Clinical benefit of recombinant factor VIIa in management of bleeds and surgery in two brothers suffering from the Bernard-Soulier syndrome

Haemophilia

Volumn 15, Issue 1, 2009, Pages 281-284

  Tefre, K L ^a   Ingerslev, J ^b   Sorensen B ^b  

^a AARHUS UNIVERSITY   (Denmark)

^b AARHUS UNIVERSITY HOSPITAL   (Denmark)

Author keywords

Bernard Soulier syndrome; Recombinant factor VIIa

Indexed keywords

RECOMBINANT BLOOD CLOTTING FACTOR 7A; TRANEXAMIC ACID;

ADULT; ANTERIOR CRUCIATE LIGAMENT RECONSTRUCTION; ARTHROSCOPIC SURGERY; ARTICLE; BERNARD SOULIER DISEASE; BLEEDING; BLOOD TRANSFUSION; CASE REPORT; DRUG EFFICACY; EYE SURGERY; HUMAN; HUMAN CELL; MALE; ORCHIDOPEXY; PRIORITY JOURNAL; STRABISMUS; THROMBOCYTE COUNT; THROMBOCYTOPENIA; TOOTH EXTRACTION; UPPER GASTROINTESTINAL BLEEDING;

ADULT; BERNARD-SOULIER SYNDROME; BLOOD LOSS, SURGICAL; COAGULANTS; DRUG ADMINISTRATION SCHEDULE; FACTOR VIIA; HEMORRHAGE; HEMOSTASIS, SURGICAL; HUMANS; MALE; RECOMBINANT PROTEINS; YOUNG ADULT;

EID: 63049133152     PISSN: 13518216     EISSN: 13652516     Source Type: Journal    
DOI: 10.1111/j.1365-2516.2008.01902.x     Document Type: Article

References (11)

1. Lanza F. Bernard-Soulier syndrome (hemorrhagiparous thrombocytic dystrophy). Orphanet J Rare Dis 2006; 1: 46.
2. Bernard-Soulier Syndrome website. Available at: http://www.bernardsoulier.org (last accessed on 7 December 2007).
3. Solum NO, Clemetson KJ. The discovery and characterization of platelet GPIb. J Thromb Haemost 2005; 3: 1125-32.
4. Clemetson KJ. A short history of platelet glycoprotein Ib complex. Thromb Haemost 2007; 98: 63-8.
5. Cuthbert RJG, Watson HK, Handa SI, Abbott I, Ludlam CA. DDAVP shortens the bleeding time in Bernard-Soulier Syndrome. Thromb Res 1988; 49: 649-50.
6. Peters M, Heijboer H. Treatment of a patient with Bernard-Soulier syndrome and recurrent nosebleeds with recombinant factor VIIa. Thromb Haemost 1998; 80: 352.
7. Ozelo MC, Svirin P, Larina L. Use of recombinant factor VIIa in the management of severe bleeding episodes in patients with Bernard-Soulier syndrome. Ann Hematol 2005; 84: 816-22.
8. Almeida AM, Khair K, Hann I, Liesner R. The use of recombinant factor VIIa in children with inherited platelet function disorders. Br J Haematol 2003; 121: 477-81.
9. Hacihanefioglu A, Tarkun P, Gonuææu E. Use of recombinant factor VIIa in the management and prophylaxis of bleeding episodes in two patients with Bernard-Soulier syndrome. Thromb Res 2007; 120: 455-7.
10. Ingerslev J, Stenbjerg S, Taaning E. A case of Bernard-Soulier syndrome: study of platelet glycoprotein Ib in a kindred. Eur J Haematol 1987; 39: 182-4.
11. Sørensen B, Ingerslev J. Thrombelastography and recombinant factor VIIa -haemophilia and beyond. Semin Hematol 2004; 41 (Suppl. 1): 140-4.