PB2412 ADULT THALASSEMIA PATIENTS OF PEDIATRIC HEMATOLOGY CLINIC: A SINGLE CENTER EXPERENCE IN TURKEY

Abstract

Background:Advances in understanding and treatments in transfusion dependent thalassemia (TDT) improved the clinical outcomes. Disease‐related complications seem to be the challenging problem in aging patients with thalassemia who now survive longer than in the past.Aims:Here we aimed to describe clinical features of adult thalassemia patients followed on the pediatric hematology clinic.Methods:All the patients with TDT and older than 25‐years were reviewed retrospectively. Demogrophical, cilinical features, status of iron overload and treatment and disease related complications were recorded.Results:A total of 69 patients were evaluated. Thirty three were female and 36 were male. Mean age was 32.9 years (range 25‐53 years). Mean duration of follow‐up and iron chelation therapy were 31,2 years (range 14‐50 years), and 25 years (range 7‐ 31 years), respectively.Fortyfour patients had splenectomy. Mean ferritin levels was found to be 1645 ng/ml (range between 361‐8600 ng/ml). Cardiac iron status were evaluated in 44 patients and cardiac T2∗was normal (>20 msec) in 40 patients, moderate and severe iron overload was shown in 1 and 3 patients, respectively. 1 patients had moderate. Liver iron status was normal (<2 mg) in 16 out of 53 patients with liver T2∗ MRI. Liver iron overload was found to be mild (2‐5 mg) in 11 patients, moderate (5‐10 mg) in 10 patients and severe (>10 mg) in 16 patients. A strong positive correleation was found between serum ferritin levels and liver iron overload (p < 0.001). There was no significant association between cardiac iron overload and serum ferritin levels. Also a slight association was shown cardiac and liver iron overload (p = 0.03).Summary/Conclusion:In our country the adult TDT patients are still on follow‐up in pediatric hematology clinics based on the experience of the pediatric hematologists on those patients. Although these older patients were constituted a new cohort, in this study the clinical features and status of iron overload in older TDT patients were found to be acceptable and similar to younger TDT's regarding despite long period of regular transfusion and histroical features of the treatment.