Resolution of chronic hepatic sequestration in a patient with homozygous sickle cell disease receiving hydroxyurea.
Hepatic sequestration is an uncommon complication in patients with homozygous sickle cell disease. Although transfusion therapy has been effective for the acute condition, no definitive treatment of chronic hepatic sequestration has been identified. We describe a 17-year-old male patient with hemoglobin SS and chronic hepatic sequestration who was treated with long-term (60 months) hydroxyurea. After 36 months of HU therapy, the patient had both an excellent hematologic response and a resolution of hepatic sequestration, as evidenced by disappearance of clinical hepatomegaly, normalization of liver volume on serial computed tomography scans, as well as decreased sinusoidal dilatation and congestion and red blood cell sickling on liver biopsy. The findings in this case suggest that hydroxyurea may benefit patients who have unusual complications of sickle cell disease, such as chronic erythrocyte sickling in the liver.
Jeng
Michael R
MR
Departments of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105, USA.
Rieman
Martha D
MD
Naidu
Paula E
PE
Kaste
Sue C
SC
Jenkins
Jesse J
JJ
Serjeant
Graham
G
Wang
Winfred C
WC
D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000188:drug therapy* / Q000473:pathology; D002908:Chronic Disease; D006529:Hepatomegaly / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D008297:Male
D006918:Hydroxyurea