When is plasma apheresis recommended?

Plasmapheresis: Clinical Indications and Recommendations

Primary Indications by Disease Category

Plasmapheresis is recommended as first-line therapy for thrombotic thrombocytopenic purpura (TTP), symptomatic hyperviscosity syndrome, and severe Guillain-Barré syndrome, while serving as salvage therapy for refractory autoimmune conditions after failure of standard treatments. 1, 2

Category I: First-Line Therapy (Strongest Evidence)

• Thrombotic Thrombocytopenic Purpura (TTP): Daily plasma exchange for 14 days or until antibodies become undetectable 1

• Symptomatic Hyperviscosity Syndrome: Replace approximately two-thirds of plasma volume (typically 3-4 liters in adults) for paraproteinemia-related hyperviscosity 1, 2

• Severe Guillain-Barré Syndrome: Initiate for patients with self-care limitation, walking impairment, dysphagia, facial weakness, or respiratory muscle weakness 1, 2

• Cardiac Transplant Antibody-Mediated Rejection: Combined with immunosuppression, perform 4-5 sessions over 10-14 days, with 1-year survival of 75% and 5-year survival of 51% in hemodynamically unstable patients 1

Category II: Adjunctive Therapy for Severe/Refractory Disease

Renal Conditions

• ANCA-Associated Vasculitis with Severe Renal Disease: Add plasmapheresis for patients requiring dialysis or with rapidly increasing serum creatinine, using 60 mL/kg volume replacement for 7-10 treatments total 1, 2

• Diffuse Pulmonary Hemorrhage in ANCA Vasculitis: Initiate daily until bleeding stops, then every other day for total of 7-10 treatments 1, 2

• Critical Practice Point: Discontinue cyclophosphamide after 3 months in patients who remain dialysis-dependent without extrarenal manifestations 1

Neurological Conditions

• Myasthenia Gravis: For significant symptoms or immune checkpoint inhibitor-related cases, permanently discontinue checkpoint inhibitor and initiate 5-day plasmapheresis course combined with 1-2 mg/kg methylprednisolone daily 1, 2

• Immune Checkpoint Inhibitor-Related Encephalitis: For severe or progressing symptoms with oligoclonal bands, administer pulse methylprednisolone 1 g IV daily for 3-5 days plus IVIG 2 g/kg over 5 days 1

Autoimmune Pulmonary Conditions

• Autoimmune Pulmonary Alveolar Proteinosis (aPAP): Reserved for patients who remain significantly symptomatic requiring high-flow supplemental oxygen (≥4L/min) OR requiring two or more whole lung lavages over one year, DESPITE receiving exogenous GM-CSF and rituximab or having previously failed these treatments 3, 2

• Evidence Quality Note: This recommendation is based on only nine case reports with very low certainty evidence; three of nine cases showed no significant clinical benefits, and one showed only short-lived improvement with relapse at five months 3

• Higher intensity regimens may be needed to successfully suppress GM-CSF autoantibodies in aPAP 2

Dermatologic Conditions

• Refractory Pemphigus Vulgaris: When combined with corticosteroids and immunosuppressants, not recommended as routine treatment for newly diagnosed cases 2

Technical Specifications

Volume Calculations

• Standard plasma volume: Body weight (kg) × 0.045 L (or 45 mL/kg) 1

• High-volume plasmapheresis: 60 mL/kg for severe renal disease (approximately 1.3 times standard volume) 1

• Standard exchange: 1-2 plasma volumes per session 1

Treatment Protocols

• Typical courses: 2-6 sessions for neurologic conditions; daily exchanges for 5 days in cardiac AMR 1

• Replacement fluids: 5% albumin solution is most common; fresh-frozen plasma or crystalloid alternatives 1, 2

Critical Safety Considerations

Mortality and Complications

• Overall mortality: 0.05% based on systematic reviews of >15,500 patients 1, 2

• No increased risk of infection, blood pressure instability, cardiac arrhythmias, or pulmonary embolism in Cochrane meta-analysis of 556 Guillain-Barré patients 1

• Coagulation defects can occur from removal of clotting factors 2

• Hemodynamic shifts, increased infection risk, and thrombosis are potential complications 2

Absolute Requirements

• Never use as monotherapy for antibody-mediated rejection—always combine with immunosuppression 1, 2

• Timing with rituximab is critical: Administer rituximab AFTER plasmapheresis, as the procedure removes the drug from circulation 1, 2

• Always combine with immunosuppressive medications to prevent rebound antibody production 2

Monitoring Requirements

• Monitor for hemodynamic changes, coagulation abnormalities, and electrolyte imbalances 1

• Track disease-specific markers: GM-CSF antibody titers in aPAP, ADAMTS13 in TTP 1

• Monitor IgG trough levels, blood counts, and serum chemistry after immunoglobulin apheresis 4

• Close monitoring of HCV or HBV infection after apheresis, especially when combined with immunosuppressants 4

Common Pitfalls to Avoid

• Do not use fixed volumes without weight-based calculation—plasma volume varies significantly with body size 1

• Do not use as monotherapy—always combine with definitive treatment (immunosuppression, chemotherapy) as the procedure only provides temporary removal of pathogenic substances 1

• Avoid in dialysis-dependent vasculitis patients without extrarenal manifestations after 3 months of treatment 1

• Procedural risks are higher in children than adults, though complications can be anticipated and avoided 1