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A Rare Case of Bleeding Disorder: Glanzmann's Thrombasthenia

BACKGROUND: Glanzmann's thrombasthenia (GT) is a rare bleeding disorder, which is characterized by a lack of platelet aggregation. It is characterized by qualitative or quantitative abnormalities of the platelet membrane glycoprotein IIb/IIIa. Physiologically, this platelet receptor normally bi...

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Autores principales: Swathi, Jami, Gowrishankar, A., Jayakumar, S. A., Jain, Karun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5676411/
https://www.ncbi.nlm.nih.gov/pubmed/29063905
http://dx.doi.org/10.4103/aam.aam_59_16
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author Swathi, Jami
Gowrishankar, A.
Jayakumar, S. A.
Jain, Karun
author_facet Swathi, Jami
Gowrishankar, A.
Jayakumar, S. A.
Jain, Karun
author_sort Swathi, Jami
collection PubMed
description BACKGROUND: Glanzmann's thrombasthenia (GT) is a rare bleeding disorder, which is characterized by a lack of platelet aggregation. It is characterized by qualitative or quantitative abnormalities of the platelet membrane glycoprotein IIb/IIIa. Physiologically, this platelet receptor normally binds several adhesive plasma proteins, and this facilitates attachment and aggregation of platelets to ensure thrombus formation at sites of vascular injury. The lack of resultant platelet aggregation in GT leads to mucocutaneous bleeding whose manifestation may be clinically variable, ranging from easy bruising to severe and potentially life-threatening hemorrhages. OBJECTIVE: To highlight this rare but potentially life-threating disorder, GT. CASE REPORT: We report a case of GT that was first detected because of the multiple episodes of gum bleeding. The patient was an 18-year-old girl who presented with a history of repeated episodes of gum bleeding since childhood. Till the first visit to our hospital, she had not been diagnosed with GT despite a history of bleeding tendency, notably purpura in areas of easy bruising, gum bleeding, and prolonged bleeding time after abrasions and insect stings. GT was diagnosed on the basis of prolonged bleeding time, lack of platelet aggregation with adenosine di phosphate, epinephrine and collagen. CONCLUSION: GT should always be considered as differential diagnosis while evaluating any case of bleeding disorder.
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spelling pubmed-56764112017-11-17 A Rare Case of Bleeding Disorder: Glanzmann's Thrombasthenia Swathi, Jami Gowrishankar, A. Jayakumar, S. A. Jain, Karun Ann Afr Med Case Report BACKGROUND: Glanzmann's thrombasthenia (GT) is a rare bleeding disorder, which is characterized by a lack of platelet aggregation. It is characterized by qualitative or quantitative abnormalities of the platelet membrane glycoprotein IIb/IIIa. Physiologically, this platelet receptor normally binds several adhesive plasma proteins, and this facilitates attachment and aggregation of platelets to ensure thrombus formation at sites of vascular injury. The lack of resultant platelet aggregation in GT leads to mucocutaneous bleeding whose manifestation may be clinically variable, ranging from easy bruising to severe and potentially life-threatening hemorrhages. OBJECTIVE: To highlight this rare but potentially life-threating disorder, GT. CASE REPORT: We report a case of GT that was first detected because of the multiple episodes of gum bleeding. The patient was an 18-year-old girl who presented with a history of repeated episodes of gum bleeding since childhood. Till the first visit to our hospital, she had not been diagnosed with GT despite a history of bleeding tendency, notably purpura in areas of easy bruising, gum bleeding, and prolonged bleeding time after abrasions and insect stings. GT was diagnosed on the basis of prolonged bleeding time, lack of platelet aggregation with adenosine di phosphate, epinephrine and collagen. CONCLUSION: GT should always be considered as differential diagnosis while evaluating any case of bleeding disorder. Medknow Publications & Media Pvt Ltd 2017 /pmc/articles/PMC5676411/ /pubmed/29063905 http://dx.doi.org/10.4103/aam.aam_59_16 Text en Copyright: © 2017 Annals of African Medicine http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
spellingShingle Case Report
Swathi, Jami
Gowrishankar, A.
Jayakumar, S. A.
Jain, Karun
A Rare Case of Bleeding Disorder: Glanzmann's Thrombasthenia
title A Rare Case of Bleeding Disorder: Glanzmann's Thrombasthenia
title_full A Rare Case of Bleeding Disorder: Glanzmann's Thrombasthenia
title_fullStr A Rare Case of Bleeding Disorder: Glanzmann's Thrombasthenia
title_full_unstemmed A Rare Case of Bleeding Disorder: Glanzmann's Thrombasthenia
title_short A Rare Case of Bleeding Disorder: Glanzmann's Thrombasthenia
title_sort rare case of bleeding disorder: glanzmann's thrombasthenia
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5676411/
https://www.ncbi.nlm.nih.gov/pubmed/29063905
http://dx.doi.org/10.4103/aam.aam_59_16
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