Hepatic sequestration in sickle cell anemia.

INTRODUCTION Sickle cell anemia is a chronic debilitating disease affecting a significant portion of patients of African American origin. These patients present to the physicians with myriad of life threatening complications like acute chest syndrome, septic shock, decompensated congestive heart failure secondary to severe pulmonary hypertension, stroke, and multi-organ failure. However, there are multiple other less common clinical presentations which would require a high index of suspicion. One such condition is hepatic sequestration which though infrequent is potentially life threatening. The clinical diagnoses of a sickle cell patient with hepatic vaso-occlusion vary from a relatively benign sickle cell hepatic crisis, to a more severe hepatic sequestration, to an extremely fatal sickle cell intra-hepatic cholestasis. 1,2 There is quite a bit of variability and overlap of these entities leading to difficulties in effective management. This case report and the accompanying review, tries to elucidate the clinical presentation, diagnosis, and treatment strategies in adult patients with hepatic sequestration.