What is the immediate management of a hemolytic (Hemolysis) transfusion reaction?

Immediate Management of Hemolytic Transfusion Reaction

Stop the transfusion immediately at the first sign of a reaction and initiate supportive care while simultaneously notifying the blood bank and verifying patient identification. 1

Immediate Actions (First 5 Minutes)

• Discontinue the blood product immediately when any signs appear: tachycardia, hypotension, fever, rash, breathlessness, or hemoglobinuria 1
• Maintain IV access with normal saline to support blood pressure and renal perfusion 1
• Contact the transfusion laboratory immediately and return the blood product with all associated documentation for investigation 1
• Double-check all documentation to identify potential administration errors, including verifying the four core identifiers (first name, last name, date of birth, patient ID number) 1
• Visually inspect the blood product for discoloration, clots, or leakage 1

Initial Pharmacologic Management

For Acute Hemolytic Reactions:

• Antihistamines for allergic symptoms (urticaria, pruritus) 1
• Steroids (methylprednisolone or prednisone) for severe reactions 1
• Intramuscular or intravenous epinephrine if the reaction is life-threatening or anaphylactic 1

For Delayed Hemolytic Transfusion Reaction with Hyperhemolysis:

First-line immunosuppressive therapy should be initiated promptly in patients with life-threatening hemolysis: 2, 3

• IVIg: 0.4-1 g/kg/day for 3-5 days (up to total dose of 2 g/kg) 2, 1
• High-dose steroids: Methylprednisolone or prednisone 1-4 mg/kg/day 2, 1
• Second-line agent: Eculizumab for refractory cases 2, 4
• Rituximab: 375 mg/m² repeated after 2 weeks, primarily indicated for prevention of additional alloantibody formation in patients requiring future transfusions 2, 1

Critical Management Principle: Transfusion Avoidance

Avoid further transfusion unless the patient is experiencing life-threatening anemia with ongoing hemolysis, as additional transfusions may worsen hemolysis and potentially induce multiorgan failure and death. 2

• If transfusion is absolutely warranted for life-threatening anemia, use extended matched red cells (C/c, E/e, K, Jka/Jkb, Fya/Fyb, S/s) 2, 1
• This is particularly critical in hyperhemolysis, where hemoglobin drops below pretransfusion levels, indicating clearance of both transfused and patient's own red cells 2

Supportive Care

• Erythropoietin with or without IV iron should be initiated in all patients 2
• Folic acid supplementation 1 mg daily 3
• Aggressive hydration to maintain renal perfusion and prevent acute kidney injury 5
• Monitor for complications: bleeding diathesis, renal failure, and shock from complement activation products (C3a, C5a) 5

Diagnostic Workup During Acute Management

• Send blood samples for repeat type and crossmatch, direct antiglobulin test (DAT), hemolysis markers (LDH, haptoglobin, bilirubin, free hemoglobin) 3
• Hemoglobin electrophoresis can help distinguish severe DHTR (complete absence of HbA) from hyperhemolysis syndrome 6
• Monitor reticulocyte count: relative reticulocytopenia suggests hyperhemolysis 2

Common Pitfalls to Avoid

• Do not transfuse additional blood in hyperhemolysis without immunosuppressive therapy, as this can exacerbate hemolysis and cause death 2
• Do not delay immunosuppressive therapy in severe cases with life-threatening hemolysis, as this increases morbidity and mortality 3
• Most hemolytic reactions result from ABO-incompatible blood due to patient or product misidentification, emphasizing the critical importance of verification procedures 7
• Recognize that hyperhemolysis can occur with no identifiable antibody and a negative direct antiglobulin test, making clinical recognition essential 2

Special Considerations for Sickle Cell Disease

For patients with sickle cell disease at high risk for acute hemolytic transfusion reaction (history of multiple or life-threatening DHTRs, or when compatible blood cannot be found), consider prophylactic immunosuppression with IVIg, steroids, and/or rituximab before transfusion through shared decision-making between hematologist and transfusion medicine specialist. 2, 1