Document Type : Original Article(s)
Authors
1
Assistant Professor, Pediatric Hematology, School of Medicine, Isfahan University of Medical Sciences
2
Professor, Pediatric Cardiology, School of Medicine, Isfahan University of Medical Sciences
3
Pediatrician, Al-Zahra Hospital, Isfahan University of Medical Sciences
Abstract
Background:Hypercoagulopathy is one of the complications of thalassemia; high incidence of right sided heart failure (due to pulmonary hypertension) in some surveys indicates on the importance of this complication. In this research we evaluated a frequency of protein C and S, and antithrombin-III (AT-III) deficiency in thalassemia major patients in Isfahan.Methods:Serum level of protein C and S, and AT-III were measured in 41 thalassemia major patients (age 7-20 years), and the relation between serum level and age, sex, splenectomy, transfusion intervals, pre transfusion Hb, and serum ferritin level was detected. All of these patients were also evaluated for cardiac disease and pulmonary hypertension by doppler echocardiography.Findings:Overall, 43% of cases had protein C deficiency, 53% protein S deficiency, and 30% AT-III deficiency; 82% of all patients had at least one factor deficiency. The frequency of protein C, S and AT-III deficiency was higher in patient older than 10 years, with transfusion interval more than 21 days, and with less pre-transfusion Hb level. High ferritin level was also significantly related to protein C and S deficiency (p < 0.05). With echocardiography, dilated heart was found in 8 (11.8% in non splenectomized, and 57% in splenectomized) (p < 0.001); only 2 patients had evidences of pulmonary hypertension.Conclusion:Also hypercoagulopathy due to protein S, protein C and AT-III deficiency is common, but unlike some researches, we didn't find high incidence of pulmonary hypertension in our patients. According to the high frequency of factor deficiency, more attention for hypercoagulable state is required in thalassemia major patients with risk factor.Key words:Thalassemia major, hypercoagulopathy, pulmonary hypertension
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