Pediatric Therapeutic Plasma Exchange Indications and Patterns of Use in US Children's Hospitals Pamela F. Weiss, 1,2,3,4* Andrew J. Klink, 1,2 David F. Friedman, 3,5,6 and Chris Feudtner 2,3,4,7,8 1 Division of Rheumatology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania 2 Center for Pediatric Clinical Effectiveness, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania 3 Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 4 Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 5 Division of Pathology and Laboratory Medicine, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania 6 Division of Pediatric Hematology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania 7 Division of General Pediatrics, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania 8 Leonard Davis Institute of Health Economics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania Purpose: Therapeutic plasma exchange (TPE) has been increasingly used over the past decade as a first-line and lifesaving treatment for various conditions classified by the American society for apheresis (ASFA). To date, the degree to which utilization of TPE in pediatrics mirrors recommendations is unknown. Methods: Using inpatient administrative data from 42 children’s hospitals we conducted an 8-year retrospective cohort study of children (18 years) with an international classification of diseases-9-clinical modification (ICD-9-CM) discharge diagno- sis indicating an ASFA Category I or II condition, or a procedure code indicating receipt of TPE during hospital- ization. Results: TPE was performed during 4,190 hospitalizations of 3,449 patients, of whom 310 (9.0%) and 77 (2.2%) had a primary discharge diagnosis of an ASFA Category I or II condition, respectively. Rates of TPE use for Category I conditions were highest for children with thrombotic thrombocytopenic purpura (TTP), Good- pasture’s syndrome, and myasthenia gravis. TPE use in children’s hospitals significantly increased from 2003 to 2010, but TPE was performed during only 13.4 and 9.3% of hospitalizations for ASFA Category I and II condi- tions, respectively. There was significant between-hospital variation in the use of TPE for Category I conditions as a group and individual Category I conditions including TTP. Conclusion: We found low levels of TPE use across hospitals for key indications, including TTP, a condition for which TPE is considered a first-line and life- saving procedure. The variation identified may contribute to varying clinical outcomes between hospitals, war- rants further investigation, and represents an important opportunity to improve quality of care. J. Clin. Apheresis 00:000–000, 2012. V V C 2012 Wiley Periodicals, Inc. Key words: plasmapheresis; children; adolescents; epidemiology INTRODUCTION Therapeutic plasma exchange (TPE) has been increasingly used over the past decade as a first-line, often life-saving treatment for various conditions. The American society for apheresis (ASFA) assigns condi- tions to 1 of 4 categories based on the quality of pub- lished evidence and strength of recommendations [1– 5]. Classification as an ASFA Category I condition means that TPE is considered a first-line therapeutic option. Examples of Category I conditions include Guillain-Barre ´ syndrome, thrombotic thrombocytopenic purpura (TTP), and myasthenia gravis. TPE is gener- ally accepted as supportive or adjunctive therapy for ASFA Category II conditions. Examples of Category II diseases are familial hypercholesterolemia, antineutro- phil cytoplasmic antibody (ANCA)-associated vasculi- tis, and multiple sclerosis. The use of TPE for Category III and IV conditions is either not established or dis- couraged, respectively. The majority of evidence used in the ASFA guidelines is from adult studies and the recommendations do not distinguish between child- hood- and adult-onset disease. The degree to which TPE utilization in pediatrics mirrors the ASFA recommendations is unknown. For ASFA Category I conditions such as TTP, prompt ini- tiation of TPE may reduce expected mortality from 90 to 10% [6,7]. Poor adherence to ASFA recommenda- tions for TTP should therefore raise concern over the quality of care for children with this disorder and fur- *Correspondence to: Dr. Pamela F. Weiss, Room 1526, North Cam- pus, Division of Rheumatology, The Children’s Hospital of Philadel- phia, 3535 Market Street, Philadelphia, PA 19104, USA. E-mail: weisspa@email.chop.edu. Received 30 January 2012; Accepted 20 June 2012 Published online in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/jca.21242 V V C 2012 Wiley Periodicals, Inc. Journal of Clinical Apheresis 00:000–000 (2012)