Plasmapheresis Indications and Procedures

Mechanism and Procedure

Plasmapheresis is an extracorporeal therapy that separates and removes plasma containing pathogenic elements from the patient's blood while returning the cellular components, as recommended by the National Comprehensive Cancer Network 1, 2
The removed plasma is typically replaced with albumin, fresh-frozen plasma, or crystalloid solutions before being returned to the patient, according to Circulation 3

The American Academy of Neurology recommends plasmapheresis as first-line therapy for severe Guillain-Barré syndrome, with a strength of evidence rated as high 4
Plasmapheresis is also recommended for myasthenia gravis with significant symptoms, with a strength of evidence rated as moderate 4
Treatment for severe immune-mediated neuropathies, especially those with rapid progression, is recommended, with a strength of evidence rated as low 4

The American Society of Nephrology recommends plasmapheresis for patients with ANCA-associated vasculitis requiring dialysis or with rapidly increasing serum creatinine, with a strength of evidence rated as high 5, 6
Plasmapheresis is used in anti-glomerular basement membrane (anti-GBM) disease, with a strength of evidence rated as moderate 5
Beneficial in severe cryoglobulinemia with acute kidney involvement, with a strength of evidence rated as low 4

The American Society of Hematology recommends plasmapheresis as first-line therapy for symptomatic hyperviscosity due to paraproteinemia, with a strength of evidence rated as high 4
Preventive treatment before rituximab therapy in patients with IgM ≥ 4 g/dL to avoid symptomatic IgM flares, with a strength of evidence rated as moderate 4

The European Respiratory Society suggests plasmapheresis for patients with confirmed autoimmune pulmonary alveolar proteinosis (aPAP) who remain significantly symptomatic despite receiving exogenous GM-CSF and rituximab, with a strength of evidence rated as low 7
Plasmapheresis is recommended for patients with diffuse pulmonary hemorrhage in ANCA vasculitis, with a strength of evidence rated as high 5, 6

The British Society for Dermatology recommends plasmapheresis in refractory cases of pemphigus vulgaris when combined with corticosteroids and immunosuppressants, with a strength of evidence rated as moderate 8, 9, 10, 11
Plasmapheresis is a treatment option for antibody-mediated rejection in cardiac transplantation, with a strength of evidence rated as low 3
Management of severe immunotherapy-related toxicities, particularly neurologic adverse events, is recommended, with a strength of evidence rated as high 1, 2

Plasmapheresis should generally be combined with immunosuppressive medications to prevent rebound antibody production, as recommended by the British Society for Dermatology, with a strength of evidence rated as moderate 8, 9, 10
Not recommended as routine treatment for newly diagnosed pemphigus but may be considered in refractory cases, with a strength of evidence rated as low 8, 9, 10, 11
In pulmonary alveolar proteinosis, higher intensity plasmapheresis regimens may be needed to successfully suppress GM-CSF autoantibodies, with a strength of evidence rated as low 7

Mortality associated with plasmapheresis is estimated at 0.05% based on systematic reviews, as reported in the European Respiratory Journal, with a strength of evidence rated as high 7
Removal of important plasma proteins including clotting factors can result in coagulation defects, with a strength of evidence rated as moderate 8, 9, 10
Plasmapheresis can be associated with hemodynamic shifts, increased infection risk, and thrombosis, with a strength of evidence rated as high 5, 6
When combined with immunosuppressants, there is increased risk of opportunistic infections requiring prophylactic measures, with a strength of evidence rated as high 1, 2

Timing is critical when used with other therapies - for example, rituximab should be administered after plasmapheresis since the procedure removes the drug, as recommended by the American Journal of Kidney Diseases, with a strength of evidence rated as high 5, 6

The American Society of Critical Care recommends using fibrinogen concentrate (3-4 g or 50 mg/kg) or cryoprecipitate (15-20 units in a 70 kg adult) for fibrinogen replacement during plasma exchange, rather than fresh frozen plasma, in patients on anticoagulation, with active bleeding, or undergoing invasive procedures within 48 hours 12
Fresh frozen plasma is impractical for raising fibrinogen above 1.5 g/L due to volume requirements, with a strength of evidence based on expert opinion and clinical experience 13
Fibrinogen concentrate provides more rapid and predictable correction than cryoprecipitate, with moderate-quality evidence from prospective studies 14, 12