期刊
AMERICAN JOURNAL OF HEMATOLOGY
卷 82, 期 4, 页码 255-265出版社
WILEY
DOI: 10.1002/ajh.20809
关键词
sickle cell disease (SCD); thalassemia (Thal); iron overload; hospitalization; morbidity; mortality
类别
资金
- NCRR NIH HHS [M01 RR01271, M01 RR00211, K12 RR017613] Funding Source: Medline
- NIDDK NIH HHS [R01 DK057778] Funding Source: Medline
A natural history study was conducted in 142 Thalassemic (Thai), 199 transfused Sickle Cell Disease (Tx-SCD, n = 199), and 64 non-Tx-SCD subjects to describe the frequency of iron-related morbidity and mortality. Subjects recruited from 31 centers in the US, Canada or the UK were similar with respect to age (overall: 25 +/- 11 years, mean SD) and gender (52% female). We found that Tx-SCD subjects were hospitalized more frequently compared with Thai or non-Tx-SCD (P < 0.001). Among those hospitalized, Tx-SCD adult subjects were more likely to be unemployed compared with Thal (RR = 1.6, 95% Cl 1.0-2.5) or non-Tx-SCD (RR = 3.1, 95% Cl 1.3-7.3). There was a positive relationship between the severity of iron overload, assessed by serum ferritin, and the frequency of hospitalizations (r = 0.20; P = 0.009). Twenty-three deaths were reported (6 Thal, 17 Tx-SCD) in 23.5 +/- 10 months of follow-up. Within the Tx-SCD group, those who died began transfusion (25.3 vs. 12.4 years, P < 0.001) and chelation therapy later (26.8 vs. 14.2 years, P = 0.01) compared with those who survived. The unadjusted death rate in Thal was lower (2.2/100 person years) compared with that in Tx-SCD (7.0/100 person years; RR = 0.38: 95% Cl 0.12-0.99). However, no difference was observed when age at death was considered. Despite improvements in therapy, death rate in this contemporary sample of transfused adult subjects with Thal or SCD is 3 times greater than the general US population. Long term follow-up of this unique cohort of subjects will be helpful in further defining the relationship of chronic, heavy iron overload to morbidity and mortality.
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