed the TPE does not reduce the risk of ESRD or death in patients with ANCA-associated vasculitis. Based on these findings, an interim updated fact sheet was recently published. In this interim fact sheet, the category recommendation for rapidly progressive glomerulonephritis in the setting of microscopic polyangiitis, granulomatosis with polyangiitis, or renal-limited vasculitis with Cr ≥5.7mg/dL (includes “on dialysis”) was changed from category I to category II. Similarly, the grade of evidence was changed from IA to IB to acknowledge previously described important limitations of the PEXIVAS study including the lack of biopsy to define disease severity and the long follow-up period, which may make it difficult to detect initial improvement in the subset of patients at first presentation. This recent seminal publication and its implication for therapeutic apheresis will be discussed. Other topics with new information that will be addressed in this presentation include Hereditary TTP. A recent review on the prevalence, pathogenesis, clinical features of this disorder, as well as therapeutic options was published. Although Hereditary TTP is not currently categorized in the Therapeutic Apheresis guidelines, indications for TPE as well as the use of plasma infusion, and eventually rhADAMTS13 enzyme in this disorder will be discussed. Similarly, Hemophagocytic Lymphohistiocytosis/Macrophage Activating Syndrome (HLH/MAS) will be reviewed including a recent retrospective case series showing use of TPE in combination with immunosuppressive therapy in this disorder.