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Hepatic manifestations of sickle cell disease

Authors
Subhas Banerjee, MD
Michael R DeBaun, MD, MPH
Section Editors
Sanjiv Chopra, MD, MACP
Stanley L Schrier, MD
Deputy Editor
Anne C Travis, MD, MSc, FACG, AGAF

INTRODUCTION

Sickle cell disease (SCD) encompasses a group of hemoglobinopathies characterized by a single amino acid substitution in the ß-globin chain. The most frequently occurring form of SCD is sickle cell anemia (HbSS), followed by HbSC and HbSß-thalassemia. In SCD the majority of the hemoglobin (greater than 50 percent) is hemoglobin S. In the United States, 6 to 10 percent of African-American newborns have sickle cell trait (HbSA), and approximately 0.2 percent have sickle cell anemia (HbSS) [1]. Sickle cell trait is not considered SCD. (See "Introduction to hemoglobin mutations".)

The liver can be affected by a number of complications due to the disease itself and its treatment [2-4]. In addition to the vascular complications from the sickling process, patients with SCD have often received multiple transfusions, placing them at risk for viral hepatitis, iron overload, and (combined with the effects of chronic hemolysis) the development of pigment gallstones, all of which may contribute to the development of liver disease. (See "Epidemiology and transmission of hepatitis C virus infection", section on 'Blood transfusion' and "Epidemiology, transmission, and prevention of hepatitis B virus infection", section on 'Transfusion' and "Red blood cell transfusion in sickle cell disease", section on 'Excessive iron stores'.)

The term "sickle cell hepatopathy" has sometimes been used to reflect the overlapping causes of liver dysfunction in these patients. Sickle cell hepatopathy occurs predominantly in patients with homozygous sickle cell anemia, and to a lesser extent in patients with HbSC disease or HbSß-thalassemia.

This topic will review the hepatic manifestation of SCD. Other issues related to SCD are discussed elsewhere. (See "Overview of the clinical manifestations of sickle cell disease" and "Overview of the management and prognosis of sickle cell disease".)

EPIDEMIOLOGY

The overall incidence of liver disease in patients with sickle cell disease (SCD) has not been well established. The major risk factor for liver disease in patients with SCD is receiving multiple blood transfusions, which is associated with infection (hepatitis B and C) and excessive iron stores.

                           

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Literature review current through: Nov 2016. | This topic last updated: Tue Jan 26 00:00:00 GMT+00:00 2016.
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