title: Report of the ASFA apheresis registry on muscle specific kinase antibody positive myasthenia gravis.
abstract: BACKGROUND
  Anti-muscle specific kinase antibody positive (MuSK Ab) myasthenia gravis (MG) patients are known to have different clinical course compared to anti-acetylcholine receptor Ab positive MG patients. Therapeutic plasma exchange (TPE) has been reported to be effective; however, little is known of the response and of TPE procedural information. An ASFA Apheresis Registry was developed to analyze those data.
  
  
  METHODS
  The study collected detailed de-identified patient data, TPE procedures, and treatment outcome/complications. Collected data was described in aggregate.
  
  
  RESULTS
  A total of 15 MuSK Ab MG patients with exacerbation of MG symptoms, 13 females/2 males, median age 44, were investigated. Thirty TPE courses (median 5 procedures/course, total 145 procedures) were evaluated. All TPE procedures were performed with citrate anticoagulation, 1 - 1.25 plasma volume exchange in 100% fluid balance, and 90% of courses used only albumin as replacement. Calcium was added to albumin or given orally as needed. TPE was performed every other day in 55% of courses. Adverse events occurred in 3.4% of procedures. Ten patients (67%) experienced relapses within a median of 7 weeks. Objective symptoms were resolved in more than 75% of courses. Overall subjective improvement rates were 94.1%/93.3% after 3/4 TPE procedures, respectively. Thirty-one percent of patients responded poorly with minimal recovery.
  
  
  CONCLUSIONS
  Overall subjective improvement was seen up to 94% of patients after one course of TPE. Some patients were poor-responders. Five TPE may be adequate for initial course with additional TPE as needed. Based upon this preliminary data, we will modify our future data collection. J. Clin. Apheresis 32:5-11, 2017. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Division of Transfusion Medicine, Department of Pathology, University of Michigan, Ann Arbor, Michigan.
authors.forename: Chisa
authors.initials: C
authors.lastname: Yamada
authors.affiliation: Division of Laboratory Medicine, Department of Pathology, University of Alabama at Birmingham, Birmingham, Alabama.
authors.forename: Huy P
authors.initials: HP
authors.lastname: Pham
authors.affiliation: Bloodworks Northwest, Seattle, Washington DC.|Division of Laboratory Medicine, Yale Medical School, New Haven, Connecticut.
authors.forename: Yanyun
authors.initials: Y
authors.lastname: Wu
authors.affiliation: Division of Transfusion Medicine, Department of Pathology, University of Michigan, Ann Arbor, Michigan.
authors.forename: Laura
authors.initials: L
authors.lastname: Cooling
authors.affiliation: Division of Transfusion Medicine, Department of Pathology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Haewon C
authors.initials: HC
authors.lastname: Kim
authors.affiliation: American Red Cross, St. Paul, Minnesota.
authors.forename: Shanna
authors.initials: S
authors.lastname: Morgan
authors.affiliation: Division of Transfusion Medicine, Department of Pathology and Cell Biology, Columbia University, Ney York, New York.
authors.forename: Joseph
authors.initials: J
authors.lastname: Schwartz
authors.affiliation: Division of Transfusion Medicine, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.
authors.forename: Jeffrey L
authors.initials: JL
authors.lastname: Winters
authors.affiliation: Division of Laboratory Medicine, Department of Pediatrics, Center for Cancer and Blood Disorders, Children's National Medical Center, George Washington School of Medicine and Health Science, Washington, DC.|Department of Pathology, Center for Cancer and Blood Disorders, Children's National Medical Center, George Washington School of Medicine and Health Science, Washington, DC.
authors.forename: Edward C C
authors.initials: EC
authors.lastname: Wong
mesh_terms: D000328:Adult; D001323:Autoantibodies; D005260:Female; D006801:Humans; D008297:Male; D009157:Myasthenia Gravis / Q000276:immunology / Q000628:therapy*; D010951:Plasma Exchange / Q000009:adverse effects / Q000379:methods*; D020794:Receptor Protein-Tyrosine Kinases / Q000276:immunology; D011950:Receptors, Cholinergic / Q000276:immunology; D012042:Registries; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D011950:Receptors, Cholinergic; C095714:MUSK protein, human; D020794:Receptor Protein-Tyrosine Kinases
keywords: ASFA apheresis registry; MuSK MG; therapeutic plasma exchange