Liver involvement in acute vaso-occlusive crisis of sickle cell disease: prevalence and predisposing factors.
OBJECTIVE
To study the prevalence and predisposing factors of liver involvement in sickle cell disease (SCD) of patients with acute vaso-occlusive crisis.


METHODS
We prospectively evaluated 41 consecutive patients (44% M, median age 39 years, range 16-56 years) with homozygous (HbSS; 12 cases) or sickle cell-beta thalassemia (HbSbeta-thal; 29 cases), admitted to our Medical Department in the period 2002 to 2004. Severity of crisis was graded by in-house-modified APACHE score; presence of asplenia or functional hyposplenism was also considered. Hematological and biochemical parameters including various relevant enzymes/isoenzymes were followed daily.


RESULTS
Despite the fact that only 9 patients (22%) presented with acute painful hepatomegaly, liver involvement was evident in 16 (39%); hepatocellular-type injury was found in 1 patient, cholestatic in 8, and mixed in 7. Severity of crisis was not related to liver involvement (score 20.6 versus 18.2), but liver involvement occurred in the presence of normal spleen function (p<0.001) and platelet counts <500,000/mm(3) (p<0.001) were. Patients with liver involvement, compared with those without, had higher total and direct bilirubin levels (4.3 versus 2.9 mg/dL, p=0.050; 1.9 versus 0.8 mg/dL, p=0.010, respectively), lower hematocrit (19% versus 23%, p=0.030) and longer hospitalization (10 versus 6.3 days, p<0.001).


CONCLUSIONS
In SCD, there is a 39% prevalence of acute veno-occlusive involvement of the liver, a figure that is much higher than previously reported. The type of injury is mostly mixed hepatocellular-cholestatic or purely cholestatic and its course is usually benign. Liver involvement occurs more often in patients with normal spleen function and is not associated with the overall severity of the acute episode, both observations being unreported previously.
Koskinas
John
J
Academic Department of Medicine, Hippokration General Hospital, 114 Vas. Sophias Avenue, GR-11527 Athens, Greece.
Manesis
Emanuel K
EK
Zacharakis
George H
GH
Galiatsatos
Nikolaos
N
Sevastos
Nikolaos
N
Archimandritis
Athanasios J
AJ
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008099:Liver / Q000503:physiopathology; D008107:Liver Diseases / Q000150:complications*; D008297:Male; D008875:Middle Aged; D017086:beta-Thalassemia / Q000150:complications