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Therapeutic Plasma Exchange for Myasthenia Gravis

FOR HEALTHCARE PROFESSIONALS

Plasma exchange as first-line therapy for myasthenia gravis (MG)

Plasma exchange is recommended as first-line therapy by the American Society for Apheresis (ASFA), the European Federation of Neurological Societies (EFNS), the German Neurological Society (GNS) and the Myasthenia Gravis Foundation of America (MGFA) for acute/crisis and refractory myasthenia gravis and pre-thymectomy. The Spectra Optia™ Apheresis System from Terumo Blood and Cell Technologies is the first therapeutic apheresis system indicated to treat MG.

When mapping a treatment plan for MG, seeing results as quickly as possible can be critical in order to hasten recovery or maintain optimal symptom management.

Plasma exchange rapidly reduces disease mediators, essentially quieting the immune response and relieving symptoms.¹ For an MG crisis, this may mean hastening recovery, such as enabling the patient to come off the ventilator faster and achieve quicker functional outcome improvement.¹˒²˒³

"Although clinical trials suggest that intravenous immunoglobulin (IVIg) and plasma exchange are equally effective in the treatment of impending or manifest myasthenic crisis, expert consensus suggests that plasma exchange is more effective and works more quickly."³


Time to clinical effect of therapies for MG¹

Treatment

Time to Clinical Effect

Pyridostigmine

10 – 15 minutes

Plasma exchange

1 – 14 days

IVIg

1 – 4 weeks

Prednisone

2 – 8 weeks

Mycophenolate mofetil

2 – 6 months

Cyclosporine

2 – 6 months

Azathioprine

3 – 18 months


Speed to recovery for MG and MuSK+ MG patients

Studies show therapeutic plasma exchange (TPE) provides significant clinical benefits when used as a stand-alone therapy for MG patients, including those with moderate-severe generalized, refractory, crisis, and muscle-specific tyrosine kinase MG (MuSK+ MG). TPE has been shown to be effective for the majority of patients in these groups — not all patients respond the same to therapy.³˒⁴˒⁵˒⁶

  • A reduction in disease severity by 2 grades experienced by 89% of 18 patients⁷
  • Improvements in the MG disease scale shown in 78.7% of 33 patients⁸
  • An improvement in functional grading from a median of 4 to 1⁹ experienced in 100% of 11 patients
  • Full recovery from MG crisis in 81% of 16 patients¹⁰

Multiple studies and expert opinions support that plasma exchange has superior efficacy over IVIg for MuSK+ MG.¹˒³˒⁵˒¹¹˒¹²

Retrospective chart review of 53 MuSK+ MG patients at nine university-based centers in the U.S. showed that 51% of patients improved with plasma exchange versus 20% with IVIg.¹²

In a 2011 review of 110 MuSK+ MG patients from two large clinics in Italy and the U.S., plasma exchange produced improvement in 93% of patients, compared with 61% who improved after IVIg. Clinical improvement was determined by a treating physician.¹¹

European Federation of Neurological Societies (EFNS) 2010¹³

  • Acute exacerbation (Level A)
  • Pre-thymectomy (Level B)

American Society for Apheresis (ASFA) 2016⁶

  • Moderate-severe Category I (Grade 1B)
  • Pre-thymectomy Category I (Grade 1C)

Myasthenia Gravis Foundation of America (MGFA) Consensus 2016³

  • Short-term treatment in myasthenic crisis
  • Pre-thymectomy
  • Refractory MG
  • Prior to corticosteroids

German Neurological Society (GNS) 2016¹⁴

  • Myasthenic crisis — Category I

Contraindications
Leukocytapheresis is contraindicated in acute myeloid leukemia FAB M3 (acute promyelocytic leukemia) because of the accompanying disseminated intravascular coagulation.9,10 Other contraindications for the use of the Spectra Optia system are limited to those associated with the infusion of solutions and replacement fluids as required by the apheresis procedure and those associated with all types of automated apheresis systems.

Adverse events of apheresis procedures can include
Anxiety, headache, light-headedness, digital and/or facial paresthesia, fever, chills, hematoma, hyperventilation, nausea and vomiting, syncope (fainting), urticaria, hypotension, allergic reactions, infection, hemolysis, thrombosis in patient and device, hypocalcemia, hypokalemia, thrombocytopenia, hypoalbuminemia, anemia, coagulopathy, fatigue, hypomagnesemia, hypogammaglobulinemia, adverse tissue reaction, device failure/disposable set failure, air embolism, blood loss/anemia, electrical shock, fluid imbalance, and inadequate separation of blood components.

