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

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 into several approaches, including:

• First-line treatments:
  • Corticosteroids, such as prednisolone, are commonly used as the first-line treatment for warm-antibody AIHA 2, 3, 4, 5
  • Rituximab, a monoclonal antibody, is often used in combination with corticosteroids or as a single agent for cold agglutinin disease and warm-antibody AIHA 2, 6, 3, 4, 5
• Second-line treatments:
  • Splenectomy, the surgical removal of the spleen, is considered a second-line treatment for warm-antibody AIHA, particularly for patients who are refractory to corticosteroids or rituximab 6, 3, 5
  • Immunosuppressants, such as cyclophosphamide or azathioprine, may be used in some cases 6, 5
• Emerging therapies:
  • B-cell-directed therapies, such as rituximab and other monoclonal antibodies, aim to reduce autoantibody production 2, 6, 4, 5
  • Plasma cell-directed therapies target the cells responsible for producing autoantibodies 2, 5
  • Complement inhibitors, such as sutimlimab, have been approved for the treatment of cold agglutinin disease 4, 5
  • Phagocytosis inhibition aims to reduce the destruction of red blood cells by the immune system 2, 5

Special Considerations

The choice of treatment for AIHA depends on various factors, including the type of AIHA, degree of hemolysis, underlying diseases, and patient characteristics 6, 4, 5. Supportive treatments, such as vitamin supplementation, recombinant erythropoietin, and thrombosis prophylaxis, may also be necessary to manage the condition 6, 5.