The use of activated recombinant coagulation factor VII during haemarthroses and synovectomy in a patient with congenital severe factor V deficiency

Factor V deficiency is a rare hereditary bleeding disorder. Currently, FV concentrates are not available, and the treatment of spontaneous bleeding or bleeding associated with invasive procedures is transfusion of fresh frozen plasma (FFP). However, FFP transfusion can lead to the development of inh...

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Bibliographic Details
Published in:Haemophilia : the official journal of the World Federation of Hemophilia 2005-03, Vol.11 (2), p.167-170
Main Authors: González‐Boullosa, R., Ocampo‐Martínez, R., Alarcón‐Martín, M. J., Suárez‐Rodríguez, M., Domínguez‐Viguera, L., González‐Fajo, G.
Format: Article
Language:English
Subjects:
Blood Coagulation Factors - therapeutic use
factor V deficiency
Factor V Deficiency - complications
Factor V Deficiency - drug therapy
Factor V Deficiency - physiopathology
Factor VII - therapeutic use
Factor VIIa
Female
haemarthrosis
Hemarthrosis - etiology
Hemarthrosis - physiopathology
Hemarthrosis - prevention & control
Hematoma - therapy
Hemostasis - physiology
Humans
Middle Aged
NovoSeven
Physical Therapy Modalities
Postoperative Complications - therapy
recombinant FVIIa
Recombinant Proteins - therapeutic use
Shoulder Joint - physiopathology
Shoulder Joint - surgery
Synovectomy
Treatment Outcome
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Summary:Factor V deficiency is a rare hereditary bleeding disorder. Currently, FV concentrates are not available, and the treatment of spontaneous bleeding or bleeding associated with invasive procedures is transfusion of fresh frozen plasma (FFP). However, FFP transfusion can lead to the development of inhibitor to FV, and is associated with several potential transfusion reactions including allergic reactions. We report a patient with congenital severe FV deficiency with repeated haemarthroses of a shoulder joint, and progressively severe allergic reactions to FFP transfusions. In addition, the patient also developed acute pulmonary oedema. Activated recombinant coagulation factor VII (rFVIIa) was used as an alternative haemostatic agent to FFP. We describe the use of rFVIIa in this patient during haemarthroses, synovectomy, and physiotherapy.
ISSN:1351-8216
1365-2516
DOI:10.1111/j.1365-2516.2005.00956.x