Sickle Cell Disease. A Serum Defect in the Thromboplastin Generation Test

1. A defective ability of sickle cell disease (SCD) serum to support thromboplastin generation was demonstrated.

2. The serum defect was associated primarily with homozygous SCD, although defects of varying severity were also noted in the heterozygous forms.

3. The deficiency appears to be due to an accelerated loss of thrombin or thrombin-like factors from serum.