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///2018 Abstract Details
2018 Abstract Details2019-08-02T15:57:01-05:00

Competing Goals: Cesarean Delivery During Acute Chest Syndrome with Delayed Hemolytic Transfusion Reaction and Hyperhemolysis Syndrome

Abstract Number: T4D-6
Abstract Type: Case Report/Case Series

Shawn R Palmeri MD1 ; Chelsey L Woodrum MD2; Grace Lim MD, MS3; Lauri J Adler MD4; Ryan C Romeo MD5; Jonathan H Waters MD6

Competing Goals: Cesarean Delivery During Acute Chest Syndrome with Delayed Hemolytic Transfusion Reaction and Hyperhemolysis Syndrome

Background. Patients with sickle cell disease (SCD) experience pain crises when sickled red blood cells (RBCs) adhere to each other and lead to small vessel occlusion. Patients with SCD require multiple blood transfusions throughout their lifetime, increasing the risk for alloimmunization. Alloimmunization increases the risk of subsequent transfusion-related complications such as delayed hemolytic transfusion reactions and hyperhemolytic syndrome (HHS), a life-threatening condition in which both donor and host RBCs are at risk of hemolysis with additional allogeneic transfusions.

Case. A 33-year-old G7P2042 with HgbSS disease presented with an acute pain crisis at 31 weeks gestation with dichorionic/diamniotic twins . Other history included three prior cesarean deliveries, bilateral pulmonary emboli and alloimmunization (anti-U and anti-Jka antibodies). An international search for donor blood yielded only four compatible units. During admission, the patient developed acute chest syndrome (ACS). She received allogeneic transfusion for an acute drop in hemoglobin. Several days later, she developed laboratory evidence of severe hemolysis and HSS. In the context of severe anemia (Hgb 6.2 g/dL) and declining maternal status, a cesarean delivery was indicated. Multidisciplinary planning resulted in safe cesarean delivery.

Discussion. This case illustrates the complex considerations surrounding management of anemia and ACS in patients at high risk for postpartum hemorrhage, who is concurrently experiencing HHS. Transfusion is typically a mainstay of therapy for ACS in SCD (see table). Given the patient’s degree of hemolysis after initial transfusion, there was significant concern regarding further correction of surgically induced anemia. Hematology consultants ultimately recommended transfusion only for intraoperative hemorrhage. Postoperatively, transfusion was deferred since the patient exhibited no untoward symptoms despite a hemoglobin nadir of 5.1g/dL. The case highlights the importance of prompt diagnosis, management of transfusion complications, and multidisciplinary planning to prevent significant morbidity and mortality in this high-risk population.

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SOAP 2018