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The parturient with severe sickle cell disease and red blood cell alloimmunization
Abstract Number: FCI-461
Abstract Type: Case Report Case Series
Despite progress in treatment of sickle cell disease (SCD), pregnancy remains a high risk situation for both mother and fetus. There have been no published reports of the parturient with SCD with alloimmunization to red blood cell antigens, despite rates of red blood cell alloantibodies being 18-38% amongst SCD patients.1 We present a case of a parturient with a complicated history of SCD presenting with severe anemia and red blood cell alloimmunization requiring cesarean delivery (CD).
A 25 year old G2P0010 presented with homozygous SCD complicated by a history of multiple recurrent occlusive events including bone crises, acute chest syndrome, multi-organ infarcts, frontal lobe stroke, and bilateral internal carotid artery occlusions. Her pathology was further complicated by multiple alloantibodies from previous transfusions. A recent severe hemolytic reaction demonstrated presence of an additional unspecified antibody. She was admitted to hospital at 29 weeks 3 days gestation with hemoglobin (Hb) 53. She was difficult to crossmatch due to the alloimmunization. Given the scarcity of compatible blood and high risk of transfusion reaction, the decision was made to transfuse only if absolutely necessary. With supportive measures and darbepoietin, she had gradual return of Hb to baseline levels in the mid-80’s.
An induction of labour was planned in hopes of avoiding an operative delivery. Given her SCD, intraoperative cell salvage was not an option. Crossmatched blood remained severely limited. At 36 weeks gestation her Hb dropped to 41 and her platelet count to 64. The decision was made to proceed to CD at this time due to severe fetal IUGR. She was given IVIG, methylprednisolone, and rituximab prior to preoperative transfusion of 1 unit packed red blood cells. In the operating room, standard CAS monitors were applied. General anesthesia was administered with rapid sequence induction. Arterial line and second large bore IV were obtained post induction. Hb on initial baseline gas was 60. Care was taken to avoid hypoxia, hypotension, hypothermia, and acidosis. A female infant was delivered uneventfully with Apgars 1 at 1 minute and 8 at 5 minutes. The uterus was initially noted to be atonic with uterine inversion during placenta removal. Carbetocin 100 mcg, tranexamic acid 1g and carboprost 250 mcg were administered with improvement of uterine tone. Total blood loss was 700 mL. She did not receive further transfusion intraoperatively. She had a planned admission to the intensive care unit, intubated and ventilated. Hb was 42 on postoperative day (POD) 1. Her postpartum course was complicated by an acute chest crisis, an episode of demand ischemia, and ongoing anemia requiring a second unit of PRBC transfusion. She was discharged home POD 15. This case highlights the complexities in managing the complex parturient with SCD and alloimmunization to red blood cell antibodies undergoing CD.
1. Transfus Med Hemother 2014;41(6):431-5