29 Dec Glanzmann thrombasthenia is a genetic platelet disorder in which the platelet have qualitative or quantitative deficiencies of the fibrinogen. Hematology-dental integrated management in Glanzmann thrombasthenia. Acta Med Colomb [online]. , vol, n.1, pp ISSN PDF | CASO CLÍNICO: paciente de 29 años de edad, primigrávida, con diagnóstico de trombastenia de Glanzmann establecido en la niñez. La finalización del.
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Glanzmann thrombasthenia GT is a rare autosomal recessive bleeding syndrome affecting the megakaryocyte lineage and characterized by lack of platelet. Treatment can depend on what type of bleed you may have or what type of procedure you may be undergoing. For all other comments, please send your remarks via contact us. This receptor glanzmann activated when the platelet is stimulated by ADP, epinephrinecollagen, or thrombin.
This test is highly specific for GT, glznzmann platelet aggregation fails to occur with any agonist, except ristocetin, where the reaction is preserved.
Avoid anti-inflammatory drugs e. Some of the reasons that platelets do not work as they should are: A systematic review of management and outcomes of 35 pregnant women with GT revealed that hemorrhage during or after delivery trombastenia de glanzmann common and severe, and occurred up to 20 days postpartum. This manuscript offers a comprehensive review of our understanding of GT and the available treatment options.
Administration of recombinant factor VIIa is an increasingly used therapeutic alternative. Regular dental care is essential to prevent gingival bleeding. Diagnosis Most individuals affected with Glanzmann thrombasthenia have a normal number of platelets but have a prolonged bleeding time, which means it takes longer than usual for a standardized cut to stop bleeding.
See Clinical Presentation for more detail.
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Received tgombastenia from Medscape for employment. Braunsteiner H, Pakesch F. Refractoriness At some point in time, platelet transfusions may not work as well as expected or at all in treating a bleeding episode or preventing bleeding during a procedure. Platelets are derived from one or more blood donors, and as such have a very low but possible risk of transmitting infectious agents e.
She underwent anterior-posterior nasal packing with no more bleeding after the procedure. Ann Pediatr Paris ; 18 2: When these two signs are associated with a normal platelet count and morphology, the diagnosis of GT is clear-cut. Pregnancy and in particular, delivery, represent a particularly severe hemorrhagic risk. Pathophysiology of Haemostasis and Thrombosis.
Each glycoprotein has a specific target, either another platelet or a target in the vessel wall, and a specific role to play in forming the platelet plug.
Open in a separate window. There may a slightly higher female preponderance for Glanzmann thrombasthenia. Identification of 19 new mutations in 30 patients. Two of the 59 patients were given a high dose of rFVIIa trombastenis a continuous infusion, in combination with an anti-fibrinolytic agent, during surgical procedures and experienced thromboembolic events: Bleeding problems can glannzmann severe in patients with Glanzmann thrombasthenia, but the prognosis remains good with appropriate supportive care.
Eur J Hum Genet.
The full terms of the License are available at http: Often even seemingly simple bleeds like nose bleeds or monthly periods may result in significant blood loss or iron deficiency and become life-threatening. Partner received none from No financial interests for none.
Missense mutations have been further studied, and display interruption in integrin maturation or subunit formation. Management algorithm for major bleeding episode or urgent trombasgenia procedure. Acquired Glanzmann’s thrombasthenia associated with Hodgkin’s lymphoma: Deletions and insertions, nonsense and missense mutations are common causes of GT. Additionally, she had a history of bleeding after minimal trauma and gingivorrhagia. Alan T Nurden 1.
If HLA-matched platelets are not available, patients should receive leukocyte-reduced platelets, as this has been shown to reduce the rate of HLA immunization. Acquired Glanzmann’s thrombasthenia without thrombocytopenia: Iron replacement therapy may be recommended.
Int Trombastfnia Gen Med.