What is the treatment for hemolysis (breakdown of red blood cells)?

From the Guidelines

The treatment for hemolysis should prioritize addressing the underlying cause and severity, with a focus on supportive care, immunosuppressive therapy, and transfusion support as needed, according to the most recent guidelines from the American Society of Hematology in 2020 1.

Key Considerations

• For mild cases, supportive care including rest, hydration, and monitoring may be sufficient.
• Severe hemolysis often requires blood transfusions to replace lost red blood cells and maintain adequate oxygen delivery to tissues.
• Corticosteroids like prednisone (typically 1-2 mg/kg/day) are commonly prescribed to suppress the immune response in autoimmune hemolytic anemia.
• Immunosuppressants such as rituximab, cyclophosphamide, or azathioprine may be added for refractory cases.

Treatment Approach

• For patients experiencing life-threatening hemolysis, first-line immunosuppressive agents include IVIg and high-dose steroids, with eculizumab as a second-line agent 1.
• Rituximab is primarily indicated for potential prevention of additional alloantibody formation in patients who may require further transfusion.
• Avoidance of further transfusion is recommended unless patients are experiencing life-threatening anemia with ongoing hemolysis.
• If transfusion is warranted, extended matched red cells should be considered.

Supportive Care

• Supportive care including erythropoietin with or without IV iron should be initiated in all patients.
• Serial monitoring of the hemoglobin, hematocrit, quantification of HbA and HbS fractions, reticulocyte count, bilirubin, LDH, and urinalysis (for hemoglobinuria) is also advised.
• Folic acid supplementation (1 mg daily) is often recommended to support increased red blood cell production.

From the Research

Treatment Options for Hemolysis

• Corticosteroids are the first-line therapy for warm autoimmune hemolytic anemia (AIHA) 2
• For refractory or relapsed cases, options include:
  • Splenectomy, effective in approximately 70% of cases 2
  • Rituximab, effective in approximately 70-80% of cases 3, 2
  • Immununosuppressive drugs such as azathioprine, cyclophosphamide, cyclosporin, and mycophenolate mofetil 2
• Additional therapies that may be used include:
  • Intravenous immunoglobulins (IVIGs) 4, 2
  • Danazol 2
  • Plasma-exchange, which may be combined with low doses of IVIG 4
• For severe or refractory cases, last option treatments may include:
  • High-dose cyclophosphamide 2
  • Alemtuzumab 2
• Novel therapies, such as B-cell-directed therapy, phagocytosis inhibition, plasma cell-directed therapy, and complement inhibition, are being developed and may offer promising alternatives for the treatment of AIHA 5

Specific Treatment Approaches

• Therapeutic plasma exchanges (TPEs) combined with low doses of IVIG may be effective in patients with severe warm AIHA who do not respond to corticosteroid and IVIG treatment 4
• Rituximab may be effective in patients with mixed autoimmune hemolytic anemia, including those who do not respond to steroid therapy 6
• A single cycle of rituximab may lead to prompt and durable complete remission in some patients with severe hemolysis associated with mixed autoimmune hemolytic anemia 6