Liver involvement in acute vaso-occlusive crisis of sickle cell disease: Prevalence and predisposing factors

Abstract

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.

Liver stiffness increases acutely during sickle cell vaso‐occlusive crisis

“…17 In the cohort of patients in this manuscript, both ALT and ALP correlated with increasing TE during VOC. However, TE was also noted to increase in some who did not have substantial increases in ALT or ALP suggesting that increase in liver stiffness is not always reflected in biochemical changes.…”

“…9,17,47,48 It has been suggested that this is due to widespread sickling with resultant obstruction in the sinusoids leading to hepatocyte anoxia and ischemia, and ultimately necrosis and death. Available definitions of hepatic involvement in acute VOC are imprecise given the overlap between sickle cell and hepatic serum biochemical parameters and the poor correlation of the serum biomarkers with acute VOC, which was corroborated in this study.…”

“…10,15–18 Clinical gastrointestinal manifestations include right upper quadrant pain, acute painful hepatomegaly, abdominal distention, fever, jaundice, and painful abdominal crisis. 17,19,20 Biochemically, serum transaminases abnormalities vary along with bilirubin levels. 10,19,21 Prior studies attempting to utilize biochemical parameters as tools to distinguish acute VOC with and without hepatic involvement have been unsuccessful, and specific correlations between clinical, biochemical and histological features of acute VOC remain unclear.…”

“…10,19,21 Prior studies attempting to utilize biochemical parameters as tools to distinguish acute VOC with and without hepatic involvement have been unsuccessful, and specific correlations between clinical, biochemical and histological features of acute VOC remain unclear. 10,17,22 Additionally, the use of liver biopsy as a tool of hepatic assessment during acute VOC has proven to be a dangerous practice with resultant complications in up to 80% of patients including hemorrhage, organ failure and death. 14,23,24 Given the lack of clear definitions and unsatisfactory tools available for assessing hepatic involvement, the dangers of liver biopsy during acute VOC and the lack of a clear gold-standard, novel methods of delineating liver damage in VOC are needed.…”

The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS)

“…Our results are in accordance with the results of other studies that reported a high frequency of liver complications in adult SCD patients and their improvement under HU therapy. 21,23,24 Avascular necrosis of bone (ANB) is another frequent and severe complication of SCD. 25,26 In the LaSHS study, at baseline, 12% of patients had a history of symptomatic ANB; a result comparable with that of Milner et al, 25 who found a prevalence of 10% of ANB in 2590 patients with SCD.…”