Blood Transfusion for Pediatric Splenic Sequestration
For acute splenic sequestration crisis in pediatric patients with sickle cell disease, transfuse 3 to 5 mL/kg of packed red blood cells in careful aliquots, checking post-transfusion hemoglobin before administering the next dose, with extreme caution to avoid exceeding 10 g/dL due to risk of acute overtransfusion when sequestered red cells are released from the spleen. 1
Transfusion Protocol
Initial Volume and Administration
- Administer 3 to 5 mL/kg of packed red blood cells as the recommended starting volume 1
- Give transfusions in careful aliquots rather than as a single bolus 1
- Check hemoglobin level after each aliquot before ordering the next dose 1
- This conservative approach is critical because sequestered red cells may be acutely released from the spleen as the crisis resolves 1
Critical Safety Threshold
- Never transfuse to a hemoglobin greater than 10 g/dL 1
- Overtransfusion beyond this threshold can lead to dangerous hyperviscosity when the spleen releases its sequestered blood volume back into circulation 1
- This represents a unique hazard specific to splenic sequestration that differs from other transfusion scenarios 1
Pre-Transfusion Fluid Resuscitation
Crystalloid Administration
- Before initiating blood transfusion, administer up to three boluses of 20 mL/kg of crystalloid replacement for initial resuscitation 2
- Blood replacement should only be considered after crystalloid resuscitation 2
- Aggressive hydration is crucial throughout management, with careful monitoring to prevent overhydration 1
Transfusion Trigger
- While general pediatric transfusion triggers suggest considering transfusion when hemoglobin is less than 7 g/dL, splenic sequestration represents an acute emergency requiring immediate intervention 2
- The defining feature is a decrease in hemoglobin of more than 2 g/dL below the patient's baseline value with rapidly enlarging spleen 1
Monitoring During Transfusion
Essential Parameters
- Continuous monitoring of vital signs, oxygen saturation, and hemoglobin levels is essential 1
- Document baseline oxygen saturation and administer oxygen therapy to maintain SpO2 above baseline or 96%, whichever is higher 1
- Regular assessment by hematology specialists is recommended 1
Signs of Effective Resuscitation
- Reduction in heart rate 2
- Improved mental status 2
- Return of peripheral pulses and normal skin color 2
- Increase in blood pressure 2
- Adequate urinary output 2
- Increased extremity warmth 2
Common Pitfalls to Avoid
Overtransfusion Risk
The most critical error is transfusing too aggressively to "normal" hemoglobin levels (>10 g/dL), which fails to account for the large volume of red cells trapped in the spleen that will be released as the crisis resolves 1. This can result in acute hyperviscosity and potentially precipitate vaso-occlusive complications.
Delayed Recognition
Delayed recognition of splenic sequestration can lead to rapid progression to hypovolemic shock and death 1, 3. Parents and caregivers should be taught to check the child's spleen daily and urgently seek medical attention for enlargement 1.
Massive Transfusion Protocol
If massive transfusion becomes necessary, apply a 1:1:1 ratio of blood products in children 2. However, the goal in splenic sequestration is to avoid reaching this threshold through careful, measured transfusion.
Additional Supportive Care
Pain Management
- For severe pain, administer parenteral opioids such as morphine by scheduled around-the-clock dosing or patient-controlled analgesia 1
Temperature Management
- Maintain normothermia, as hypothermia can lead to shivering and peripheral stasis, which increases sickling 1