What is the effectiveness of plasma exchange (PLEX) in Autoimmune Hemolytic Anemia (AIHA)?

From the Guidelines

Plasmapheresis (PLEX) is not a first-line treatment for autoimmune hemolytic anemia (AIHA) and should be reserved for severe, refractory cases that have failed to respond to other therapies, such as glucocorticoids and immunosuppressive agents 1. The effectiveness of PLEX in AIHA is limited, and it is generally considered a second or third-line therapy.

Key Points to Consider

• PLEX should be used in conjunction with more definitive immunosuppressive therapy, rather than as a standalone treatment.
• The benefit of PLEX is transient, as it does not address the underlying antibody production, and autoantibodies typically return within days to weeks after treatment 1.
• PLEX may be most beneficial in warm AIHA cases with fulminant hemolysis causing life-threatening anemia or in cold agglutinin disease with severe symptoms.
• Patients should be monitored for complications, including bleeding, hypocalcemia, and infection risk.

Treatment Approach

• First-line treatments for AIHA include glucocorticoids, such as prednisone 1-2 mg/kg/day, and immunosuppressive agents, such as rituximab 1.
• Second-line treatment options for AIHA include splenectomy, intravenous immunoglobulins, and/or immunosuppressive therapy with agents, such as cyclosporine A, azathioprine, or low-dose cyclophosphamide 1.
• PLEX may be considered for severe, refractory cases that have failed to respond to other therapies, with a typical regimen involving 5-7 exchanges over 7-14 days, with each session processing 1-1.5 plasma volumes.

From the Research

Effectiveness of Plasma Exchange in AIHA

The effectiveness of plasma exchange (PE) in autoimmune hemolytic anemia (AIHA) is supported by several studies:

• PE may be preferred as a third-line option after immunosuppressive treatments and play a role as a bridge to splenectomy in severe and refractory anemia, especially in cases of cardiovascular imbalance in fulminant hemolysis 2.
• Therapeutic plasma exchanges (TPEs) combined with low doses of intravenous immunoglobulins (IVIGs) may be considered as a therapeutic option in patients with severe warm AIHA without response to corticosteroid and IVIG treatment 3.
• In cases where steroids fail, there is a lack of rapidly effective therapeutic options, and PE may be considered as a last option treatment for severe or refractory cases 4.

Clinical Applications of Plasma Exchange

The clinical applications of PE in AIHA include:

• Removal of immune complexes, protein-bound toxins, autoantibodies, and high molecular weight solutes and protein-bound solutes 2.
• Rapid reduction of hemolysis and improvement of hemoglobin levels in patients with severe AIHA 3.
• Bridge to splenectomy in patients with severe and refractory AIHA 2.

Limitations and Future Directions

The current evidence on the effectiveness of PE in AIHA is limited by:

• The lack of validated predictors for severe clinical course and poor outcomes in AIHA 5.
• The need for prospective clinical trials to establish evidence-based therapeutic approaches for AIHA 6.
• The rarity and heterogeneity of AIHA, which complicate the recognition and management of severe cases 5.