Should a 2-year-old with a hemoglobinopathy and severe anemia be transfused?

From the Guidelines

A 2-year-old child with a hemoglobinopathy and a hemoglobin level below 7 g/dL should generally receive a blood transfusion to improve morbidity, mortality, and quality of life. The decision to transfuse is based on the principle of improving oxygen delivery to tissues and reducing the risk of complications associated with severe anemia. According to the most recent guidelines, restrictive thresholds for red blood cell transfusion are appropriate for almost all children over 3 months of age, with a target hemoglobin level of 70 g/L or higher 1.

Key Considerations for Transfusion

• The standard approach is to transfuse packed red blood cells at 10-15 mL/kg, typically given over 2-4 hours with careful monitoring of vital signs.
• Prior to transfusion, blood typing and crossmatching should be performed, and the child should be assessed for symptoms of anemia such as fatigue, pallor, tachycardia, or poor feeding.
• For children with hemoglobinopathies like sickle cell disease or thalassemia, transfusion not only treats symptomatic anemia but also suppresses production of abnormal hemoglobin and reduces complications, as supported by studies on hydroxyurea use in sickle cell disease 1.
• The target post-transfusion hemoglobin is typically 9-10 g/dL, though this may vary based on the specific hemoglobinopathy and clinical situation.

Monitoring and Long-term Considerations

• Regular monitoring during and after transfusion is essential to detect potential adverse reactions such as fever, allergic reactions, or fluid overload.
• Long-term transfusion therapy requires additional considerations including iron chelation to prevent iron overload, especially after 12 to 20 transfusions 1.
• The volume of blood needed should be modified depending on the size of the patient, with a formula available to calculate the volume of red blood cells to transfuse 1.

From the Research

Transfusion Guidelines for a 2-Year-Old with Hemoglobinopathy

• The decision to transfuse a 2-year-old with a hemoglobinopathy and a hemoglobin (Hb) level under 7 g/dL should be based on clinical criteria and surrogate parameters such as Hb concentration 2.
• For patients with unimpaired cardiopulmonary and vascular function, transfusion is generally indicated at Hb values of 6 g/dL or less 2.
• Randomized controlled trials have shown that a restrictive transfusion strategy (trigger: Hb 7-8 g/dL) in certain patient groups is as effective as a more liberal strategy (trigger: Hb about 10 g/dL) 2.
• The clinical manifestations of hemoglobinopathies can range from mild hypochromic anemia to severe, lifelong, transfusion-dependent anemia with multiorgan involvement 3.
• Blood transfusions should be given only when strictly indicated, and the goal is to achieve a sufficient increase in hemoglobin levels 2, 4.

Considerations for Transfusion in Pediatric Patients

• Every child should have a baseline hematology evaluation with Hb and hematocrit levels starting at age 12 months, or younger if clinically indicated 5.
• The interpretation of complete blood count (CBC) results is a skill that requires practice, and clinicians can learn to identify possible diagnoses before consulting a specialist 5.
• The hemostatic effect of packed red cell transfusion in patients with anemia has been studied, and results show that red cell transfusion might decrease the activated partial thromboplastin time (APTT) and bleeding time (BT) in some anemic patients 6.

Hemoglobin Increase After Transfusion

• The aspired increase in Hb can be achieved with red blood cell concentrates of any storage duration 4.
• Confounding variables resulting in higher Hb increase include lower Hb values prior to transfusion, the length of Hb measurement intervals before and after transfusion, as well as the method of RBC cell separation 4.