The Glanzmann’s Thrombasthenia in Tunisia: A Cohort Study

Hejer Elmahmoudi, Meriem Achour, Nejla Belhedi, Hend Ben Neji, Kaouther Zahra, Balkis Meddeb, Emna Gouider

Abstract


Background: The Glanzmann’s thrombasthenia (GT) is a rare autosomal-recessive bleeding disorder with uncommon neonatal revelation. It is due to abnormalities of quantitative and/or qualitative alph IIb beta3 integrin. This cell adhesion receptor is essential for platelet aggregation and allows the formation of a hemostatic plug if the vessel is damaged by injury. The clinical picture of GT is variable, with mucocutaneous bleeding due to non-functional platelets. Management requires a good expertise in bleeding disorders. We describe the clinical and the epidemiological data of GT in Aziza Othmana Hospital Hemophilia Center.

Methods: This was a retrospective study of all patients with GT monitored and treated in our hemophilia center during the period of 2011 - 2015.

Results: Twenty-seven patients among the 35 patients included in our hemophilia center registry were studied. The most common sign encountered is the gingival bleeding. In our women cohort, one completed her pregnancy. The consanguinity is present with a frequency of 62%. Treatments used depending on the case are tranexamic acid, platelet transfusion, packed red blood cells and rFVIIa, respectively.

Conclusion: GT is relatively frequent in Tunisia and especially in the North of the country which can be explained by the high consanguinity in our population.




J Hematol. 2017;6(2-3):44-48
doi: https://doi.org/10.14740/jh330e


Keywords


Glanzmann’s thrombasthenia; Tunisia; Bleeding disorders; Consanguinity; Flow cytometry; Platelet aggregation

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