<abstract xmlns="http://www.w3.org/1999/xhtml"><p> 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.</p></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.

Tightrope walking with Glanzmann’s disease

The Journal of Haemophilia Practice

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

{"article-title":"Tightrope walking with Glanzmann\u2019s disease"}

Glanzmann’s disease or thrombasthenia is a very rare genetic platelet function disorder, caused by a quantitative or qualitative defect or absence of platelet...

Tightrope walking with Glanzmann’s disease

Article Category: CASE REPORT

Pubblicato online: 14 mar 2018

Pagine: 29 - 31

DOI: https://doi.org/10.17225/jhp00099

Parole chiave
Glanzmann’s disease, thrombasthenia, treatment, bleeding, coagulation disorde

© 2018

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Tightrope walking with Glanzmann’s disease

Article Category: CASE REPORT

Pubblicato online: 14 mar 2018

Pagine: 29 - 31

DOI: https://doi.org/10.17225/jhp00099

Parole chiaveGlanzmann’s disease, thrombasthenia, treatment, bleeding, coagulation disorde

© 2018

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Parole chiave
Glanzmann’s disease, thrombasthenia, treatment, bleeding, coagulation disorde