Hepatic Crisis in Sickle Cell Anaemia – A Case Report

Abstract

Hepatic crisis in sickle cell disease (SCD) has been reported in about 10% of adults but occurs rarely in children. This is a report of a case of a hepatic crisis in a 15-year-old male with SCD with the aim of highlighting the presentation, diagnosis and management. A 15-year-old male, known SCD patient (homozygous Haemoglobin SS) presented with a 6-day history of worsening jaundice, 5-day history of fever and a 4-day history of abdominal pain and vomiting. On examination, he was deeply jaundiced with a greenish tinge, in painful distress with marked right hypochondrial tenderness and hepatomegaly of 12 cm. (compared to no palpable liver when he was seen a week prior to presentation). His haematocrit done was 13% (compared to a haematocrit of 26% a week to presentation). Serum aspartate and alanine aminotransferase were mildly elevated. He also had conjugated hyperbilirubinemia. An abdominal ultrasound scan showed diffuse hepatomegaly. Hepatitis B surface antigen (HBsAg), hepatitis C virus (HCV), Human immunodeficiency virus (HIV) I and II assay were negative. He was managed with serial blood transfusions. He also received antibiotics and analgesics. He made a rapid clinical recovery and was discharged home after 4 days on admission. This case is being reported to provide insight into the presentation, diagnosis and management of this condition. It is especially useful for clinicians who are not familiar with the intrahepatic vaso- occlusive syndromes in sickle cell disease.