DOI: 10.1002/jca.21705 Guidelines on the Use of Therapeutic Apheresis in Clinical Practice – Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue Anand Padmanabhan1 | Laura Connelly-Smith2 | Nicole Aqui3 | Rasheed A. Balogun4 | Reinhard Klingel5 | Erin Meyer6 | Huy P. Pham7 | Jennifer Schneiderman8 | Volker Witt9 | Yanyun Wu10 | Nicole D. Zantek11 | Nancy M. Dunbar12 | Guest Editor: Joseph Schwartz13 1Medical Sciences Institute & Blood Research Institute, Versiti & Department of Pathology, Medical College of Wisconsin, Milwaukee, Wisconsin 2Department of Medicine, Seattle Cancer Care Alliance & University of Washington, Seattle, Washington 3Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 4Department of Medicine, University of Virginia, Charlottesville, Virginia 5Apheresis Research Institute, Cologne, Germany & First Department of Internal Medicine, University of Mainz, Mainz, Germany 6Department of Hematology/Oncology/BMT/Pathology, Nationwide Children’s Hospital, Columbus, Ohio 7Department of Pathology, Keck School of Medicine of the University of Southern California, Los Angeles, California 8Department of Pediatric Hematology/Oncology/Neuro-oncology/Stem Cell Transplant, Ann & Robert H. Lurie Children’s Hospital of Chicago, Northwestern University, Chicago, Illinois 9Department for Pediatrics, St. Anna Kinderspital, Medical University of Vienna, Vienna, Austria 10Bloodworks NW & Department of Laboratory Medicine, University of Washington, Seattle, Washington, Yale University School of Medicine, New Haven, Connecticut 11Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota 12Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire 13Department of Pathology and Cell Biology, Columbia University Medical Center, New York, New York Correspondence ABSTRACT Nancy M. Dunbar, MD; Dartmouth- The American Society for Apheresis (ASFA) Journal of Clinical Apheresis (JCA) Hitchcock Medical Center, Lebanon, Special Issue Writing Committee is charged with reviewing, updating and categoriz- NH 03756. Email: [email protected] ing indications for the evidence-based use of therapeutic apheresis (TA) in human dis- ease. Since the 2007 JCA Special Issue (Fourth Edition), the committee has incorporated systematic review and evidence-based approaches in the grading and cat- egorization of apheresis indications. This Eighth Edition of the JCA Special Issue con- tinues to maintain this methodology and rigor in order to make recommendations on the use of apheresis in a wide variety of diseases/conditions. The JCA Eighth Edition, like its predecessor, continues to apply the category and grading system definitions in fact sheets. The general layout and concept of a fact sheet that was introduced in the Fourth Edition, has largely been maintained in this edition. Each fact sheet succinctly J Clin Apher. 2019;34:171–354. wileyonlinelibrary.com/journal/jca © 2019 Wiley Periodicals, Inc. 171 172 PADMANABHAN ET AL. summarizes the evidence for the use of TA in a specific disease entity or medical con- dition. The Eighth Edition comprises 84 fact sheets for relevant diseases and medical conditions, with 157 graded and categorized indications and/or TA modalities. The Eighth Edition of the JCA Special Issue seeks to continue to serve as a key resource that guides the utilization of TA in the treatment of human disease. 1 | INTRODUCTION table, disease description, current management, rationale for TA, technical notes (e.g., volumes treated, frequency, replace- The Writing Committee of the Journal of Clinical Apheresis ment fluid), duration and discontinuation of treatment, and (JCA) Special Issue 2019 is pleased to present the Eighth Edi- provided a maximum of 20 key references highlighting tion of the JCA Special Issue. After more than 2 years of important or new studies and/or reviews (Figure 1). Two other engaging collaborative work, and the rigorous critical review committee members, along with an external expert for select of fact sheets contained herein, we believe that this document fact sheets, provided secondarypeer-reviewofeachfactsheet. will appeal to both practitioners with a focus in the area of The entire writing committee performed a third and final review apheresis medicine and other physicians who may need to uti- of all fact sheets with category and grade assigned by consensus lize therapeutic apheresis (TA) occasionally for the care of in the same manner as described in previous editions with con- their patients. This latest iteration of evidence-based ASFA sistent application of evaluationcriteria.Thisevidence-based categories is based upon a stringent review of up-to-date liter- approach is designed