Approximately 10% of patients with sickle cell anemia will experience an acute hepatic crisis.
Duration: self-limited, from 3-14 days
(1) acute right upper quadrant pain
(3) low grade fever
(5) tender hepatomegaly
(1) usually mild elevation in the transaminases unless there is extensive hypoxic injury
(2) hyperbilirubinemia, usually < 15 mg/dL
(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.
(1) acute viral hepatitis
(2) toxic hepatopathy
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Specialty: Gastroenterology, Hematology Oncology, Genetics