title: Inhibitor development and substitution therapy in a developing country: Turkey.
abstract: Prevalence of inhibitor in developing countries, such as Turkey, where fresh frozen plasma (FFP) is still in use due to high cost of concentrates, is unknown. To determine the frequency of inhibitors in Turkish haemophiliacs exposed to blood products, 53 haemophilia A patients (age range 1-20; median: 11 years) and 12 haemophilia B patients (age range 3-20; median: 10 years), were evaluated; 31 haemophilia A patients (23 severe) received plasma-derived concentrates and 22 patients (10 severe) only FFP. No haemophilia B patients developed inhibitor, compared with seven of 53 (13%) haemophilia A patients, all with a severe defect (7/33; 21%) and treated with concentrates (7/23; 30%), whereas severe patients treated with FFP showed a lower risk to develop inhibitors (0/10, P = 0.07). Inhibitors were detected after 8-125 exposure days (median: 52). Intermediate-purity concentrates and pasteurization seemed to be linked with a higher risk of inhibitor compared to high-purity concentrates and solvent-detergent inactivation for seven patients with inhibitor. In four of seven inhibitor patients low-dose concentrate was administered at 25 IU kg-1 twice weekly and inhibitor disappeared in 1-4 months. This regimen might be recommended for immune tolerance in developing countries for its lower cost.
authors.affiliation: Ege University Hospital, Department of Pediatric Hematology, Izmir, Turkey. Kkavakli@med.ege.edu.tr
authors.forename: K
authors.initials: K
authors.lastname: Kavakli
authors.forename: A
authors.initials: A
authors.lastname: Gringeri
authors.forename: R
authors.initials: R
authors.lastname: Bader
authors.forename: G
authors.initials: G
authors.lastname: Nisli
authors.forename: A
authors.initials: A
authors.lastname: Polat
authors.forename: Y
authors.initials: Y
authors.lastname: Aydinok
mesh_terms: D000293:Adolescent; D000328:Adult; D001779:Blood Coagulation Factors / Q000037:antagonists & inhibitors*; D002648:Child; D002675:Child, Preschool; D006467:Hemophilia A / Q000097:blood*; D002836:Hemophilia B / Q000097:blood*; D006801:Humans; D007223:Infant; D010949:Plasma / Q000502:physiology*; D014421:Turkey
chemical_list: D001779:Blood Coagulation Factors