IVIG for hemolytic anemia may be an option if your symptoms don’t improve with conventional drug therapy or if you are unsuitable for surgery to remove the spleen.
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A Quick Overview of Autoimmune Hemolytic Anemia
Autoimmune hemolytic anemia (AIHA) happens when your immune system produces autoantibodies that attack and break down your red blood cells (RBCs), mistaking them for foreign substances. Consequently, your RBC levels drop (anemia).
AIHA is a rare blood disorder. Estimates suggest 15 to 60 new cases per 1,000,000 people in the US [1]. Though AIHA can affect people of any age and gender, the risk appears higher in older adults and women.
Two distinct types of AIHA exist:
- Warm antibody hemolytic anemia: The autoantibodies attack RBCs at normal body temperature.
- Cold antibody hemolytic anemia (cold agglutinin disease): The autoantibodies attack RBCs at temperatures lower than normal body temperature.
IVIG for Autoimmune Hemolytic Anemia: When It May Be an Option
IVIG is typically used with other medicines for severe cases of warm AIHA. When used appropriately, IVIG rapidly increases RBC levels and improves symptoms. High-dose IVIG is commonly used with a corticosteroid, such as prednisone or methylprednisolone.
If your condition doesn’t improve, you may need other treatments, such as:
- Splenectomy (spleen removal surgery)
- Rituximab
- Immunosuppressants to lower antibody production. Examples include azathioprine, cyclophosphamide, cyclosporin, and mycophenolate mofetil.
The use of IVIG, alone or with prednisone, is common in children with autoimmune hemolytic anemia.
Specific Situations When IVIG May Be an Option
Your hematologist may recommend IVIG for hemolytic anemia if:
- Hemolysis (RBC destruction) does not stop with corticosteroids.
- You are not a suitable candidate for splenectomy or immunosuppression. In such cases, IVIG may be used for both intermittent and maintenance therapy.
- You have an underlying autoimmune condition or primary immune deficiency.
How Does IVIG Work in Hemolytic Anemia?
The exact mechanism is unclear. However, IVIG infusion is thought to work by:
- Preventing autoantibodies from binding to their receptors
- Helping your body clear autoantibodies from the bloodstream
IVIG for Hemolytic Anemia: How Effective Is It?
Only a few studies have looked into IVIG efficacy for hemolytic anemia.
For instance, in a 1993 study, 73 participants reported a response rate of about 40%, and the rate was higher in children [2]. In this study, the response lasted about 3 weeks. Common IVIG side effects included:
- Headache
- Back pain
- Nausea
- Allergic reactions
This is an area of research, and more extensive trials are needed to ascertain the success rate of IVIG for hemolytic anemia.
IVIG may be more effective in post-infection AIHA and in children. Moreover, response rates may be higher if you have lower initial hemoglobin levels and hepatomegaly (enlarged liver).
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First Line of Treatment for Hemolytic Anemia
No evidence-based treatment for AIHA exists. However, corticosteroids are the first-line treatment with a success rate of 70% to 85% [3]. In treatment-resistant cases or relapses, your doctor may recommend splenectomy as the second-line treatment.
Can IVIG Cause Anemia?
IVIG can cause transient anemia in some patients. However, there have been reports of severe hemolytic anemia, especially in those with non-O blood group type. The risk of hemolysis is higher if you receive large doses of IVIG.
What Is a New Treatment for Hemolytic Anemia?
The US FDA approved mitapivat (brand name: Pyrukynd) to treat hemolytic anemia in adults who lack pyruvate kinase. Mitapivat was FDA-approved in February 2022 and is available as tablets to take by mouth.
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REFERENCES:
- Sylvie Bozzi, Siddhi Umarje, Kalyani Hawaldar, Jennifer Tyma, Jill Schinkel, Barnabie Agatep, Zulkarnain Pulungan, Natalia Petruski-Ivleva; Prevalence and Incidence of Primary Autoimmune Hemolytic Anemia and Cold Agglutinin Disease in the United States, 2016-2022: A Retrospective Study in Administrative Claims. Blood 2023; 142 (Supplement 1): 5202. doi: https://doi.org/10.1182/blood-2023-181618
- Flores, G et al. “Efficacy of intravenous immunoglobulin in the treatment of autoimmune hemolytic anemia: results in 73 patients.” American journal of hematology vol. 44,4 (1993): 237-42. doi:10.1002/ajh.2830440404
- Zanella, Alberto, and Wilma Barcellini. “Treatment of autoimmune hemolytic anemias.” Haematologica vol. 99,10 (2014): 1547-54. doi:10.3324/haematol.2014.114561
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Dr. Mark Alfonso, PharmD was born and raised in Pueblo, CO. He received his pharmacy degree from the University of Colorado School of Pharmacy at the Anschutz Medical Campus in 2010. He was board certified in medication therapy management in 2022. The most rewarding part of his job is helping to answer patient questions and concerns. His areas of expertise are community pharmacy and medication therapy management. In his free time, he enjoys reading and running.