Total Extraction as a Treatment for Anaemia in a Patient of Glanzmann’s Thrombasthenia with Chronic Gingival Bleed: Case Report
Identifieur interne : 000433 ( Main/Exploration ); précédent : 000432; suivant : 000434Total Extraction as a Treatment for Anaemia in a Patient of Glanzmann’s Thrombasthenia with Chronic Gingival Bleed: Case Report
Auteurs : Abhishek Ghosh ; Saurabh Kumar ; Rabin Chacko ; Arun Paul CharluSource :
- Journal of Clinical and Diagnostic Research : JCDR [ 2249-782X ] ; 2016.
Abstract
Glanzmann’s Thrombasthenia (GT) is a rare autosomal recessive bleeding disorder affecting the megakaryocyte lineage and is characterized by lack of platelet aggregation on stimulation. The molecular basis is linked to quantitative and qualitative abnormalities of αIIbβ3 integrin. Most of the patients with severe Glanzmann’s thrombasthenia have spontaneous gum bleeding and persistent low haemoglobin levels. Often these patients are addressed with local haemostatic measures and platelet coverage. We report a case of a severe Glanzmann’s thrombasthenia with chronic gingivitis and associated spontaneous gum bleed with chronic low haemoglobin levels, managed subsequently with total dental extraction under appropriate platelet and recombinant factor VIIa coverage. Further follow up of the patient substantiated the treatment protocol with increased and stable haemoglobin levels, thus emphasizing the need for total dental extraction in patients with severe Glanzmann’s with chronic spontaneous gum bleed, as a definitive treatment option, which has not been reported so far in the literature.
Url:
DOI: 10.7860/JCDR/2016/16383.7123
PubMed: 26894187
PubMed Central: 4740716
Affiliations:
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<front><div type="abstract" xml:lang="en"><p>Glanzmann’s Thrombasthenia (GT) is a rare autosomal recessive bleeding disorder affecting the megakaryocyte lineage and is characterized by lack of platelet aggregation on stimulation. The molecular basis is linked to quantitative and qualitative abnormalities of αIIbβ3 integrin. Most of the patients with severe Glanzmann’s thrombasthenia have spontaneous gum bleeding and persistent low haemoglobin levels. Often these patients are addressed with local haemostatic measures and platelet coverage. We report a case of a severe Glanzmann’s thrombasthenia with chronic gingivitis and associated spontaneous gum bleed with chronic low haemoglobin levels, managed subsequently with total dental extraction under appropriate platelet and recombinant factor VIIa coverage. Further follow up of the patient substantiated the treatment protocol with increased and stable haemoglobin levels, thus emphasizing the need for total dental extraction in patients with severe Glanzmann’s with chronic spontaneous gum bleed, as a definitive treatment option, which has not been reported so far in the literature.</p>
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