Acute splenic sequestration crisis in an adolescent with S-C hemoglobinopathy

Acute splenic sequestration crisis occurs rarely as a complication of sickle hemoglobinopathy. It is a medical emergency requiring immediate restoration of intravascular volume with transfusions of fresh packed RBCs. If the blood of the patient under discussion had not contained strong irregular antibodies, the delay in blood transfusion and the subsequent fatal outcome might have been avoided. Whether young children with sickle hemoglobinopathies should be checked periodically for the presence of irregular antibodies is an unanswered question. Monitoring might be important in view of the small but definite risk during childhood of the development of acute splenic sequestration crisis.