Clinical Features of Acute Sickle 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