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

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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.

Original languageEnglish
Pages (from-to)131-133
Number of pages3
JournalHemoglobin
Volume41
Issue number2
DOIs
Publication statusPublished - Mar 4 2017

Keywords

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

ASJC Scopus subject areas

  • Hematology
  • Clinical Biochemistry
  • Genetics(clinical)
  • Biochemistry, medical

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