Acute hepatic sequestration associated with pneumococcal infection in a 5-year-old Boy with sickle beta degrees -thalassemia: a case report and review of the literature.

A 5-year-old black male with sickle beta degrees -thalassemia presented with fever and a vaso-occlusive crisis. Within hours, he developed progressive hepatomegaly with an acute drop in the hemoglobin level that was refractory to repeated red blood cell transfusion. His condition deteriorated and eventually he succumbed to cardiorespiratory failure related to sepsis. Blood cultures grew Streptococcus pneumoniae. To the best of our knowledge, this is the first reported case of hepatic sequestration in a young child and the first reported in a patient with sickle beta degrees -thalassemia. We describe here the clinicohematologic and pathologic features of this case consistent with acute hepatic sequestration and present a review of the relevant literature.