Hepatobiliary Manifestations of Sickle Cell Anemia

Abstract

Sickle cell anemia is one of the common hemoglobinopathies around the world. It results from a single change of one amino acid valine instead of glutamic acid in the hemoglobin beta change. This change leads to polymerization of the hemoglobin when the oxygen saturation is lowered, resulting in deformity of the red blood cells and microvascular occlusion. Sickle cell anemia can affect any part of the body and one of the main organs to be affected is the hepatobiliary system either directly from the sicklening process or indirectly as a result of chronic hemolysis and multiple blood transfusions. This manifests in several clinical conditions which poses diagnostic and therapeutic dilemmas to the treating physicians. These hepatobiliary manifestations will be outlined in this review.

Keywords: Hepatobiliary complications; Sickle cell anemia.

Figure 1

Abdominal ultrasound showing multiple gallstones.

Figure 2

Intraoperative photograph showing acute gangrenous cholecystitis in a patient with SCA.

Figure 3

Percutaneous cholangiogram showing gallstones and common bile duct stones.

Figure 4

ERCP showing bile duct stones pre and post laparoscopic cholecystectomy.

Figure 5

Biliary sludge in the gallbladder and bile ducts.

Figure 6

ERCP showing dilated bile ducts without an obstructive cause (Sickle cell cholangiopathy).

Figure 7

CT-scan of the abdomen showing the liver with iron overload in a patient with SCA and multiple blood transfusions.