Sickle Cell Hepatic Sequestration

Hepatic sequestration is acute liver enlargement from red cell pooling in sinusoids with falling haemoglobin.

• Rare; occurs in children and adults with HbSS; part of sickle hepatopathy spectrum (with intrahepatic cholestasis, ischaemic hepatitis, hepatic crisis).
• Mild form resolves; severe form associated with cholestasis and liver failure.
• Mortality high in intrahepatic cholestasis (acute sickle hepatopathy).

• Right upper quadrant pain, rapidly enlarging tender liver, jaundice, fever.
• Falling haemoglobin, pallor, tachycardia.
• Severe cholestasis: coagulopathy, encephalopathy, renal failure.

• Haemoglobin fall, reticulocytosis, raised bilirubin (direct), ALT, ALP; extremely high bilirubin (over 50 mg/dL) and coagulopathy in cholestatic form.
• Ultrasound: hepatomegaly; exclude gallstones, cholangitis, hepatitis, Budd Chiari.
• Viral hepatitis serology, drug history, ferritin, INR, ammonia.
• Liver biopsy rarely (risky).

• Supportive: oxygen, hydration, analgesia, avoid hepatotoxic drugs.
• Simple transfusion for anaemia (cautious volume).
• Exchange transfusion (HbS target under 30%) for severe hepatic crisis, progressive cholestasis, liver failure, coagulopathy.
• Vitamin K 10 mg IV, fresh frozen plasma if bleeding.
• Treat triggers such as infection.
• Chronic transfusion programme after severe episode.
• Liver transplant has been used for liver failure in selected cases.
• Hydroxyurea for prevention of vaso occlusive events.