to achieve several objectives. First, it pro- ature, analysis of the quality of evidence, and the strength of vides uniformity to ASFA category assignment and disease dis- recommendation derived from this evidence. cussion while minimizing personal bias. Second, it provides the This Special Issue is a compilation of fact sheets for 84 dis- strength of recommendation [strong (1) vs. weak (2)] using a eases (Table 1). To clarify terminology used in this table and defined grading schema. Finally, it provides comprehensive, yet throughout this document, “Disease” refers to a specific dis- succinct information easily shared with healthcare providers ease or medical condition (e.g., myasthenia gravis [disease]; requesting information on the potential utility of apheresis in a transplantation, liver [medical condition]) and represents the given clinical setting. The entire process of fact sheet develop- pathology discussed in the fact sheet. “Indication” refers to ment is shown in Figure 2. the use of apheresis in specific situations encountered in the Several diseases or conditions underwent review in con- disease (e.g. acute, short-term treatment [indication]). Each sideration for the development of a new fact sheet (Table 6). disease, TA modality and indication is assigned a category To meet criteria for a new fact sheet, the committee required (Table 2) and grade (Table 3) as in previous editions. In this a minimum of 10 cases published in the last decade in peer- edition, we have continued to use the table format at the start reviewed journals, ideally by more than one group. Based of each fact sheet to summarize disease name, TA modality on these criteria, there were no new disease categories added (Table 4), indication(s), category, and grade. Several diseases to the JCA 2019 Special Issue. Strong consideration was or conditions that are category IV, which have been described given for the addition of a new fact sheet on Alzheimer’s in detail in previous editions and do not have significant new disease. The published evidence for the use of TPE in evidence since the last publication, are summarized in a sepa- Alzheimer’s disease is currently limited. Preliminary data rate table (Table 5). from the recently concluded phase IIb/III Alzheimer Man- The 2019 JCA Special Issue Writing Committee com- agement by Albumin Replacement (AMBAR) study is avail- prised 13 members from diverse fields including Transfu- able in abstract form but is not yet published in a peer- sion Medicine/Apheresis, Hematology/Oncology, Pediatrics, reviewed journal. Thus, Alzheimer’s disease was not Nephrology, and Critical Care Medicine from locations across selected for inclusion in the JCA 2019 Special Issue. the United States and Europe. Each disease or condition was Some previously published fact sheets were renamed to assigned to one committee member as primary author. That group fact sheets together by similar disease pathology and/or primary author reviewed any new developments in the under- treatment. For example, “ANCA-associated rapidly progressive standing, current management, and treatment of the disease or glomerulonephritis” and “Henoch-Schönlein purpura” were condition as well as any changes in the evidence surrounding renamed “Vasculitis, ANCA-associated” and “Vasculitis, IgA the use of TA as a treatment modality. Only peer-reviewed (Henoch-Schönlein purpura)” respectively with a separate fact PubMed-indexed publications available in English were con- sheet for “Vasculitis, other.” All fact sheets involving trans- sidered when reviewing literature published since the last fact plantation have been renamed “Transplantation, transplant- sheet update. The primary author updated each fact sheet type…” The “Aplastic anemia, pure red cell aplasia” fact sheet PADMANABHAN ET AL. 173 TABLE 1 Category and Grade Recommendations for Therapeutic Apheresis Disease TA modality Indication Category Grade Page Acute disseminated TPE Steroid Refractory II 2C 187 encephalomyelitis (ADEM) Acute inflammatory demyelinating TPE Primary Treatment I 1A 189 polyradiculoneuropathy (Guillain- IA Primary Treatment I 1B Barré syndrome) Acute liver failure TPE-HV I 1A 191 TPE III 2B Age related macular degeneration, Rheopheresis High-risk II 2B 193 dry Amyloidosis, systemic ß2-microglobulin Dialysis-related II 2B 195 column amyloidosis TPE Other causes IV 2C Anti-glomerular basement TPE Diffuse alveolar I 1C 197 membrane disease (Goodpasture hemorrhage (DAH) syndrome) TPE Dialysis- independence I 1B TPE Dialysis-dependence, no III 2B DAH Atopic (neuro-) dermatitis ECP III 2A 199 (atopic eczema), recalcitrant IA III 2C TPE/DFPP III 2C Autoimmune