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Tightrope walking with Glanzmann’s disease Cover
By: Greta Mulders and  Johan Boender  
Open Access
|Mar 2018

Abstract

Glanzmann’s disease or thrombasthenia is a very rare genetic platelet function disorder, caused by a quantitative or qualitative defect or absence of platelet membrane glycoprotein IIb/IIIa. Gastrointestinal bleeds are variable and may be severe, unpredictable and sometimes even untreatable. We present an 86-yearold woman with Glanzmann’s disease, who went to her local hospital with complaints of chest pain, tiredness and shortness of breath. After examination and laboratory research, she was diagnosed with severe anaemia caused by angiodysplasia in the gastrointestinal tract. The patient was followed weekly at our outpatient clinic for several months. Multiple treatments were given to correct her anaemia and its underlying cause. Unfortunately, the patient remained transfusiondependent throughout. She was treated with many blood transfusions, recombinant FVIIa infusions and tranexamic acid. When the burden of the disease and side-effects of the treatment became unbearable, the joint decision was made to stop treatment. The patient then unexpectedly recovered from her gastrointestinal bleeding. This case study highlights the dilemmas clinicians often face when dealing with fragile elderly people who suffer from various disorders.

DOI: https://doi.org/10.17225/jhp00099 | Journal eISSN: 2055-3390
Language: English
Page range: 29 - 31
Published on: Mar 14, 2018
Published by: Haemnet Ltd
In partnership with: Paradigm Publishing Services
Publication frequency: 1 issue per year

© 2018 Greta Mulders, Johan Boender, published by Haemnet Ltd
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.