Clinical management of adult sickle-cell disease

This review provides an overview of the clinical management of sickle-cell disease (SCD) with recently published findings.

Unfortunately, negative observations did not confirm the hope that therapies acting on nitric oxide-cyclic GMP signaling, NSAIDs or Gardos channel inhibitor would control SCD vaso-occlusive crises. The safety of hydroxycarbamide was further supported by two observational studies covering 20 years and over 2 years in young children. Concerning the management of chronic visceral complications of SCD, the STOP II trial showed the risk of discontinuing blood exchange transfusion for children with transcranial Doppler-assessed accelerated blood-flow velocities. The French multicenter Etendard study found that only 25% of SCD patients with tricuspid regurgitation velocity (TRV) 2.5 m/s or more on echocardiograms had catheterization-confirmed pulmonary hypertension. However, elevated TRV, regardless of its cause, was associated with higher mortality. Finally, recent results identified new therapeutic strategies for the treatment and prevention of renal dysfunction, priapism and skin ulcers, but prospective studies are needed to confirm those findings.

SCD treatment relies on concomitant preventive and curative measures to control its acute and chronic manifestations. Pathophysiologic advances have enabled better management, with new therapeutics highly likely in the near future.