A 30-year-old man with known sickle cell disease (HbSS) presents with sudden onset right upper quadrant pain, jaundice, worsening anemia (Hb dropped from 8 to 5 g/dL), and a tender rapidly enlarging liver. Reticulocyte count is 18%. The most likely diagnosis is:

• A Acute cholecystitis from pigment gallstones
• B Vaso-occlusive crisis with hepatomegaly
• C Fulminant hepatic failure from HBV reactivation
• D Acute hepatic sequestration crisis ✓

Explanation

Acute hepatic sequestration crisis in sickle cell disease is characterized by sudden trapping of large volumes of sickled red cells in the hepatic sinusoids, causing rapid hepatomegaly, right upper quadrant pain, acute fall in hemoglobin (often >2 g/dL from baseline), and rising reticulocyte count (indicating bone marrow response). It is analogous to splenic sequestration but occurs in the liver. Treatment is urgent simple or exchange transfusion to restore hemoglobin and reverse sequestration. Acute cholecystitis is associated with bilirubin stones but not acute hemoglobin drop.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

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Written and medically reviewed by the StethoPrep medical team.