Hemobilia: Secondary to Sickle Cell Crisis - The First Case Report

Abstract

Purpose: Hemobilia refers to bleeding into hepatobiliary tract and an uncommon cause of gastrointestinal bleeding. Causes include blunt abdominal trauma, liver biopsy, cholelithiasis, hepatic or biliary neoplasm, etc. Hemobilia can present as life threatening overt or occult gastrointestinal bleeding depending on primary etiology. Relevant clinical history is important for diagnosis. Diagnosis is confirmed with endoscopy or Endoscopic Retrograde Cholangiopancreaticography. Arterial embolization, surgical intervention and treatment of primary pathology are the treatment options. This is the first case report of hemobilia secondary to sickle cell crisis. A 64 y/o male with history of hemoglobin SC-variant of sickle cell disease, hypertension, anemia, bronchial asthma, CVA, dyslipidemia, BPH presented to ER with severe back pain for one day, found to have hematuria, and subsequently developed acute renal failure. His admission blood pressure was 156/69, heart rate was 74. He was afebrile with respiratory rate of 18. He was treated for possible sickle cell crisis and cord compression was ruled out with imaging studies. Following day he developed hypoxia possibly secondary to acute chest syndrome in the setting of sickle cell crisis. Chest X-ray revealed acute infiltrate in left lower lobe. Initial Liver function shows total bilirubin 2.1, AST-112, ALT-57, ALP-182. Hepatitis serology was negative. CT abdomen/pelvis showed asplenia, common bile duct measuring 1 cm, with no aneurysm identified off the celiac circulation. Sonogram didn't reveal cholelithiasis/choledocholithiasis. Due to leukocytosis and progressive increase in bilirubin and transaminase, patient underwent Endoscopic Retrogrades Cholangiopancreatography, which showed active bleeding via major papilla with surrounding clot. Cannulation was unsuccessful. He received multiple packed cells and FFP transfusions, IV fluids and antibiotics. Unfortunately outcome was fatal. Discussion: Hemoglobin SC is a variant of sickle cell disease in which hemoglobin C is inherited along with hemoglobin S (Sickle hemoglobin). Hemoglobin S occurs due to substitution of valine for glutamic acid in sixth position of globin chain. Due to presence of hemoglobin S redcell becomes sickling upon deoxygenation, later on polymerized and causes vaso-occlusive manifestations. Pt with hemoglobin SC present with intermediate severity but Pt with this disease are at risk for similar life threatening complication as sickle cell disease (Hemoglobin SS) does. Acute hepatic ischemia, benign cholestasis, hepatic sequestration crisis can result in some cases. This is the first case report of hemobilia associated with Hb SC disease and sickle cell crisis.