Glanzmann's thrombasthenia (GT) is a rare, genetically inherited platelet disorder in which the platelet glycoprotein IIb/IIIa (GP IIb/IIIa) complex is either deficient or, dysfunctional. The incidence is about 1 in 1,000,000. This case report deals with a 4 year-old girl diagnosed with GT presenting with dental caries and periapical lesions in the primary mandibular first molars. To provide the best care, an interdisciplinary approach was followed by a team consisting of pediatric dentists, pediatricians and anesthesiologists. Complete oral rehabilitation was planned under general anesthesia which included extractions, multiple esthetic restorations and space maintainers with the utmost care to prevent unwarranted bleeding.
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Hamostaseologie
January 2025
Center for Thrombosis and Hemostasis, University Medical Center Mainz, Mainz, Germany.
Congenital platelet disorders are rare and targeted treatment is usually not possible. Inherited platelet function disorders (iPFDs) can affect surface receptors and multiple platelet responses such as defects of platelet granules, signal transduction, and procoagulant activity. If iPFDs are also associated with a reduced platelet count (thrombocytopenia), it is not uncommon to be misdiagnosed as immune thrombocytopenia.
View Article and Find Full Text PDFCureus
December 2024
Obstetrics and Gynecology, Latifa Hospital, Dubai, ARE.
Glanzmann thrombasthenia (GT) is an autosomal recessive platelet functional bleeding disorder caused by mutations in the ITGA2B or ITGB3 genes, often presenting as mucocutaneous bleeding. GT typically presents in infancy, but this study reports a rare case of neonatal presentation in a female infant born to consanguineous parents. The mother, a 27-year-old woman with a family history of GT, presented at 36 weeks gestation for an elective cesarean due to a breech presentation.
View Article and Find Full Text PDFJ Family Med Prim Care
November 2024
Department of Basic Science College of Medicine, Princess Nourah bint Abdulrahman University, Riyadh, Saudi Arabia.
Background: Glanzmann thrombasthenia (GT) is a rare disease with an autosomal recessive inheritance pattern. This disorder is not so uncommonly encountered in routine clinical practice and laboratory settings in Pakistan let alone in the rest of the world. To describe the bleeding phenotype of GT and treatment outcomes in over one hundred patients in north Pakistan.
View Article and Find Full Text PDFRes Pract Thromb Haemost
November 2024
University of Strasbourg, Faculty of Dental Surgery, Strasbourg, France.
Background: Glanzmann thrombasthenia (GT) is a very rare autosomal inherited bleeding disease affecting megakaryocyte lineage with impacts on oral health such as gingival bleeding, which requires specific management protocols. Very few clinical cases have been published in the dental and hematologic literature.
Objectives: This study focuses on a series of 21 patients affected specifically by GT and their hemorrhagic prophylaxis management with the use of recombinant activated factor VII (rFVIIa) for dental extractions and full-mouth debridement.
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