Delayed hemolytic transfusion reaction in sickle cell disease

Patients (pts) with sickle cell disease (SCD) are at particular risk for delayed hemolytic transfusion reaction (DHTR) because they may be transfused at intervals over many years, they may receive exchange transfusions increasing exposure to foreign cells, and they frequently form alloantibodies because of antigenic differences from the donor population. We report A pts with SCD (3 SS, 1 S-B * thal) who developed severe DHTR 7-9 days after exchange transfusion (3 pts) and after simple transfusion (1 pt). 3 pts had known prior alloimmunization and developed DHTR despite receiving red cells compatible with all recognized antibodies. The 3 exchange-transfused pts suffered significant complications of their DHTR necessitating additional transfusion. A 22 year old woman, transfused prior to atrial septal defect repair, developed supraventricular tachycardia. A 26 year old pregnant woman required early delivery by c-section because of fetal distress, and the fetus was passively immunized. A 23 year old man with priapism developed severe anemia after DHTR and had an additional reaction when transfused again. All 4 pts had fever at the time of DHTR. 3 had evidence of intravascular hemolysis. The frequency and severity of DHTR in SCD argue for minimizing transfusion in SCD, using phenotypically matched cells, and employing simple, rather than exchange, transfusion. Routine red cell phenotyping of pts, extended matching of donor cells, and screening for alloantibodies after transfusion should be considered in SCD pts.