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Fatal Hyperhemolysis Syndrome in a Postpartum Patient with Sickle Cell Disease
Abstract Number: FCA-38
Abstract Type: Case Report Case Series
A 31-year-old G3P1 with sickle cell disease (SCD) underwent urgent repeat cesarean delivery (CD) at 32w5d for preeclampsia with severe features. Previous transfusions resulting in multiple alloantibodies significantly limited availability of compatible blood. Despite a hemoglobin of 6.3 g/DL, Hematology and Transfusion Medicine recommended against transfusion unless frank hemorrhage occurred. CD under spinal anesthesia was completed with 600 mL of blood loss. Albumin and limited crystalloid were given to avoid hemodilution. Cell salvage returned inadequate volume to transfuse. Baseline nasal cannula oxygen was continued. The patient was stable throughout surgery. Postoperative blood pressure (124/84mmHg), heart rate (92bpm), respiratory rate (8) and temperature (36.5°C) were stable.
Several hours later, the patient became somnolent. Vitals were stable with the exception of low temperature. Magnesium was discontinued, and the patient was actively warmed. Given her medical complexity, she was transferred to stepdown care. Five hours later, she became acutely unresponsive and hypotensive. Labs revealed severe metabolic acidosis, hypoxemia and anemia (PH 6.9/PCO2 25/PO2 75/BD -25/HCO3 5/O2sat 57%/Hgb 2.5). She was intubated, started on multiple vasopressors, and underwent emergent exploratory laparotomy for a positive FAST exam. Hemoperitoneum (350ml) originating from the rectus abdominus muscle was evacuated. She received 7 units of RBCs (5 unmatched). Postoperative labs were consistent with hyperhemolysis syndrome. Per hematology, she was treated with Eculizumab, Rituximab, high-dose steroids, and Erythropoietin. Despite improving hemolysis, the patient developed multisystem organ failure with diffuse myocarditis, renal failure, and DIC. She was transfused two more matched RBCs for diffuse ST segment elevations, hypotension, and a Hgb of 3.3 g/DL. She was ultimately transitioned to comfort care and passed away shortly after terminal extubation.
Hyperhemolysis syndrome (HHS) is a transfusion complication characterized by hemolysis of both native and transfused RBCs and is well-described in SCD. It is characterized by posttransfusion Hgb levels that are lower than pretransfusion. Post-cesarean anemia was multi-factorial in the setting of potential hemorrhage, DIC, sickle cell hemolytic crisis and sequestration, and was further complicated by the development of HHS after transfusion. Administering uncrossmatched blood also put her at risk for a hemolytic transfusion reaction. Treatment of HHS includes avoiding further transfusions and administering IVIG, steroids, the C5 convertase inhibitor Eculizumab to inhibit complement activation, erythropoietin to overcome erythropoiesis suppression, folic acid to stimulate hemoglobin synthesis, and Rituximab for rapid recovery of reticulocytes and reduction of alloantibody production.
Banks M, Shikle J. Arch Pathol Lab Med. 2018.