Some patients with sickle cell anemia may develop an acute hepatic crisis with prominent cholestasis. Widespread sickling of erythrocytes within sinusoids results in an ischemic hepatopathy.


Distinguishing features from acute hepatic crisis:

(1) greater cholestasis, with higher serum total bilirubin and an elevated serum alkaline phosphatase

(2) presence of multi-organ failure (renal failure, coagulopathy, metabolic, encephalopathy)

(3) higher mortality rate


Clinical features:

(1) acute right upper quadrant pain

(2) nausea and vomiting

(3) fever

(4) marked jaundice

(5) tender hepatomegaly

(6) dysfunction or failure in other organs, especially the kidneys


Histologic features:

(1) diffuse ballooning of hepatocytes

(2) widespread sickling of erythrocytes in sinusoids

(3) intracanalicular cholestasis


Laboratory findings:

(1) leukocytosis

(2) variable elevation of LDH and serum transaminases (from normal to marked elevation)

(3) variable elevation of serum alkaline phosphatase

(4) marked elevation in serum total bilirubin with conjugated usually greater than unconjugated

(5) variable coagulopathy (prolonged PT and aPTT, decreased fibrinogen, thrombocytopenia)

(6) often an elevation in serum creatinine

(7) variable lactic acidosis


The mortality rate is high if multi-organ failure develops. Survival may be increased by early initiation of an aggressive program of exchange transfusions and correction of coagulation defects.


Differential diagnosis:

(1) acute hepatic crisis

(2) fulminant viral hepatitis

(3) acute cholecystitis or mechanical cholestasis


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