Not to be forgotten, acute sickle cell hepatopathy

摘要

We reporta case of an 18-year-old femalewith sickle cell disease(SCD) e hemoglobin SS e who presented with acute abdominalpain, transaminitis, and hepatomegaly. Her past medical historyncluded chronic abdominal pain, acute chest syndrome, cholecys-tectomy, and hearing loss that was corrected with a cochlearimplant. This patient presented to the emergency room with 3months history of intermittent worsening abdominal pain episodesthat were previously diagnosed as vasoocclusive pain crises. Onexam, the patient was hemodynamically stable and had mainlymoderate to severe epigastric and right upper quadrant abdominalpain with the liver being palpable 2cm below the right costalmargin. Laboratory work up showed elevation of liver enzymes,gamma-glutamyl transferase (GGT), total and direct bilirubin, andwhite blood count (WBCs), which all gradually improved duringher hospitalization (Fig. 1). In addition, her hemoglobin leveldecreased by 2.5 g/dL over the course of 6-days. During hospitaliza-tion, the patient had an abdominal ultrasound and a computerizedtomography, both of which showed intrahepatic and extra-hepaticbiliary ductal dilation. She also had an upper endoscopy thatshowed acute erosive esophagitis. Her pain was managed withhydromorphone using patient-controlled analgesia (PCA) and keta-mine infusions. Although herhemoglobin slightly dropped, shewasoverall clinically stable and did not require a simple packed redblood cell (pRBCs) transfusion or exchange transfusion. A fewdays later, the patient was discharged with oral pain medications,fluconazole and omeprazole. The patient's clinical course was sug-gestive of hepatic vaso-occlusive crisis and/orhepatic sequestrationwith concurrent candidal esophagitis.