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Thromb Haemost 2006; 96(04): 488-491
DOI: 10.1160/TH06-05-0267
Blood Coagulation, Fibrinolysis and Cellular Haemostasis
Schattauer GmbH

Prevalence of thromboembolic events among 8,860 patients with thalassaemia major and intermedia in the Mediterranean area and Iran

Ali Taher
1   Internal Medicine, American University of Beirut, Beirut, Lebanon
,
Hussain Isma’eel
1   Internal Medicine, American University of Beirut, Beirut, Lebanon
,
Ghassan Mehio
1   Internal Medicine, American University of Beirut, Beirut, Lebanon
,
Daniela Bignamini
2   Centro Anemie Congenite, Ospedale Maggiore Policlinico, IRCCS, Milano, Italy
,
Antonis Kattamis
3   First Department of Paediatrics, University of Athens School of Medicine, Athens, Greece
,
Eliezer A. Rachmilewitz
4   Haematology Department, The E. Wolfson Medical Center, Holon, Israel
,
Maria Domenica Cappellini
2   Centro Anemie Congenite, Ospedale Maggiore Policlinico, IRCCS, Milano, Italy
› Author Affiliations
Further Information

Publication History

Received 15 May 2006

Accepted after resubmission 14 August 2006

Publication Date:
29 November 2017 (online)

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Summary

Beta-thalassaemia isa congenital haemolytic anaemia characterized by partial (intermedia, TI) or complete (major, TM) deficiency in the production of β-globin chains.The primary aim of this study was to determine the prevalence of thromboembolic events in patients with β-thalassaemia.To achieve this,a multiple-choice questionnaire was sent to 56 tertiary referral centres in eight countries (Lebanon, Italy, Israel, Greece, Egypt, Jordan, Saudi Arabia and Iran), requesting specific information on patients who had experienced a thromboembolic event.The study demonstrated that thromboembolic events occurred ina clinically relevant proportion (1.65%) of 8,860 thalassaemia patients (TI – 24.7% or TM – 75.3%) from the Mediterranean and Iran. Thromboembolism occurred 4.38 times more frequently in TI thanTM (p<0.001), with more venous events occurring inTI and more arterial events occurring in TM.Thrombosis in thalassaemia was also more common in females, splenectomized patients and those with profound anaemia (haemoglobin <9 g/dl). Due to the increased risk of thromboembolic events, the rationale for splenectomy should perhaps be re-assessed and the role of transfusion therapy for the prophylaxis of thrombosis, among other complications, be evaluated prospectively.

Keywords

Thrombosis - thalassaemia intermedia - thalassaemia major
 

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