What to do immediately for a patient with sickle cell disease and acute chest syndrome who develops fever and chills during a blood transfusion?

Immediate Management: Stop the Transfusion

Stop the transfusion immediately when a patient with sickle cell disease and acute chest syndrome develops fever and chills during blood transfusion, as this represents a potential acute transfusion reaction that requires urgent evaluation before any further blood product administration. 1, 2

Rationale for Stopping Transfusion

• Fever and chills during transfusion are classic signs of an acute transfusion reaction, which can range from benign febrile non-hemolytic reactions to life-threatening acute hemolytic transfusion reactions. 3
• Patients with sickle cell disease have a 7-30% risk of alloimmunization, making them particularly susceptible to hemolytic transfusion reactions. 1
• Continuing the transfusion while a reaction is occurring can worsen the severity of hemolysis and potentially lead to multiorgan failure and death. 2, 4

Immediate Actions After Stopping Transfusion

Stop and Assess

• Immediately discontinue the transfusion and maintain IV access with normal saline. 5
• Notify the blood bank immediately and return the blood product with all tubing for investigation. 5
• Obtain blood cultures if fever is present, as this could also represent bacterial contamination of the blood product or concurrent sepsis. 1, 5

Laboratory Evaluation

• Send stat labs including: complete blood count, direct antiglobulin test (Coombs), indirect bilirubin, lactate dehydrogenase, haptoglobin, and repeat type and screen. 2, 5
• Compare current hemoglobin to pre-transfusion baseline to assess for acute hemolysis. 2
• Obtain urinalysis to check for hemoglobinuria, which indicates intravascular hemolysis. 2

Supportive Care While Evaluating

• Continue aggressive IV hydration to maintain renal perfusion and prevent acute kidney injury from hemoglobinuria. 1, 6
• Maintain oxygen therapy to keep SpO2 above baseline or 96% (whichever is higher). 1
• Monitor vital signs closely for progression to hypotension or shock. 5

When to Resume or Modify Transfusion Strategy

If Febrile Non-Hemolytic Reaction

• If workup shows no evidence of hemolysis and symptoms resolve with antipyretics, transfusion may be resumed slowly with premedication (acetaminophen ± diphenhydramine). 3
• Use leukoreduced blood products for future transfusions to prevent recurrent febrile reactions. 3

If Acute Hemolytic Reaction Suspected

• Do not resume transfusion until transfusion medicine specialist consultation is obtained. 7, 2
• For severe acute chest syndrome still requiring urgent transfusion despite the reaction, engage hematology and transfusion medicine for risk-benefit discussion about using the least incompatible blood available while maintaining absolute ABO compatibility. 2
• Consider immunosuppressive therapy (IVIg, steroids) for patients at high risk for hemolytic transfusion reactions who have acute need for transfusion. 7

If Severe ACS Requires Exchange Transfusion

• For severe acute chest syndrome with bilateral infiltrates or rapidly progressive disease, automated or manual red cell exchange should be performed rather than simple transfusion once the acute reaction is managed. 7, 1, 4
• Exchange transfusion is preferred because it rapidly reduces HbS levels to <30% without significantly increasing blood viscosity. 7, 4

Critical Pitfalls to Avoid

• Never continue transfusing through fever and chills without stopping to evaluate for hemolytic reaction, as this can be fatal. 2, 5
• Do not assume all fever during transfusion is benign in sickle cell patients, given their high alloimmunization rate. 1, 3
• Do not delay hematology consultation when transfusion reactions occur in the setting of severe acute chest syndrome, as these patients may need exchange transfusion rather than simple transfusion. 7, 1

Why Other Options Are Incorrect

• Hydroxyurea is a chronic disease-modifying therapy that has no role in acute management of transfusion reactions or acute chest syndrome. 1
• Hydration alone is insufficient when an acute transfusion reaction is occurring—the transfusion must be stopped first, though hydration should continue as supportive care. 1, 6