What is the recommended plasma exchange regime for a patient?

Plasma Exchange Regimen

For ANCA-associated vasculitis with severe kidney disease (creatinine >500 μmol/L or dialysis-dependent), perform 7 plasma exchange sessions over 14 days (every other day), exchanging 1.0-1.5 plasma volumes per session, using 5% albumin as replacement fluid. 1

Standard Protocol Parameters

Session Frequency and Duration

• 7 exchanges over 14 days (alternate day schedule) is the evidence-based regimen for ANCA-associated vasculitis with severe renal involvement 1
• For anti-GBM overlap syndrome with ANCA vasculitis, perform daily exchanges for 14 days or until anti-GBM antibodies are undetectable 1
• For neuromyelitis optica spectrum disorder, 5-7 sessions on alternate days is the standard approach 2, 3

Volume Exchange

• Exchange 1.0-1.5 plasma volumes per session, calculated at 40 mL/kg body weight 4, 3
• Most institutions use 1.0-1.25 plasma volumes as the target 3
• Each session typically processes the calculated volume over 2-3 hours 5

Replacement Fluid

• 5% albumin is the preferred replacement fluid for most indications to minimize transfusion reactions and hemodynamic fluctuations 5, 4, 3
• Fresh frozen plasma should be reserved exclusively for thrombotic microangiopathies or patients with active bleeding risk, as it is highly allergenic and expensive 4

Vascular Access Requirements

• Central venous catheter is typically required to achieve adequate blood flow rates for efficient plasma separation 1, 6
• Peripheral venous access may be attempted in select patients with excellent peripheral veins, but central access is more reliable 6
• Monitor for catheter-related complications including infection, thrombosis, and bleeding 1, 5

Timing Relative to Immunosuppressive Therapy

Cyclophosphamide

• Administer intravenous cyclophosphamide after the plasma exchange session to avoid drug removal 1

Rituximab

• Hold plasma exchange for 48-72 hours after rituximab infusion to allow adequate drug exposure 1, 5
• For severe optic neuritis, initiate rituximab immediately following completion of the plasma exchange series 2

Anticoagulation During Procedure

• Citrate anticoagulation is preferred over heparin-based regimens 4
• Monitor for citrate-induced hypocalcemia, which can cause muscle cramps or more severe symptoms 5

Monitoring and Complications

Hemodynamic Monitoring

• Maintain continuous blood pressure and heart rate monitoring throughout each session 5
• Ensure adequate volume status before initiating plasma exchange to prevent hypotension 5
• Hypotension occurs in approximately 3.6% of procedures 5, 7

Laboratory Monitoring

• Monitor coagulation parameters, as plasma exchange removes clotting factors 1, 5
• Check electrolytes regularly, particularly calcium levels 1, 5
• Track the target antibody or protein levels when applicable (e.g., anti-GBM antibodies) 1

Common Complications

• Muscle cramps (6.4%), allergic reactions (4.5%), severe hypotension (3.6%), fever (1.8%) 7
• Line-related bacteremia from central venous access 1, 5
• Coagulation disorders from removal of clotting factors 1, 5

Disease-Specific Modifications

ANCA-Associated Vasculitis

• Use the 7 exchanges over 14 days regimen for patients with serum creatinine >5.7 mg/dL (>500 μmol/L) requiring dialysis or rapidly increasing creatinine 1
• Also indicated for diffuse alveolar hemorrhage with hypoxemia 1
• Combine with reduced-dose glucocorticoid regimen (not standard-dose) to minimize infection risk 1

Hyperviscosity Syndrome (Waldenström Macroglobulinemia)

• Perform plasma exchange emergently for symptomatic hyperviscosity before initiating systemic chemotherapy 1, 8
• A single 3-4 liter exchange lowers IgM by 60-75% and viscosity by at least 50% 1
• Multiple exchanges may be necessary; continue until symptoms resolve 1, 8

Multiple Myeloma with Acute Renal Failure

• Plasma exchange combined with corticosteroids is indicated for rapidly progressive renal failure secondary to light chain deposition 1
• Most effective when initiated early in patients with rapidly declining renal function 1

Response Assessment

Expected Timeline

• For ANCA vasculitis, assess renal function recovery within the first 3 months 1
• For neuromyelitis optica, maximal symptom improvement typically appears by the fourth or fifth treatment 3
• Most patients (52%) achieve mild to moderate clinical improvement and do not require additional courses 3

Criteria for Additional Sessions

• If inadequate response after initial 7-session course, consider extending treatment 3
• For anti-GBM overlap syndrome, continue daily exchanges until antibodies are undetectable regardless of session number 1

Critical Pitfalls to Avoid

• Do not delay plasma exchange beyond 4-6 weeks of symptom onset in acute inflammatory conditions, as efficacy diminishes with time 2
• Do not use plasma exchange for low-risk ANCA vasculitis (creatinine <500 μmol/L without dialysis requirement), as infection risk outweighs benefit 1
• Do not substitute fresh frozen plasma for albumin except in thrombotic microangiopathies or active bleeding, as it dramatically increases allergic reactions 4
• Do not administer rituximab immediately before plasma exchange, as the drug will be removed; maintain 48-72 hour separation 1, 5