Contributions
Abstract: P09
Type: Poster presentation
Presentation during EHA Scientific Conference on Bleeding Disorders:
On Friday, September 16, 2016 from 14:00 - 15:30
Location: Cristal + Coral
Introduction
Congenital afibrinogenemia belongs to the group of autosomal recessive bleedings disorders and represents the absolute deficiency of fibrinogen. Patients with afibrinogenemia can, despite the absence of fibrinogen, they suffer bleeding or both venous and arterial thromboembolic disease.
The relationship between afibrinogenemia and thrombosis has been debated and poorly documented in the literature.
Case
A 34-year-old woman diagnosed with congenital afibrinogenemia and contraceptive hormonal treatment with a long-term bleeding history: autolimietd joint bleeding treated with fibrinogen concentrates (FC), right adrenal hematoma and intracerebral bleeding without sequelae in august 2013.
She was admitted to her community hospital complaining of right chest pain. A CT scan was performed and a pulmonary thromboembolism in the right lobar artery was diagnosed. She was treated with fibrinogen concentrates to maintain fibrinogen levels above 100mg/dL and started bemiparin 5000 units /day. She was discharged, and a month later a new control CT scan was done, detecting the persistence and the increase of the artery thrombus. The patient was admitted in our Hospital, and started anticoagulation treatment with enoxaparin 60mg/kg bid and cryoprecipitate (due to a fibrinogen concentrates shortage at our hospital at that moment) to maintain levels of fibrinogen above 100mg/dL. After a multidisciplinary meeting with cardiovascular surgery, we decided to continue with anticoagulation treatment with enoxaparine 60 mg/kg bid, maintaining levels of anti-Xa between 0.5-0.7 UI/mL and a prophylaxis treatment with fibrinogen concentrates 3 grs every other day, to maintain levels between 70-150mg/dL.
After two years with this treatment, due to elevated risk of osteopenia related to heparin and patient preference, we decided to switch enoxaparine to apixaban 2.5mg bid.
Conclusions
Patients with congenital afibrinogemia and thrombotic events may beneficiate with use of direct oral anticoagulants and concomitant prophylaxis treatment with fibrinogen concentrates. This could be a safe alternative of anticoagulation treatment for these patients.
References
1. Margaglione et al,Haemophilia (2015), 21, e411--e455
2. Lucia Stanciakova et al, Expert Review of Hematology, 2016, VOL. 9, NO. 7, 639–648
3. Michael Nagler et al, Thrombosis and Haemostasis 116.4/2016
4. Cristina Santoro et al, Seminars in Thrombosis & Hemostasis Vol. 42 No. 5/2016
5. Amihai Rottenstreich et al, J Thromb Thrombolysis (2016) 42:261–266
6. Castaman G et al, Haemophilia (2009), 15, 533–537
7. M Teresa et al, Haemophilia (2015), 21, 88–94
Abstract: P09
Type: Poster presentation
Presentation during EHA Scientific Conference on Bleeding Disorders:
On Friday, September 16, 2016 from 14:00 - 15:30
Location: Cristal + Coral
Introduction
Congenital afibrinogenemia belongs to the group of autosomal recessive bleedings disorders and represents the absolute deficiency of fibrinogen. Patients with afibrinogenemia can, despite the absence of fibrinogen, they suffer bleeding or both venous and arterial thromboembolic disease.
The relationship between afibrinogenemia and thrombosis has been debated and poorly documented in the literature.
Case
A 34-year-old woman diagnosed with congenital afibrinogenemia and contraceptive hormonal treatment with a long-term bleeding history: autolimietd joint bleeding treated with fibrinogen concentrates (FC), right adrenal hematoma and intracerebral bleeding without sequelae in august 2013.
She was admitted to her community hospital complaining of right chest pain. A CT scan was performed and a pulmonary thromboembolism in the right lobar artery was diagnosed. She was treated with fibrinogen concentrates to maintain fibrinogen levels above 100mg/dL and started bemiparin 5000 units /day. She was discharged, and a month later a new control CT scan was done, detecting the persistence and the increase of the artery thrombus. The patient was admitted in our Hospital, and started anticoagulation treatment with enoxaparin 60mg/kg bid and cryoprecipitate (due to a fibrinogen concentrates shortage at our hospital at that moment) to maintain levels of fibrinogen above 100mg/dL. After a multidisciplinary meeting with cardiovascular surgery, we decided to continue with anticoagulation treatment with enoxaparine 60 mg/kg bid, maintaining levels of anti-Xa between 0.5-0.7 UI/mL and a prophylaxis treatment with fibrinogen concentrates 3 grs every other day, to maintain levels between 70-150mg/dL.
After two years with this treatment, due to elevated risk of osteopenia related to heparin and patient preference, we decided to switch enoxaparine to apixaban 2.5mg bid.
Conclusions
Patients with congenital afibrinogemia and thrombotic events may beneficiate with use of direct oral anticoagulants and concomitant prophylaxis treatment with fibrinogen concentrates. This could be a safe alternative of anticoagulation treatment for these patients.
References
1. Margaglione et al,Haemophilia (2015), 21, e411--e455
2. Lucia Stanciakova et al, Expert Review of Hematology, 2016, VOL. 9, NO. 7, 639–648
3. Michael Nagler et al, Thrombosis and Haemostasis 116.4/2016
4. Cristina Santoro et al, Seminars in Thrombosis & Hemostasis Vol. 42 No. 5/2016
5. Amihai Rottenstreich et al, J Thromb Thrombolysis (2016) 42:261–266
6. Castaman G et al, Haemophilia (2009), 15, 533–537
7. M Teresa et al, Haemophilia (2015), 21, 88–94