Relative risk of death associated with different disease states
| Disease state . | Reference group . | Relative risk . |
|---|---|---|
| BMT success | Healthy black persons | 2.0 |
| BMT success, cGVHD | Healthy black persons | 2.3 |
| BMT rejection | Sickle cell patients | 2.0 |
| PBT healthy | Sickle cell patients | 0.43-150 |
| PBT noncompliant w/chelating therapy | Sickle cell patients | 1.0-8.03-151 |
| PBT noncompliant w/transfusion regimen | Sickle cell patients | 0.83-150 |
| PBT alloimmunized | Sickle cell patients | 0.83-150 |
| Disease state . | Reference group . | Relative risk . |
|---|---|---|
| BMT success | Healthy black persons | 2.0 |
| BMT success, cGVHD | Healthy black persons | 2.3 |
| BMT rejection | Sickle cell patients | 2.0 |
| PBT healthy | Sickle cell patients | 0.43-150 |
| PBT noncompliant w/chelating therapy | Sickle cell patients | 1.0-8.03-151 |
| PBT noncompliant w/transfusion regimen | Sickle cell patients | 0.83-150 |
| PBT alloimmunized | Sickle cell patients | 0.83-150 |
BMT = bone marrow transplantation; cGVHD = chronic graft versus host disease; PBT = periodic blood transfusion.
Because the only abnormal symptom of the patients in question is an abnormal transcranial Doppler, we may assume that these patients would have had a better chance of survival than typical sickle cell patients. In addition, according to data reported by Styles and Vichinsky,7 patients who were routinely transfused were hospitalized 10 times less often for vaso-occlusive crises and more than 15 times less often for acute chest syndrome than in previous years when they had not been routinely transfused. We can only hypothesize that the transfused patient will have an additional benefit with respect to survival.
Risk of death increases as iron levels increase, and decreases slowly as the physician intervenes to reduce iron load. The magnitude of the elevated risk of death was obtained from Olivieri et al.21