Hepatic dysfunction in sickle cell disease: a new system of classification based on global assessment.
OBJECTIVE
Hepatic dysfunction in adults with sickle cell disease varies in character and severity from self-limited cholestasis to life-threatening acute liver failure and cirrhosis. Because previous attempts to describe patterns of liver disease have not reflected clinical experience, we aimed to characterize the presentation, clinicopathologic findings, and natural history of such patients.


METHODS
We reviewed the clinical, laboratory, radiographic, and histologic features with the natural history of 38 patients (mean age, 33 years) with Hb SS, SC, or S-beta thalassemia referred to a tertiary liver center for assessment.


RESULTS
Distinct disease patterns were identified that comprised massive hepatocellular necrosis (5%), acute severe sequestration and cholestasis in the context of sepsis (18%), cirrhosis (18%), chronic, fluctuating sequestration without cholestasis (21%), mechanical biliary obstruction (8%), siderosis without cirrhosis (8%), generalized cholangiopathy (8%), venous outflow obstruction (3%), and miscellaneous (11%). Of the 20 who required emergency admission, 8 did not survive their index admission, and 3 patients died during follow-up admissions (4 months-4 years later). There were 3 instances of hemorrhage related to liver biopsy. One patient underwent transplantation but died. Hematologic and biochemical markers did not discriminate well between survivors and nonsurvivors. The incidence of a second hepatic pathology (ie, viral hepatitis, autoimmune disease, transfusional siderosis) was 37% and was associated with the finding of more advanced histologic fibrosis.


CONCLUSIONS
Patterns of hepatic dysfunction in sickle cell disease are diverse and demand clear characterization for each individual; however, groups with a poor prognosis can be identified after collation of clinical, laboratory, and radiologic data. Findings at biopsy (which is associated with higher risk of bleeding in this group) might be anticipated by noninvasive test results.
Berry
Philip A
PA
Institute of Liver Studies, King's College Hospital, London, United Kingdom. philaberry@hotmail.com
Cross
Timothy J S
TJ
Thein
Swee Lay
SL
Portmann
Bernard C
BC
Wendon
Julia A
JA
Karani
John B
JB
Heneghan
Michael A
MA
Bomford
Adrian
A
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications*; D001706:Biopsy; D002758:Cholangiography; D002760:Cholangiopancreatography, Endoscopic Retrograde; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology; D008107:Liver Diseases / Q000145:classification* / Q000175:diagnosis / Q000209:etiology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography