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How Low Can the Hemoglobin Go? Hyperhemolysis in a Pregnant Patient with Sickle Cell Disease
Abstract Number: T-65
Abstract Type: Case Report/Case Series
Introduction: Hyperhemolysis is a rare, life-threatening transfusion reaction characterized by destruction of donor and recipient red blood cells resulting in severe anemia below pre-transfusion levels . It is usually associated with sickle cell disease (SCD) or a history of pregnancy  but very rarely reported in parturients. It differs from delayed hemolytic transfusion reaction as it is more severe, often no new antibodies are found, and it is associated with reticulocytopenia [1,2,3]. Following an initial episode there is increased risk of hemolysis during future transfusions. Severe anemia and hemodynamic instability affect parturient and fetal well-being , and may complicate the anesthetic plan.
Case: We present a 22year old G1P0 parturient at 28 weeks gestation with SCD, a history of transfusions with development of alloantibodies who presented with sickle-like pain, fever, and severe anemia. Seven days prior, during an acute pain crisis, the patient received 1 unit of PRBCs. At the current admission the Hbg had fallen to 4.4g/dl with no source of bleeding. A diagnosis of hyperhemolysis was made with assessments of lab values including; LDH, bilirubin, Coombs test, and Hgb fractionation studies. Obstetrics, anesthesiology, and hematology services met to discuss the treatment and delivery plan. Hematology recommended delaying delivery as hgb was likely to improve, while MFM and anesthesiology services were concerned about fetal status and need for emergent delivery without the ability to transfuse. IVIG, methylprednisolone, and 2 units of antigen negative blood were given with a rise in hgb to 6.5g/dl. Hgb remained stable, darbopoetin was started and the patient was discharged to home. At 33+5 wga she re-presented with a sickle cell crisis (hgb = 6.1g/dl). Prolonged decelerations, severe IUGR and abnormal umbilical artery dopplers necessitated an urgent delivery. An epidural catheter was placed in the lateral position and slowly titrated with stable maternal and fetal hemodynamic profile. An uncomplicated primary low transverse cesarean section was performed with BTL and EBL of 600mL. Apgar scores were 9/9. No blood transfusions or plasma exchange were needed.
Discussion: Hyperhemolysis occurring in a pregnant patient places both the patient and the fetus at risk for life-threatening complications, mandating a multi-disciplinary approach. Key decisions include whether to transfuse, choice of treatment, timing of delivery, and the anesthetic plan. Providers must be aware how to coordinate and provide appropriate blood therapy during the peripartum period. Anesthetics provided during delivery should minimize the risk of post-partum hemorrhage and hemodynamic instability to decrease the risk of blood transfusion or fetal compromise.
1) Danaee et al. Transfusion Med Reviews 2015;29:220-30
2) Elenga. Amer J Clin Med Research 2013;1(3):40-4
3) Win et al. Transfusion 2001;41:323-8
4) Haider et al. BMJ 2013;346:f3443