"Primary prophylaxis" with rFVIIa in a patient with severe haemophilia A and inhibitor.
The development of antibodies that inhibit or neutralize replacement therapy with factor VIII or factor IX is today the most serious complication of haemophilia and its treatment. Inhibitor patients have more severe joint morbidity than patients without inhibitors, and older adults experience significant orthopaedic disabilities. Because of the serious and disabling consequences of persistent inhibitors, there is considerable clinical and research interest in establishing effective bypassing agent regimens to prevent bleeding in inhibitor patients in much the same way as prophylaxis procedure works in noninhibitor patients. In the majority of these patients, the bypass agent was used as a secondary prophylactic. In this report, the use of recombinant factor VIIa prior to any clinically evident joint bleed in a patient with haemophilia A and high-titre inhibitor is described.
Jimenez-Yuste
Victor
V
Haematology Department, Hospital Universitario La Paz, Paseo de la Castellana 261, Madrid, Spain. vjimenez.hulp@salud.madrid.org
Quintana
Manuel
M
Alvarez
Maria Teresa
MT
Martin-Salces
Monica
M
Hernandez-Navarro
Fernando
F
D002675:Child, Preschool; D015942:Factor VIIa / Q000627:therapeutic use*; D006467:Hemophilia A / Q000188:drug therapy*; D006801:Humans; D008297:Male; D011446:Prospective Studies; D011994:Recombinant Proteins / Q000627:therapeutic use; D016896:Treatment Outcome
D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa