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Total Extraction as a Treatment for Anaemia in a Patient of Glanzmann’s Thrombasthenia with Chronic Gingival Bleed: Case Report

Identifieur interne : 000385 ( Pmc/Checkpoint ); précédent : 000384; suivant : 000386

Total 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 Charlu

Source :

RBID : PMC:4740716

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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PMC:4740716

Le document en format XML

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<nlm:aff id="aff2"> Assistant Professor, Department of Oral and Maxillofaical Surgery,
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<nlm:aff id="aff4"> Associate Professor, Department of Oral and Maxillofacial Surgery,
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,
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<title level="j">Journal of Clinical and Diagnostic Research : JCDR</title>
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<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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<journal-id journal-id-type="iso-abbrev">J Clin Diagn Res</journal-id>
<journal-id journal-id-type="hwp">Journal of Clinical and Diagnostic Research</journal-id>
<journal-id journal-id-type="publisher-id">JCDR</journal-id>
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Fellow, Head and Neck Oncology,
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Assistant Professor, Department of Oral and Maxillofaical Surgery,
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<addr-line>Vellore, Tamil Nadu, India</addr-line>
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Head of Department Unit I, Department of Oral and Maxillofacial Surgery,
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,
<addr-line>Vellore, Tamil Nadu, India</addr-line>
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Associate Professor, Department of Oral and Maxillofacial Surgery,
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<author-notes>
<corresp id="cor1">NAME, ADDRESS, E-MAIL ID OF THE CORRESPONDING AUTHOR: Dr. Abhishek Ghosh, HCG Cancer Centre Sola-Science City Road, Off S.G. Highway, Sola, Ahmedabad, Gujarat-380060, India. E-mail :
<email>13abhishekghosh@gmail.com</email>
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<month>1</month>
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<volume>10</volume>
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<fpage>ZD11</fpage>
<lpage>ZD12</lpage>
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<date date-type="received">
<day>21</day>
<month>8</month>
<year>2015</year>
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<date date-type="rev-request">
<day>10</day>
<month>10</month>
<year>2015</year>
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<date date-type="accepted">
<day>02</day>
<month>11</month>
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<copyright-statement>© 2016 Journal of Clinical and Diagnostic Research</copyright-statement>
<copyright-year>2016</copyright-year>
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<abstract>
<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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<kwd>Total extraction</kwd>
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<name sortKey="Kumar, Saurabh" sort="Kumar, Saurabh" uniqKey="Kumar S" first="Saurabh" last="Kumar">Saurabh Kumar</name>
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