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Fatal Hyperhemolysis Syndrome in a Postpartum Patient with Sickle Cell Disease


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FatalHyperhemolysisSyndrome in a Postpartum Patient with Sickle Cell Disease

A 31-year-old G3P1 with sickle cell disease (SCD) underwent urgent repeat CD at 32w5d for preeclampsia with severe features. Previous transfusions resulting in multiple alloantibodies limited availability of compatible blood. Despite a hemoglobin of 6.3 g/DL, Hematology and Transfusion recommended againsttransfusion.CD under CSE was completed with 600 mL of blood loss. Albumin and limited crystalloid were given to avoid hemodilution. Cell salvage returned inadequate volume to transfuse. Nasalcannula oxygen was continued. She was stable throughout surgery. Postoperative BP (124/84mmHg),HR (92bpm),RR (8) and temperature (36.5°C) were stable. Hours later, she became somnolent. Vitals were stable with the exception of low temperature. Magnesium was discontinued, and she was actively warmed and transferred to stepdown. Five hours later, she became unresponsive and hypotensive. Labs revealed metabolic acidosis, hypoxemia and anemia (PH 6.9/PCO2 25/PO2 75/BD -25/HCO3 5/O2sat 57%/Hgb2.5). She was intubated, started on multiple vasopressors, and underwent an exploratory laparotomy for a positive FAST exam. Minimal hemoperitoneumwas evacuated. She received 7 units of RBCs (5 unmatched). Postoperative labs were consistent with hyperhemolysissyndrome (HHS). Per hematology, she was treated with Eculizumab, Rituximab, high-dose steroids, and EPO. Despite improving hemolysis, the she developed multisystem organ failure with myopericarditis. She was ultimately transitioned to comfort care and passed away shortly after terminal extubation.

HyperhemolysisSyndrome:  Transfusion complication by hemolysis of native and transfused RBCs that is well-described in SCD. PosttransfusionHgblevel is lower than pretransfusionlevel. 

Post-CD anemia was multi-factorial from potential hemorrhage, DIC, hemolysis and sequestration, and was further complicated by HHS after transfusion. Uncrossed RBCs put her at risk for a hemolytic transfusion reaction. 

Treatment of HyperhemolysisSyndrome:

Avoid RBC transfusion: Prevents worsening hemolysis

IVIG & Steroids: Block immune-mediated destruction of RBCs

EculizumabInhibits complement activation

Erythropoetin: Overcomes erythropoiesis suppression

Folic Acid: Stimulates hemoglobin synthesis

Rituximab:Recovers reticulocytes & reduces alloantibodies 

Plasma Exchange: Preserves RBCs & increases O2-carrying capacity

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