Abstract
Sickle cell hepatopathy is an underreported entity lacking clear management guidelines. This case highlights the potential role of hydroxyurea (HU) in improving the hepatic dysfunction seen among patients with sickle cell disease (SCD). We herein present the clinical course of a patient prior to and after the initiation of hydroxyurea with an emphasis on long-term outcomes and the patterns of liver injury over a 15-year time course.
Highlights
Sickle cell disease (SCD) is a hemoglobinopathy with multisystem involvement and a variety of hepatobiliary manifestations [1]
The causes of disturbed liver functions among patients with sickle cell disease (SCD) vary from the sequelae of intrahepatic sickling and cholestasis due to the primary disease process to the various comorbidities associated with the disease course and its treatment [2,3]
Labeling a patient as a case of sickle cell hepatopathy is achieved by establishing hyperbilirubinemia > 200 umol/L (12 mg/dL) in the absence of hyper-hemolysis and confounding etiologies that may be directly responsible for disturbed liver functions [1]
Summary
Sickle cell disease (SCD) is a hemoglobinopathy with multisystem involvement and a variety of hepatobiliary manifestations [1]. She had a severe disease course characterized by frequent emergency room visits and hospital admissions secondary to pain crises Her history was notable for frequent but irregular simple blood transfusions and a cholecystectomy at 12 years old. The trend after 2015 shows a progressive decline in direct bilirubinemia Her emergency room visits and hospital admissions secondary to hepatic and vaso-occlusive crises significantly dropped from four to six per year to one to two per year, and she reports an improvement in pain-related events. She reports no new complaints or symptoms suggestive of liver damage Her most recent laboratory results were as follows: hemoglobin, 7.9 g/dL (baseline: 7-9 g/dL); mean cell volume (MCV), 114.4 fL; white blood cell (WBC) count, 8.04 K/uL; platelet (PLT) count, 226 K/uL; albumin, 43.5 g/L; ALP, 122 U/L; AST, 52 U/L; ALT, 29 U/L; GGT, 153 U/L; total bilirubin, 92 umol/L; direct bilirubin, 15 umol/L; and ferritin, 118 ng/mL. The macrocytosis relative to her baseline MCV is reflective of good compliance
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