Glanzmann's Thrombasthenia: How Listening to the Patient Is Sometimes the Simple Key to Good Medicine!

Case Reports in Medicine
Thejus T JayakrishnanPrerna Mewawalla

Abstract

Introduction. Glanzmann's thrombasthenia is a rare clotting disorder caused by impaired platelet function. Lack of awareness of the appropriate management of rare medical conditions may lead to patient dissatisfaction and potentially poor treatment outcome. Case Report. A 78-year-old male with a history of Glanzmann's thrombasthenia was admitted to the trauma service following a fall in which he sustained a facial laceration as well as maxillary sinus and nasal fractures. He received DDAVP 20 mcg and tranexamic acid upon presentation to the emergency department (ED). In the ED, the patient requested administration of platelet transfusion but was refused due to a normal platelet count. During the course of his hospital stay, he complained of epistaxis and was noted to have a downtrending hemoglobin from 11.0 g/dl to 9.0 g/dl. The patient and his family were not comfortable when the discharge plan was finalized and demanded platelet transfusion (due to history of needing platelets in association with injuries or procedures in the past) was refused by the primary team as they continued to state that his platelet count is normal. On hospital day 3, hematology was consulted as the patient and his family were extremely angry and hema...Continue Reading

References

May 26, 2006·Orphanet Journal of Rare Diseases·Alan T Nurden
Jul 16, 2010·Journal of Thrombosis and Haemostasis : JTH·F RodeghieroUNKNOWN ISTH/SSC joint VWF and Perinatal/Pediatric Hemostasis Subcommittees Working Group
May 24, 2015·Haematologica·Man-Chiu PoonUNKNOWN Glanzmann Thrombasthenia Registry Investigators
Mar 13, 2016·Transfusion Medicine Reviews·Man-Chiu PoonRainer Zotz
Jul 23, 2018·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·Adrienne Lee, Man-Chiu Poon
Jul 29, 2018·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·Margaret L RandSara J Israels
Dec 16, 2018·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·Dearbhla DohertyMichelle Lavin
Feb 14, 2019·European Journal of Case Reports in Internal Medicine·Raquel MesquitaHelena Monteiro

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Methods Mentioned

BETA
coronary artery bypass
genotyping
flow cytometry

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