Report of the ASFA apheresis registry on muscle specific kinase antibody positive myasthenia gravis |
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Authors: | Chisa Yamada Huy P. Pham Yanyun Wu Laura Cooling Haewon C. Kim Shanna Morgan Joseph Schwartz Jeffrey L. Winters Edward C.C. Wong |
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Affiliation: | 1. Division of Transfusion Medicine, Department of Pathology, University of Michigan, Ann Arbor, Michigan;2. Division of Laboratory Medicine, Department of Pathology, University of Alabama at Birmingham, Birmingham, Alabama;3. Bloodworks Northwest, Seattle, Washington DC;4. Division of Laboratory Medicine, Yale Medical School, New Haven, Connecticut;5. Division of Transfusion Medicine, Department of Pathology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania;6. American Red Cross, St. Paul, Minnesota;7. Division of Transfusion Medicine, Department of Pathology and Cell Biology, Columbia University, Ney York, New York;8. Division of Transfusion Medicine, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota;9. 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;10. Department of Pathology, Center for Cancer and Blood Disorders, Children's National Medical Center, George Washington School of Medicine and Health Science, Washington, DC |
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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. Conclusion : 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. |
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Keywords: | ASFA apheresis registry therapeutic plasma exchange MuSK MG |
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