Approximately 10% of patients with sickle cell anemia will experience an acute hepatic crisis.


Duration: self-limited, from 3-14 days


Clinical features:

(1) acute right upper quadrant pain

(2) nausea

(3) low grade fever

(4) jaundice

(5) tender hepatomegaly


Laboratory findings:

(1) usually mild elevation in the transaminases unless there is extensive hypoxic injury

(2) hyperbilirubinemia, usually < 15 mg/dL


Pathologic findings:

(1) sickle cell thrombi causing sinusoidal obstruction

(2) hypertrophy of Kupffer cells with phagocytized red blood cells

(3) mild centrilobular necrosis

(4) variable cholestasis


Patients with concomitant liver disease (cocaine hepatotoxicity, other) may experience more extensive damage, as manifested by a marked increase in the serum transaminases and more extensive necrosis.


Differential diagnosis:

(1) acute viral hepatitis

(2) toxic hepatopathy

(3) cholelithiasis


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