Decompensated Cirrhosis and Sickle Cell Disease: Case Reports and Review of the Literature

Abstract

Although its prevalence is unknown, liver involvement by sickle cell disease is not uncommon and encompasses different clinical spectra including non cholestatic and cholestatic disorders. Few data have been provided on chronic sickle cell intrahepatic cholestasis (SCIC) clinical course, although cirrhosis has been reported in sickle cell disease. However, no effective therapeutic approaches have been recognized either to prevent or treat this condition. Here we present two cases of adult sickle cell disease patients with decompensated cirrhosis. Their liver biopsies showed sickle cell thrombi within the hepatic sinusoids. Despite erythroexchange (EEX) transfusions, both patients suffered from major sickle cell disease-related events, suggesting that EEX transfusions may not be enough to impact on advanced liver involvement by sickle cell disease.

Keywords:

• Cirrhosis
• erythroexchange (EEX) transfusion
• liver biopsy
• liver decompensation
• sickle cell disease

Acknowledgments

The study was reviewed and approved by the Institutional Review Board of the Internal Medicine Unit at the Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico in Milan, Italy.

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.