Published online Mar 27, 2019. doi: 10.4254/wjh.v11.i3.287
Peer-review started: November 26, 2018
First decision: December 10, 2018
Revised: January 16, 2019
Accepted: January 28, 2019
Article in press: January 28, 2019
Published online: March 27, 2019
Sickle cell hepatopathy is a category of pathologies that occur among patients with sickle cell disease, and has been rising in incidence as lifespan has increased. Additionally, based on autopsy studies, sickle cell hepatopathy is felt to be underreported and likely contributes to mortality in more cases than in realized. Previous studies have tried to identify risk factors associated with sickle cell hepatopathy but have been limited by small size.
With the increasing incidence of sickle cell hepatopathy, understanding risk factors and improving its recognition are important to its early diagnosis and treatment.
The main objectives of this study were to describe the prevalence of extreme hyperbilirubinemia, its effect on morbidity and mortality, and any association between sickle cell genotype and sickle cell hepatopathy.
We used a retrospective observational cohort study to evaluate the epidemiology and outcomes behind extreme hyperbilirubinemia, a form of sickle cell hepatopathy. This was conducted at a hospital with a large population of patients with sickle cell disease.
About 5% of patients in our sickle cell disease database developed extreme hyperbilirubinemia. This cohort was associated with higher rates of systemic illness, measured by quick Sequential Organ Failure Assessment scores, higher rates of blood transfusions and higher rates of exchange transfusions. There was not a higher mortality rate in the extreme hyperbilirubinemia group. There were higher rates of patients with hemoglobin SS sickle cell disease among the extreme hyperbilirubinemia group compared to a control group, compared to other genotypes. Additionally, there were not significant differences in hydroxyurea use between groups.
Our study highlights the increased morbidity and use of blood products seen among patients with extreme hyperbilirubinemia, a form of sickle cell hepatopathy. It also identifies different rates of sickle cell hepatopathy depending on the sickle cell genotype present. Finally, it shows that reported hydroxyurea doses did not have an effect on development of sickle cell hepatopathy.
Our study highlights the need for further study into types of sickle cell hepatopathy, whether strategies other than hydroxyurea can mitigate the risk of development of sickle cell hepatopathy, and whether there are any identifiable risk factors to increase rates of early diagnosis.