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.
1. Poon MC, Di Minno G, d'Oiron R, Zotz R. New insights into the treatment of Glanzmann thrombasthenia. Transfus Med Rev 2016; 30(2): 92-9.
2. Engelen ET, van Galen KP, Schutgens RE. Thalidomide for treatment of gastrointestinal bleedings due to angiodysplasia: a case report in acquired von Willebrand syndrome and review of the literature. Haemophilia 2015;21(4): 419-29.
3. Sami SS, Al-Araji SA, Ragunath K. Review article: gastrointestinal angiodysplasia - pathogenesis, diagnosis and management. Aliment Pharmacol Ther 2014; 39(1): 15-34.
4. Alikhan R, Keeling D. Von Willebrand disease, angiodysplasia and atorvastatin. Br J Haematol 2010;149(1): 159-60.
5. Leebeek FWG, Mauser-Bunschoten EP, Utrecht. Dutch guidelines for diagnosis and treatment of hemophilia and other rare bleeding disorders. ISBN 987-90-8253-195-0. 2009.
1. Solh T, Botsford A, Solh M. Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options. J Blood Med 2015; 6: 219-27.
2. Bauditz J. Effective treatment of gastrointestinal bleeding with thalidomide - chances and limitations. World J Gastroenterol 2016; 22(11): 3158-64.
3. Sohal M, Laffan M. Von Willebrand disease and angiodysplasia responding to atorvastatin. Br J Haematol 2008; 142(2): 308-9.