Acute B Virus Hepatitis with Fulminant Hepatic Failure Precipitating Crisis in Sickle Cell Disease.

ABSTRACT

Sickle cell disease (SCD) is a hemoglobinopathy which involves multiple organs in the body. Hepatobiliary manifestations in SCD are many. Acute and chronic HBV infection may be transmitted through multiple blood transfusions in SCD. We report the case of a 36-year-old male with sickle cell disease who presented to us in acute liver failure and sickle cell crisis due to acute hepatitis B virus infection. Sickle cell disorder is a broad category under which all the patients who have a point mutation (haemoglobin S-HbS) on at least one Beta chain due to substitution of valine in place of glutamic acid at position 6 are included. Due to this substitution, HbS molecules have a sticky site allowing the molecules to aggregate. This aggregation worsens in deoxygenated state leading to the development of long polymers, sickling of erythrocytes and haemolysis. The most commonly affected site in SCD is the hepatobiliary system. The presentation due to this involvement varies from benign hyperbilirubinemia to a spectrum of manifestations termed “sickle cell hepatopathy”.1