Open AccessPB2397 THROMBOEMBOLIC EVENTS IN THALASSEMIA: 20‐YEAR EXPERIENCE OF A GREEK THALASSEMIA CENTER
Author(s) -
Kyriakaki S.,
Pyrovolaki K.,
Klironomos E.,
Sfyridaki K.
Publication year - 2019
Publication title -
hemasphere
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 11
ISSN - 2572-9241
DOI - 10.1097/01.hs9.0000568052.64959.46
Subject(s) - medicine , thalassemia , thrombosis , pulmonary embolism , thrombophilia , splenectomy , protein c deficiency , thromboembolic stroke , splenic infarction , pediatrics , venous thrombosis , atrial fibrillation , spleen
Background: Thalassemia is a congenital hemolytic anemia. As standards of medical care have improved in recent years, life expectancy has increased significantly and new complications are becoming evident, such as thromboembolic events. Aims: To estimate the prevalence of thromboembolic events in thalassemia patients at our Thalassemia Center and to determine the frequency of thrombophilic disorders in this population of patients. Methods: We prospectively collected data for all thromboembolic events during the last 20 year. Thrombophilia workup was carried out in 57 of the 91 thalassemia patients. The statistical analyses were performed using the test x 2 . Results: Data were obtained from 91 patients, 70 patients with thalassemia major (TM) and 21 patients with thalassemia intermedia (TI). Of the 70 patients with TM, 5.7% (4/70) and of the 21 patients with TI, 4.7% (1/21) expressed thromboembolic event. Thrombotic events included: pulmonary embolism and stroke in a patient, priapism in two patients, acute myocardial infarction in a patient and superficial thrombophlebitis in a patient. Most patients who had a thromboembolic event were splenectomized (80%). The median time for expression of thrombosis after splenectomy was 17.4 years. Clotting factors X, II, XI, V, VII, and coagulation inhibitors (protein S, protein C, antithrombin 3)were often decreased while 60% of patients with thrombosis were found to have thrombosis predisposing genetic factors. Summary/Conclusion: We observed a clinically relevant prevalence of thromboembolic events (5.5%). Splenectomy is an important risk factor for thromboembolic events. Several clotting factors and coagulation inhibitors are depleted. The prevalence of genetic factors predisposing to thrombosis is similar in thalassemic and non‐thalassemic populations.