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Glanzmann thrombasthenia: a rare hematological disorder with oral manifestations: a case report.

The journal of contemporary dental practice, Vol. 9, No. 5. (2008), pp. 107-113.


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AIM: The aim of this report is to present a case of Glanzmann thrombasthenia (GT) with oral manifestations requiring periodontal management along with a discussion of the clinical, hematologic, and molecular level features of the disease. BACKGROUND: GT is a rare hematological disorder with oral manifestations affecting platelets and clotting. It is characterized by spontaneous bleeding from mucosal tissues and excessive bleeding following skin damage. It belongs to the group of hereditary platelet disorders and is due to a defect in one of two genes, platelet membrane glycoprotein (GP) IIb/IIIa. REPORT: A 22-year-old female patient with a history of Type I GT and long-term care using platelet transfusion was referred for management of her gingival bleeding. Dental treatment included scaling, polishing, oral hygiene instructions, along with a prescription for anti-plaque agents. There was pronounced improvement in her periodontal condition after treatment. Reduced gingival bleeding resulted in an increase in her hemoglobin level from a pre-treatment level of 2 gm% to 8 gm% during her last visit. The patient was followed for eight months with no further periodontal complications. SUMMARY: A pronounced improvement in the periodontal condition of a GT patient occurred following routine scaling and polishing procedures along with proper oral hygiene maintenance instructions. The result was a reduction of the patient's gingivitis and associated spontaneous bleeding and an improvement in her hemoglobin status. GT patients should be managed for their periodontal problems following a hematological consultation. The importance of proper maintenance of oral hygiene as well as regular recall visits should be emphasized.


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