Vascular lesions of the liver in sickle cell disease. A clinicopathological study in 26 living patients.

BACKGROUND

Hepatic lesions in sickle cell disease have been studied essentially in autopsy series. Previous reports on living patients are rare and concern a limited number of cases. The aim of the present study is to report the clinical, biochemical, and hepatic histological findings in 26 living patients with sickle cell disease and hepatobiliary disease.

PATIENTS AND METHODS

Twenty-six of 510 patients with sickle cell disease, in whom liver tissue was available for histological analysis, were selected. In 21 patients, biopsy was obtained during laparotomy for cholecystectomy; 5 patients underwent needle biopsy for hepatomegaly and/or liver test abnormalities.

RESULTS

Twenty of the 21 cholecystectomized patients, as well as the 5 other patients, had liver vascular lesions consisting of sinusoidal dilatation (23 cases), perisinusoidal fibrosis (19 cases), and acute ischemic necrosis (5 cases). It is of interest that the 21 cholecystectomized patients had clinical signs of complicated cholelithiasis, and that 20 of them had gallbladder stones, with common bile duct lithiasis in only 1 case. In the 25 patients without common bile duct obstruction, symptoms might have been due to vascular lesions, but it must also be noted that in the cholecystectomized patients they did not persist or recur following surgery. In one cirrhotic patient, marked sinusoidal lesions might have favored severe hepatocellular failure that led to liver transplantation. In another patient, fatal hepatocellular insufficiency was possibly due to ischemia. The nonvascular lesions that were observed, ie, chronic persistent or mildly active hepatitis (11 cases) and cirrhosis (2 cases), were always associated with vascular lesions.

CONCLUSION

These results suggest that in sickle cell disease: (1) hepatic lesions are mainly vascular; (2) these lesions can be responsible for acute and/or chronic ischemia that may be severe; (3) symptoms suggestive of acute cholecystitis and/or biliary tract obstruction might be, at least in part, explained by vascular lesions; and (4) biliary tract surgery indications should be considered more carefully.