An update on sickle cell disease

BACKGROUND- Neurological complications, especially stroke, are important causes of morbidity and mortality in sickle cell disease (SCD). The neurologist is often called in consultation. REVIEW SUMMARY- Recent new information has better established the epidemiology of stroke in SCD and demonstrated that high incidence rates are seen in early childhood and later in life as well. Secondary prevention of recurrent stroke in children is accomplished with chronic transfusion, and there is little experience with other preventive treatments. Primary prevention is now feasible for children using screening with transcranial Doppler. There are data from a randomized controlled clinical trial that indicates that children with high velocities on transcranial Doppler (>200 cm/sec time averaged mean in the middle cerebral or intracranial internal carotid arteries) have a very significant reduction in stroke with chronic transfusion. Hydroxyurea and bone marrow transplantation may in the future be used for stroke prevention, but there are no data at present. There is no primary stroke prevention strategy for adults and prevention of recurrent stroke has not been well studied. Transfusion is an option, as well as empiric use of antiplatelet agents, warfarin, or surgery, but there are virtually no data on risk/benefit for these treatments in adults. All SCD patients with intracranial hemorrhage should be considered for angiography to look for surgically correctable lesions. CONCLUSION- The neurologist should bear in mind that, although stroke is common in SCD, other etiologies and causes for neurological dysfunction may be present and should be considered in patients with SCD who present with neurological symptoms.