Document Detail


Screening for inherited thrombophilia might be warranted among Eastern Mediterranean sickle-beta-0 thalassemia patients.
MedLine Citation:
PMID:  17008978     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: The role of genetic thrombophilia in the development of both micro and macro vascular complications in patients with hemoglobinopathies (Sickle cell disease and thalassemia) have been investigated with some studies negating its role while others suggesting it. Lebanon is known to harbor sickle cell disease, thalassemia and sickle beta-thalassemia hemoglobinopathy patients along with a documented high prevalence of genetic thrombophilia mutations. METHODS: Twelve sickle beta-0-thalassemia patients with no pervious history of thrombotic events were selected. These patients underwent a physical examination with history, echo Doppler, along with blood withdrawal for complete blood count and PCR analysis of a sample of DNA for Factor V Leiden G1691A, Factor II G20210A, and MTHFR C677T. Results were compared to a historical control of 50 Lebanese controls and 50 LebaneseThalassemia Intermedia (TI) patients. RESULTS: The results showed that 42%, 59%, and 8% of patients carried heterozygous Factor V Leiden, abnormal (homozygous & heterozygous) MTHFR, and heterozygous Factor II mutations respectively. The sickle-thalassemia patients were 5.24 and 4.39 times more likely to have Factor V Leiden as compared to the normal controls and TI patients respectively (p < 0.05). DISCUSSION: The increased prevalence of more than one prothrombotic genetic mutation among the group indicates a probable clustering phenomenon, unknown to us to which the high consanguinity rate (77%) may have contributed. The role of the specific MTHFR and Factor V Leiden double heterozygous combination in incidence, recurrence, and guidance of duration of therapy in VTE is not well defined in the literature despite the recognized higher risk of thrombosis among this patient population. Our findings suggest that genetic thrombophilia workup is necessary in patients with sickle-beta zero thalassemia presenting with thrombotic events and studies that include a larger number of patients are necessary in order to define specific guidelines.
Authors:
Hussain Isma'eel; M S Arnaout; Wael Shamseddeen; R Mahfouz; N Zeineh; Oussama Jradi; Ali Taher
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of thrombosis and thrombolysis     Volume:  22     ISSN:  0929-5305     ISO Abbreviation:  J. Thromb. Thrombolysis     Publication Date:  2006 Oct 
Date Detail:
Created Date:  2006-09-29     Completed Date:  2006-12-21     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  9502018     Medline TA:  J Thromb Thrombolysis     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  121-3     Citation Subset:  IM    
Affiliation:
American University of Beirut Medical Center.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Anemia, Sickle Cell / blood,  complications*,  genetics
Case-Control Studies
Child
Consanguinity
DNA Mutational Analysis
Echocardiography, Doppler
Factor V / genetics
Female
Genetic Testing*
Humans
Lebanon / epidemiology
Male
Methylenetetrahydrofolate Reductase (NADPH2) / genetics
Polymorphism, Genetic
Prothrombin / genetics
Thrombophilia / complications,  genetics*
beta-Thalassemia / blood,  complications*,  genetics
Chemical
Reg. No./Substance:
0/factor V Leiden; 9001-24-5/Factor V; 9001-26-7/Prothrombin; EC 1.5.1.20/Methylenetetrahydrofolate Reductase (NADPH2)

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