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Therapeutic Plasma Exchange for Myasthenia G ravis

Therapeutic Plasma Exchange for Myasthenia G ravis. Clifford Blieden, MD Houston Methodist Hospital Department of Pathology and Genomic Medicine, Division of Transfusion Medicine. Who Are We?. P hysicians with residency training in Clinical P athology with or without Anatomic P athology

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Therapeutic Plasma Exchange for Myasthenia G ravis

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  1. Therapeutic Plasma Exchange for Myasthenia Gravis Clifford Blieden, MD Houston Methodist Hospital Department of Pathology and Genomic Medicine, Division of Transfusion Medicine

  2. Who Are We? • Physicians with residency training in Clinical Pathology with or without Anatomic Pathology • Fellowship trained in Transfusion Medicine • WE TAKE CALL!!!!

  3. Myasthenia Gravis • Neuromuscular disorder caused by circulating autoantibodies to the acetylcholine receptors in the post synaptic neuromuscular junction • Associated with a variety of other autoimmune diseases such as Hashimotos thyroiditis, type I diabetes, lupus

  4. Common Modalities for Diagnosing Myasthenia Gravis • Serologic testing • Acetylcholine receptor antibodies • Muscle specific (MuSK) receptor antibodies • 50% of seronegative MG patients may have antibodies to MuSK • Muscle electromyography • Edrophonium (cholinesterase inhibitors) test • Muscle biopsy • IgG deposition at neuromuscular junction by immunofluorescence

  5. Current Treatment Options • Medications: • Increase acetylcholine (cholinesterase inhibitors; neostigmine, pyrostigmine) • Decrease immune response (corticosteroids) • Plasma exchange to remove antibodies • Thymectomy • Thymoma and MG are frequently associated

  6. Plasmapharesis • Word is derived from “plasma” and Greek word “aphaeresis” (take away) • Blood is removed from the body and separated into separate components (plasma and red cell component) • Continuous process requiring an afferent and efferent site of blood supply.

  7. Why Plasma Exchange? • Antibodies reside in the plasma component of the blood • Removal of these antibodies temporarily decreases symptoms of myasthenia gravis • Scheduled plasma exchange regimen useful in patients with disease not helped by medications

  8. TPE and Myasthenia Gravis • TPE is used to remove antibodies present in the plasma component • May be applied in multiple settings (outpatient, inpatient, perioperative) • Regarded as a category I indication (ASFA guidelines) • Category I = disorders for which apheresis is accepted as first line therapy, either alone or in conjunction with other treatment modalities • Patients MAY experience improvement of symptoms within 24 hours

  9. Common side effects of plasma exchange • Paresthesia • Hypotension • Urticaria • Nausea • Abdominal cramps

  10. Hypocalcemia • Common occurrence with plasma exchange due to citrate, an anticoagulant necessary to prevent blood from clotting during the exchange • Citrate binds ionized calcium, hence hypocalcemia occurs • Symptoms include perioral tingling, numbness • Treated with IV or oral calcium supplementation

  11. Hypotension • Occurs in some patients • Usually mild • Important not to take certain blood pressure medications prior to plasma exchange (ACE inhibitors)

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