Hemolytic anemia medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Shyam Patel [2]

Overview

Treatment depends on the cause and nature of the breakdown.

Medical Therapy

• Corticosteroids: The cornerstone of therapy for warm autoimmune hemolytic anemia is steroids. Steroids are the first-line therapy for induction of a response. The initial regimen of steroids is usually prednisone 1-1.5 mg/kg PO daily, to be continues for 1-3 weeks until the hemoglobin increases to 10 g/dl. Steroids are then rapidly tapered. Steroids are effective in approximately 70-85% of patients (PMC4181250). The benefits of steroids are that they can induce a remission within a relatively quick timeframe, such as days to weeks.
  • Adverse effects: The use of steroids is meant for short-term, as there can be many adverse effects from long-term systemic steroid use. These adverse effects include immunosuppression with risk for infections, bone loss, muscle loss, weight gain, cataracts, and glaucoma.
• Splenectomy: This is a second-line therapy option and is typically done only if there is non-response to steroids. The response rate to splenectomy is usually 60-70%.
  • Adverse effects: Given that this is a surgery, the risks and benefits must be carefully weighed, as surgical interventions typically have higher risks than medication interventions. Splenectomy carries an inherent surgical risk in addition to the risk for infections from encapsulated organisms, such as Neisseria meningitides, Streptococcus pneumoniae, and Hemophilus influenzae. Patients who plan to undergo splenectomy should be vaccinated prior to the surgery. In the current era, the surgical risk of splenectomy is likely lower than in the prior decades, as laparoscopic and minimally invasive approaches are used.
• Rituximab: This is a monoclonal antibody that targets the CD20 antigen on B cells. The basis for the use of rituximab in hemolytic anemia is that B cells are producing antibodies that bind to red blood cell membranes and cause hemolysis. Rituximab binds to B cells and eliminates the B cells by antibody-dependent cell-mediated cytotoxicity. This medication is considered a biologic agent, as it is derived from mice.
  • Adverse effects: Risks of rituximab include infusion reaction (during the initial administration of the agent), hepatitis B reactive, and opportunistic infections, such as progressive multifocal leukoencephalopathy. The benefits of rituximab are that there are no steroid-specific adverse effects, such as bone loss, cataracts, glaucoma, stress ulcers, muscle loss, and weight gain.
• Azathioprine: This is an alternative immunosuppressive agent. It is a purine antimetabolite that is converted to 6-mercaptopurine. It can inhibit the synthesis of DNA and RNA in lymphocytes, and thus it functions by inhibiting immune cell proliferation. Azathioprine is used in the third-line setting for hemolytic anemia.
  • Adverse effects: Risks of azathioprine include leukopenia and infections. Importantly, the other adverse effects of steroids are spared.
• Transfusion support: Packed red blood cell transfusions can be administered to help treat hemolytic anemia in the short-term. It is important to note that transfusions are a temporizing measure until a more long-term or disease-modifying medication can be given.
  • Adverse effects: Transfusions are associated with the risks of volume overload, transfusion reactions, and iron overload. Volume overload includes conditions line transfusion-related acute lung injury (TRALI) or transfusion-related circulatory overload. Transfusion reactions include conditions like hemolytic transfusion reactions (from ABO blood group incompatibility) and febrile non-hemolytic transfusion reactions (from non-leukoreduced blood). Iron overload can result in conditions like cirrhosis (if iron deposition occurs in the liver), diabetes (if iron deposition occurs in the pancreas), hyperpigmentation (if iron deposition occurs in the skin), infertility (if iron deposition occurs in the gonads), and heart failure (if iron deposition occurs in the heart).