Reactions to blood products transfused during procedures can include
Hemolytic transfusion reaction, immune-mediated platelet destruction, fever, allergic reactions, anaphylaxis, transfusion-related acute lung injury (TRALI), alloimmunization, posttransfusion purpura (PTP), transfusion-associated graft-versus-host disease (TA-GVHD), circulatory overload, hypothermia, metabolic complications, and transmission of infectious diseases and bacteria.11,12

Restricted to prescription use only

  • Operators must be familiar with the system's operating instructions.
  • Procedures must be performed by qualified medical personnel.

Disclaimers and notes

  1. Saperstein D, Barohn R. Management of myasthenia gravis. Semin Neurology. 2004;24(1):41-48.
  2. Quereshi A, Choudhry M, Akbar M, et al. Plasma exchange versus intravenous immunoglobulin treatment in myasthenic crisis. Neurology. 1999;52(3):629-632.
  3. Sanders DB, Wolfe GI, Benatar M, et al. International consensus guidance for management of myasthenia gravis: Executive summary. Neurology. 2016;87(4):419-425.
  4. Ebadi H, Barth D, Bril V. Safety of plasma exchange therapy in patients with myasthenia gravis. Muscle Nerve. 2013;47(4):510-514.
  5. Guptill JT, Juel VC, Massey JM, et al. Effect of therapeutic plasma exchange on immunoglobulins in myasthenia gravis. Autoimmunity. 2016;49(7):472-479.
  6. Schwartz J, Padmanabhan A, Aqui N, et al. Guidelines on the use of therapeutic apheresis in clinical practice — Evidence-based approach from the writing committee of the American Society for Apheresis: The seventh special issue. J Clin Apher. 2016;31(3):149-162.
  7. Szczeklik W, Wawryka K, Wlodarcyzk A, et al. Complications in patients treated with plasmapheresis in the intensive care unit. Anesthesial Intensive Ther. 2013;45(1):7-13.
  8. Trikha I, Singh S, Goyal V, Shukla G, Bhasin R, Behari M. Therapeutic plasma exchange in the treatment of neuroimmunologic disorders: review of 50 cases. Neurology. 2007;254:989-995.
  9. Kaynar L, Altuptas F, Aydogedu T, et al. Therapeutic plasma exchange in patients with neurologic diseases: retrospective multicenter study. Transfus Apher Sci. 2008;38(2):109-115.
  10. Sorgun M, Erdogan S, Bay M, et al. Therapeutic plasma exchange in treatment of neuroimmunologic disorders: Review of 92 cases. Transfus Apher Sci. 2013;49(2):174-180.
  11. Guptill J, Sanders D, Evoli A. Anti-MuSK antibody myasthenia gravis: clinical findings and response to treatment in two large cohorts. Muscle Nerve. 2011;44(1):36-40.
  12. Pasnoor M, Wolfe G, Nations S, et al. Clinical findings in MuSK-antibody positive myasthenia gravis: A U.S. experience. Muscle Nerve. 2010;41(3):370-374.
  13. Skeie GO, Apostolski S, Evoli A, et al. Guidelines for treatment of autoimmune neuromuscular transmission disorders. Eur J Neurol. 2010;17(7):893-902.
  14. Meltzer N, Ruck T, Fuhr P, et al. Clinical features, pathogenesis, and treatment of myasthenia gravis: a supplement to the Guidelines of the German Neurological Society. J Neurol. 2016; 263:1473-1497.
  15. Vahdat L, Maslak P, Miller WH Jr, et al. Early mortality and the retinoic acid syndrome in acute promyelocytic leukemia: impact of leukocytosis, low-dose chemotherapy, PMN/RAR-alpha isoform and CD13 expression in patients treated with all-trans retinoic acid. Blood. 1994;84(11):3843-3849.
  16. Daver N, Kantarjian H, Marcucci G, et al. Clinical characteristics and outcomes in patients with acute promyelotic leukaemia and hyperleucocytosis. Br J Haematol. 2015;168(5):646-653.
  17. AABB. Circular of Information for the Use of Human Blood and Blood Components. Bethesda, MD: AABB; 2017.
  18. European Directorate for the Quality of Medicines & HealthCare (EDQM). Guide to the Preparation, Use and Quality Assurance of Blood Components. 19th edition. Strasbourg, France: EDQM Council of Europe; 2017.
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