Will developments in allogeneic transplantation influence treatment of adult patients with sickle cell disease?
Chakrabarti S, Bareford D. Biol Blood Marrow Transplant. 2004;10:23-31. This review points out that patients with sickle cell disease have a 25 to 30 year reduction in life expectancy compared with the general black population. The mortality increases sharply every decade after 20 years of age. Further, the clinical course often worsens in adult hood, even if symptoms were mild and organ functions were optimum in childhood. There has been little progress in the treatment of these patients and the only curative option is allogeneic stem cell transplantation. More than 160 patients with sickle cell disease have undergone transplantation, almost exclusively in patients younger than 16 years old (with matched sibling donors) who have severe manifestations of the disorder. Even though the more severely affected patients underwent allogeneic transplantation, the overall survival was 90 to 94% and the disease-free survival varied between 82 and 84%. Although the review emphasizes management o
