What are the treatment options for Autoimmune Hemolytic Anemia (AIHA)?

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Last updated: February 14, 2025View editorial policy

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From the Guidelines

Treatment options for Autoimmune Hemolytic Anemia (AIHA) include glucocorticoids as the first-line treatment, followed by second-line options such as splenectomy, intravenous immunoglobulins, and immunosuppressive therapy.

First-line Treatment

  • Glucocorticoids are the initial treatment of choice for AIHA, as most patients respond to this therapy 1.

Second-line Treatment

  • For patients who do not respond to glucocorticoids, second-line treatment options include:
    • Splenectomy, which is a reasonable treatment choice 1.
    • Monoclonal antibodies, such as rituximab, which can be used in selected cases 1.
    • Immunosuppressive therapy with agents like cyclosporine A, azathioprine, or low-dose cyclophosphamide 1.
    • Intravenous immunoglobulins, which can be used in some cases 1.

Refractory Cases

  • In patients with refractory AIHA, treatment of the underlying CLL is recommended 1.
  • In some cases, thrombopoietin analogs may be effective in treating ITP-related CLL that does not respond to corticosteroids 1.

From the Research

Treatment Options for Autoimmune Hemolytic Anemia (AIHA)

The treatment options for AIHA can be categorized based on the type of AIHA, which includes warm and cold types.

  • Warm AIHA:
    • First-line treatment: Glucocorticoids, such as prednisolone, are the primary treatment for warm AIHA, with most patients responding to this therapy 2, 3, 4.
    • Second-line treatment: Rituximab is a useful alternative for patients who are refractory to steroids or require high maintenance doses 2, 3, 4.
    • Other treatment options: Splenectomy can provide long-term remission without medication, but it is associated with various complications 2, 5.
  • Cold AIHA:
    • First-line treatment: Rituximab, with or without bendamustine, is generally used as the first-line regimen for cold AIHA, as glucocorticoids and splenectomy are often ineffective 2, 5, 4.
    • Other treatment options: Sutimlimab, a complement inhibitor, has been approved for the treatment of cold agglutinin disease (CAD) 5.

Emerging Therapies

Several emerging therapies have shown promise in the treatment of AIHA, including:

  • B-cell-directed therapies: These therapies target B-cells, which play a crucial role in the production of autoantibodies 6, 5.
  • Plasma cell-directed therapies: These therapies target plasma cells, which are responsible for the production of autoantibodies 6, 5.
  • Complement inhibitors: These therapies inhibit the complement system, which contributes to the destruction of red blood cells in AIHA 6, 5.
  • Phagocytosis inhibition: These therapies inhibit the phagocytosis of red blood cells, which can help reduce the destruction of red blood cells in AIHA 6, 5. These emerging therapies offer new perspectives for AIHA treatment and have shown great potential for clinical applications 6, 5.

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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