Transfusion Service
Hannah Schuett, MD
Cedars-Sinai Medical Center, California, United States
Severe and life-threatening complications of sickle cell disease (SCD), including acute chest syndrome (ACS), cerebrovascular events, and end organ damage often require red blood cell (RBC) transfusions as part of therapeutic management. Patients with SCD are at increased risk for severe transfusion-related complications including severe delayed hemolytic transfusion reactions (DHTR) and hyperhemolysis (HH). While uncommon, HH is both difficult to prevent and manage. These challenges are further compounded in the obstetric population, as pregnant patients with SCD may experience sudden fetal loss. In addition, worsening anemia requiring additional transfusion support may lead to red blood cell alloimmunization and hemolytic disease of the fetus and newborn.
We present a case of severe DHTR/HH in a 31-year-old woman with SCD who presented with ACS at 29 weeks gestation. Her hemoglobin (Hb) was 8.3 g/dL on admission and following automated RBC exchange was 9.2 g/dL. She did well and was discharged 2 days post exchange. She returned to the hospital 9 days after discharge with fever, flu-like symptoms, and abdominal pain; her Hb was 7.4 g/dL and 3.9 g/dL 3 days later. Six previously undetected alloantibodies [C, E, Fy(a), Jk(b), K, and S] were identified. Of the fully compatible Rh and K matched RBC units transfused during the RBC exchange, retrospective phenotyping of the units revealed 3 were Jk(b) positive, 4 were Fy(a) positive, and 1 was S positive.
She required a two-week hospitalization for severe DHTR/HH management which included treatment with prednisone, intravenous immunoglobulin, eculizumab, and erythropoietin along with supportive care in an attempt to reach 32 weeks gestation at which time an elective Cesarean section was planned. Additional blood transfusions were initially avoided. However, given her persistent severe anemia, she was transfused an additional 7 crossmatch compatible RBC units. The delivery was uncomplicated, and the baby was admitted to the neonatal intensive care unit in stable condition for routine management of prematurity. The mother’s Hb increased to 8.0 g/dL and she was discharged in stable condition.
Blood transfusions may provide life-saving intervention for individuals with SCD. However, the decision to transfuse should be carefully considered, as they can result in life-threatening complications including severe DHTR/HH. This paradox poses unique challenges in the management of this patient population, especially in pregnant individuals with SCD, where the physiologic changes of pregnancy exacerbate the acute and chronic complications of the underlying hemoglobinopathy.