| Literature DB >> 26894187 |
Abhishek Ghosh1, Saurabh Kumar2, Rabin Chacko3, Arun Paul Charlu4.
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.Entities:
Keywords: Platelet disorder; Recombinant factor VIIa; Spontaneous gingival hemorrhage; Total extraction; αIIbβ3 deficiency
Year: 2016 PMID: 26894187 PMCID: PMC4740716 DOI: 10.7860/JCDR/2016/16383.7123
Source DB: PubMed Journal: J Clin Diagn Res ISSN: 0973-709X