title: Longitudinal study of survival and causes of death in patients with thalassemia major in Greece.
abstract: Iron-induced organ degeneration is the main factor of mortality in patients with thalassemia major. Since chelation therapy is at a turning point, from the laborious parenteral route to the use of new promising oral agents, we investigated the current status of survival of these patients to present reliable data that will be useful in future comparative studies. Survival probabilities were estimated by the Kaplan-Meier method, and results were compared by the log-rank test in a total of 647 thalassemic patients (pts) (52% males) born between 1/1/58 and 1/2/04. Terminal follow-up was 1/12/04. All transfusion-dependent pts monitored in our center, or in frequent contact if they had moved elsewhere, were strictly selected, excluding all rarely transfused or intermediate cases. Pts born before 1/1/75 were classified in group A (n = 366), while pts born later were included in group B (n = 281). According to the last 5 years' mean serum ferritin level, pts were divided into three hemosiderosis groups: (1) mild (<2000 microg/L) 49%, (2) moderate (2000-4000 microg/L) 28%, and (3) severe (>4000 microg/L) 23%. Of the 647 pts, 115 died (mean age: 22.6 +/- 6.2 years), most frequently by heart failure (71.3%) followed by sepsis (7.8%). Life expectancy in the entire population was up to 59% at 46 years. Survival was higher for pts born after 1975 than those before (P < .001). Statistically significantly different survival probabilities were found between groups with mild, moderate, or severe hemosiderosis (P < .001). Effective management with improved chelation therapy could lead to better results.
authors.affiliation: Thalassemia Unit, First Department of Pediatrics, University of Athens, Athens, Greece. thalas@paidon-agiasofia.gr
authors.forename: Vassilis
authors.initials: V
authors.lastname: Ladis
authors.forename: George
authors.initials: G
authors.lastname: Chouliaras
authors.forename: Helen
authors.initials: H
authors.lastname: Berdousi
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Kanavakis
authors.forename: Christos
authors.initials: C
authors.lastname: Kattamis
mesh_terms: D000163:Acquired Immunodeficiency Syndrome / Q000401:mortality; D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001803:Blood Transfusion; D016026:Bone Marrow Transplantation / Q000401:mortality; D002423:Cause of Death; D015913:Chelation Therapy; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003131:Combined Modality Therapy; D005260:Female; D005293:Ferritins / Q000097:blood; D006115:Greece / Q000453:epidemiology; D006333:Heart Failure / Q000401:mortality; D006486:Hemosiderosis / Q000209:etiology / Q000401:mortality; D006801:Humans; D008017:Life Expectancy; D015993:Life Tables; D008107:Liver Diseases / Q000401:mortality; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D018805:Sepsis / Q000401:mortality; D016019:Survival Analysis; D013923:Thromboembolism / Q000401:mortality; D017086:beta-Thalassemia / Q000188:drug therapy / Q000453:epidemiology* / Q000401:mortality / Q000601:surgery / Q000628:therapy
chemical_list: D005293:Ferritins