(502) Hyperhemolysis, Sickle Cell Disease, and Liver Cirrhosis in a Young Woman: An ICU Perspective

Introduction: Hyperhemolysis is a life-threatening complication of Sickle Cell Disease characterized by destruction of red blood cells (RBC) in response to transfusions, limiting available resuscitative measures. Few reports exist on the management of these patients. Here, we present a case of hyperhemolysis in the setting of concomitant hepatic cirrhosis and renal failure.

Description: A 49-year-old woman with history of Sickle Cell Disease (baseline Hgb7gm/dL) and Liver Cirrhosis (from sickle cell disease) was admitted with altered mental status. Initial labs were notable for Anemia (Hgb5.7gm/dL HgbS92.2%), AKI(Cr1.34mg/dL), and Liver Failure (Bilirubin40mg/dL). Anemia progressively worsened with minimal improvement (Hgb5.0-5.3gm/dL) with 1-unit warmed pRBC (patient noted to be cold autoantibody positive). Peripheral smear confirmed hemolysis and IVIG and steroid therapy were initiated. Patient also started on CVVH for worsening AKI. Hemoglobin initially responded to a peak of 8.5gm/dL and then progressively declined to a nadir of 2.8gm/dL. Hyperhemolysis persisted with Haptoglobin < 8mg/dL, LDH>7500U/L, retic 25.8%, and direct coombs positive. Given the patient’s liver cirrhosis and associated coagulopathy (INR2.8, Fibrinogen128mg/dL) and thrombocytopenia (12k/mcL), CTA imaging was obtained which was negative for overt bleeding. Patient also with low factor X (49%), V (44%), and elevated factor VIII (683%), less indicative of DIC. Eculizumab was added on for hyperhemolysis and coagulopathy was corrected along with slow transfusion of pRBC. The patient was intubated for hypoxia, maintained on CVVH for acidosis (Lactate19.8MMol/L), sedated, paralyzed to minimize oxygen consumption and started on vasopressor and fluid support for shock. Hemoglobin was noted to become unmeasurable, with subsequent asystole cardiac arrest and death on day 14 in the setting of hypoxia and anemia.

Discussion: The rapid decline and subsequent death over a 2-week admission from hyperhemolysis demonstrates the importance of prompt recognition of the disorder and careful consideration of definitive treatments and supportive measures to maximize oxygen delivery and minimize metabolic demand. In the setting of concomitant liver and renal failure, patients face a high risk of morbidity and mortality.