title: Secondary prophylaxis in adolescent and adult haemophiliacs.
abstract: Progressive arthropathy of large joints of the limbs (knees, ankles, elbows), resulting from recurrent joint bleeds and subsequent long-term degenerative phenomena, is one of the main causes of morbidity and of deterioration of quality of life in adult severe hemophiliacs. While primary prophylaxis (i.e. the regular continuous long-term infusion of factor concentrates started before the age of two years and/or after no more than one joint bleed) is nowadays considered the gold standard for preserving joint function in patients with severe haemophilia, the benefits of secondary prophylaxis (i.e., all the long-term regular treatments not fulfilling the criteria of primary prophylaxis) are still controversial. In this review we present the literature data on secondary prophylaxis, focusing on adolescent and adults haemophiliacs along with clinical experience in Italy. On the whole, the more recently published studies suggest the effectiveness of early and delayed secondary prophylaxis. However, a number of questions are still unanswered, including the optimal dose, dosing interval and duration of secondary prophylaxis. Only large, prospective, long-term, possibly randomized studies will help to definitively assess the clinical impact of this strategy in adolescent and adult hemophiliacs.
authors.affiliation: Centro di Riferimento Regionale per la Cura delle Malattie Emorragiche Congenite, Azienda Ospedaliero-Universitaria di Parma, Parma, Italia. atagliaferri@ao.pr.it
authors.forename: Annarita
authors.initials: A
authors.lastname: Tagliaferri
authors.forename: Caterina
authors.initials: C
authors.lastname: Di Perna
authors.forename: Gianna Franca
authors.initials: GF
authors.lastname: Rivolta
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D018450:Disease Progression; D005164:Factor IX / Q000008:administration & dosage / Q000191:economics / Q000627:therapeutic use*; D005169:Factor VIII / Q000008:administration & dosage / Q000191:economics / Q000627:therapeutic use*; D005260:Female; D006395:Hemarthrosis / Q000453:epidemiology / Q000209:etiology / Q000517:prevention & control*; D006467:Hemophilia A / Q000150:complications / Q000188:drug therapy*; D002836:Hemophilia B / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D008297:Male; D011788:Quality of Life; D012189:Retrospective Studies; D055502:Secondary Prevention / Q000191:economics / Q000706:statistics & numerical data; D055815:Young Adult
chemical_list: D005169:Factor VIII; D005164:Factor IX