Figure 1. Caucasian sickle cell disease (SCD) patients. (A) Causes of death (n = 54 patients). Acute Chest Syndrome (ACS). (B) Survival curves by genotype
Summary/Conclusion:The Italian SCD population is characterized by a cohort of elderly Caucasians with a long-term follow up (median follow up 37 years; 95%CI: 36-39 years, calculated by the "reverse Kaplan-Meier'' technique) and a younger cohort of patients of African descent. The major finding of our study is that genotype or splenectomy does not affect the overall survival of SCD patients. ACS was the first cause of death; unexpectedly, liver failure was the second cause of death in elderly SCD patients, most likely due to chronic sickle cell-related liver damage. The analysis of others factors triggering chronic hepatopathy is under evaluation in this large population of elderly SCD subjects.
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