Table 3.

Transfusion recommendations based on sickle cell genotype, hemoglobin, and surgical risk classification

Sickle cell genotypeHemoglobin (g/dL)Surgical riskTransfusion recommendation
HbSS or HbSβ0- thalassemia <9 Low or moderate Simple transfusion, partial exchange, or RCE 
HbSS or HbSβ0- thalassemia >9 Low or moderate Partial exchange or RCE 
HbSC, HbSβ+-thalassemia, and HbSS on hydroxyurea and elevated HbF without severe phenotype* >9 Low No transfusion 
HbSC or HbSβ+- thalassemia >9 Moderate Partial exchange or RCE 
All genotypes — High RCE 
Sickle cell genotypeHemoglobin (g/dL)Surgical riskTransfusion recommendation
HbSS or HbSβ0- thalassemia <9 Low or moderate Simple transfusion, partial exchange, or RCE 
HbSS or HbSβ0- thalassemia >9 Low or moderate Partial exchange or RCE 
HbSC, HbSβ+-thalassemia, and HbSS on hydroxyurea and elevated HbF without severe phenotype* >9 Low No transfusion 
HbSC or HbSβ+- thalassemia >9 Moderate Partial exchange or RCE 
All genotypes — High RCE 
*

Severe phenotype is defined as having a history of stroke, recurrent acute chest syndrome, or prior severe surgical complications.

HbSC, hemoglobin SC disease.

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