Öz
Factor XI deficiency is a hemorrhage disorder which can be either autosomal recessive or/and autosomal codominant and effects both women and men. Factor XI deficiency is uncommon in non-Jewish general population and average frequency of disorder is 1/1 million. Clinical phenotype is nonhomogeneous and bleeding risk depends on individual’s reaction against hemostatic variances. There is a poor relation between the Factor XI plasma level and bleeding tendency. In case of Factor XI deficiency, uncommon, unlike hemophilia A and B, spontaneous hemorrhage, surgical or trauma sequent are at risk. Hemarthrosis and intramuscular bleeding are common in hemophilia A and B but not typically seen in Factor XI deficiency disorders. Common symptoms are easy bruising and nosebleed. History of the disorder mostly indicates the findings about the difficulty in staunching. In this case, after detecting the activated partial thromboplastin time length before the operation due to build upon mandibula, management of patients, having Factor XI deficiency hemophilia C which is a cause of rare seen hemorrhage disorder, are presented in terms of bleeding diathesis.
Anahtar Kelimeler
Kaynakça
- 1. Seligsohn U. Factor XI in haemostasis and thrombosis: past, present and future. Thromb Haemost 2007; 98: 84-9.
- 2. Seligsohn U. Factor XI deficiency in humans. J Thromb Haemost 2009; Suppl 1: 84-7.
- 3. Bolton-Maggs PH. Factor XI deficiency and its management. Haemophilia 2000; Suppl 1: 100-9.
- 4. Cetin G, Karatoprak C, Kiskac M, Zorlu M, Rezvani A, Cikrikcioglu MA. Factor XI deficiency diagnosed following use of adalimumab. Indian J Pharmacol 2014; 46: 553-4.
- 5. Kılıç SÇ, İçağasıoğlu FD, Güven AS, Berber E. Spontaneous thrombosis in a patient with factor XI deficiency homozygous for the p.Cys398Tyr mutation. Blood Transfus 2014; 12: 446-8.
- 6. Choi SJ, Kim J, Lee KA, Choi JR, Yoo J. Cys482Trp missense mutation in the coagulation factor XI gene (F11) in a Korean patient with factor XI deficiency. Ann Lab Med 2014; 34: 332-5.
- 7. Keskin EY, Gürsel T, Kaya Z, Dai L, Koçak Ü, Yenicesu İ, et al. Molecular basis and bleeding manifestations of factor XI deficiency in 11 Turkish families. Blood Coagul Fibrinolysis 2015; 26: 63-8.
- 8. Siboni SM, Biguzzi E, Pasta G, Mannucci PM, Mistretta C, Fantini NN, et al. Management of orthopaedic surgery in rare bleeding disorders. Haemophilia 2014; 20: 693-701.
- 9. Mumford AD, Ackroyd S, Alikhan R, Bowles L, Chowdary P, Grainger J, et al. BCSH Committee. Guideline for the diagnosis and management of the rare coagulation disorders: a United Kingdom Haemophilia Centre Doctors' Organization guideline on behalf of the British Committee for Standards in Haematology. Br J Haematol 2014; 167: 304-26.
- 10. Billon S, Le Niger C, Escoffre-Barbe M, Vicariot M, Abgrall JF. The use of recombinant factor VIIa (NovoSeven) in a patient with a factor XI deficiency and a circulating anticoagulant. Blood Coagul Fibrinolysis 2001; 12: 551-3.
Öz
Kaynakça
- 1. Seligsohn U. Factor XI in haemostasis and thrombosis: past, present and future. Thromb Haemost 2007; 98: 84-9.
- 2. Seligsohn U. Factor XI deficiency in humans. J Thromb Haemost 2009; Suppl 1: 84-7.
- 3. Bolton-Maggs PH. Factor XI deficiency and its management. Haemophilia 2000; Suppl 1: 100-9.
- 4. Cetin G, Karatoprak C, Kiskac M, Zorlu M, Rezvani A, Cikrikcioglu MA. Factor XI deficiency diagnosed following use of adalimumab. Indian J Pharmacol 2014; 46: 553-4.
- 5. Kılıç SÇ, İçağasıoğlu FD, Güven AS, Berber E. Spontaneous thrombosis in a patient with factor XI deficiency homozygous for the p.Cys398Tyr mutation. Blood Transfus 2014; 12: 446-8.
- 6. Choi SJ, Kim J, Lee KA, Choi JR, Yoo J. Cys482Trp missense mutation in the coagulation factor XI gene (F11) in a Korean patient with factor XI deficiency. Ann Lab Med 2014; 34: 332-5.
- 7. Keskin EY, Gürsel T, Kaya Z, Dai L, Koçak Ü, Yenicesu İ, et al. Molecular basis and bleeding manifestations of factor XI deficiency in 11 Turkish families. Blood Coagul Fibrinolysis 2015; 26: 63-8.
- 8. Siboni SM, Biguzzi E, Pasta G, Mannucci PM, Mistretta C, Fantini NN, et al. Management of orthopaedic surgery in rare bleeding disorders. Haemophilia 2014; 20: 693-701.
- 9. Mumford AD, Ackroyd S, Alikhan R, Bowles L, Chowdary P, Grainger J, et al. BCSH Committee. Guideline for the diagnosis and management of the rare coagulation disorders: a United Kingdom Haemophilia Centre Doctors' Organization guideline on behalf of the British Committee for Standards in Haematology. Br J Haematol 2014; 167: 304-26.
- 10. Billon S, Le Niger C, Escoffre-Barbe M, Vicariot M, Abgrall JF. The use of recombinant factor VIIa (NovoSeven) in a patient with a factor XI deficiency and a circulating anticoagulant. Blood Coagul Fibrinolysis 2001; 12: 551-3.
Ayrıntılar
| Birincil Dil | İngilizce |
|---|---|
| Konular | Çocuk Metabolizma Hastalıkları, Diş Hekimliği (Diğer) |
| Bölüm | Olgu Sunumu |
| Yazarlar | |
| Yayımlanma Tarihi | 30 Nisan 2018 |
| Yayımlandığı Sayı | Yıl 2018 Cilt: 19 Sayı: 1 |