Clinical features of hepatic sequestration in sickle cell anaemia.

Abstract

Episodes of hepatic sequestration were seen in four patients with sickle cell anaemia (SCA). One case presented a severe and fatal sequestration crisis whereas the others showed mild episodes associated with less striking haematological and clinical changes. These clinical pictures are similar to those seen in the splenic sequestration crises of children with SCA. Different causes of liver enlargement in SCA suggest that the clinical spectrum representing intrahepatic trapping of blood could range from the acute sequestration crisis to chronic sequestration events which very probably should play any role in the pathogenesis of the hepatomegaly frequently found in these patients.