Portal vein thrombosis in a child with homozygous sickle-cell disease

A 13-year old boy with homozygous sickle-cell (SS) disease died suddenly at home folllowing a short history of abdominal pain. Autopsy revealed venous thrombosis of the hepatic, portal, superior mesenteric and splenic veins. Venous thrombosis is rare in SS disease and thrombosis of mesenteric vessels is most frequently seen in chronic myeloproliferative disorders. Its occurrence in SS disease raises the possibility of a common pathogenesis and adds another pathology to the causes of abdominal painful crisis.