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Blood, 23 July 2009, Vol. 114, No. 4, pp. 779-784. Prepublished online as a Blood First Edition Paper on April 8, 2009; DOI 10.1182/blood-2009-01-195313. This Article Full Text Full Text (PDF) Supplemental Appendix All Versions of this Article: blood-2009-01-195313v1 114/4/779    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Google Scholar Articles by Tagariello, G. Articles by Mannucci, P. M. PubMed PubMed Citation Articles by Tagariello, G. Articles by Mannucci, P. M. Related Collections Free Research Articles Thrombosis and Hemostasis Clinical Trials and Observations Related Articles in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS Comparison of the rates of joint arthroplasty in patients with severe factor VIII and IX deficiency: an index of different clinical severity of the 2 coagulation disorders Giuseppe Tagariello1, Alfonso Iorio2, Elena Santagostino3, Massimo Morfini4, Ruggero Bisson5, Massimo Innocenti6, Maria Elisa Mancuso3, Maria Gabriella Mazzucconi7, Gian Luigi Pasta8, Paolo Radossi1, Giuseppina Rodorigo9, Cristina Santoro7, Roberto Sartori1, Antonio Scaraggi10, Luigi Pier Solimeno8, Pier Mannuccio Mannucci3, on behalf of the Italian Association Hemophilia Centre (AICE) 1 Transfusion Service, Hemophilia and Regional Blood Disease Centre, Castelfranco Veneto, Castelfranco Veneto; 2 Vascular and Internal Medicine, University of Perugia, Perugia; 3 Angelo Bianchi Bonomi Hemophilia and Thrombosis Centre, Department of Medicine and Medical Specialties, University of Milan and Istituto Di Ricovero e Cura a Carattere Scientifico (IRCCS) Maggiore Hospital, Mangiagalli and Regina Elena Foundation, Milan; 4 Agency for Hemophilia, Azienda Ospedaliera Universitaria Careggi, Florence; 5 Department of Orthopedics, Castelfranco Veneto, Castelfranco Veneto; 6 Clinica Ortopedica II, Azienda Ospedaliera Universitaria Careggi, Florence; 7 Department of Cellular Biotechnology and Haematology, University La Sapienza, Rome; 8 Traumatology Unit, IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation, Milan; 9 Unità Operativa di Angiologia e Malattie della Coagulazione "Marino Golinelli" Azienda Ospedaliera di Bologna Policlinico S Orsola, Bologna; and 10 Internal Medicine, Hemophilia Centre, Bari, Italy Data from the Italian Hemophilia Centres were collected to perform a retrospective survey of joint arthroplasty in patients with severe hemophilia. Twenty-nine of 49 hemophilia centers reported that 328 of the 347 operations were carried out in 253 patients with severe hemophilia A (HA) and 19 in 15 patients with severe hemophilia B (HB). When results were normalized to the whole Italian hemophilia population (1770 severe HA and 319 severe HB), patients with HA had a 3-fold higher risk of undergoing joint arthroplasty (odds ratio [OR], 3.38; 95% confidence interval [CI], 1.97-5.77; P < .001). These results were confirmed after adjustment for age, HIV, hepatitis C virus (HCV), and inhibitor in a Cox regression model (HR, 2.65; 95% CI, 1.62-4.33; P < .001). The survival analysis of time to joint arthroplasty in the subset of patients with severe HA was not affected by the severity of factor VIII (FVIII) gene mutations. A systematic review of literature articles reporting joint arthroplasties in HA and HB showed that the proportion of HA patients who had undergone arthroplasties was higher than that of HB patients, in agreement with the findings in our Italian cohort. These data suggest that the 2 inherited coagulation disorders have a different severity of clinical phenotype. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Articles in Blood Online: Insufficient evidence to suggest less stringent therapy in hemophilia B? Ingrid E. M. den Uijl, Goris Roosendaal, and Kathelijn Fischer Blood 2009 114: 4907. [Full Text] [PDF] Response: Comparing joint arthroplasties in severe hemophilia A with severe hemophilia B Giuseppe Tagariello, Alfonso Iorio, and Pier M. Mannucci Blood 2009 114: 4907-4908. [Full Text] [PDF] Is VIII worse than IX? Michael Makris Blood 2009 114: 750-751. [Full Text] [PDF] This article has been cited by other articles: I. E. M. den Uijl, G. Roosendaal, and K. Fischer Insufficient evidence to suggest less stringent therapy in hemophilia B? Blood, November 26, 2009; 114(23): 4907 - 4907. [Full Text] [PDF] G. Tagariello, A. Iorio, and P. M. Mannucci Response: Comparing joint arthroplasties in severe hemophilia A with severe hemophilia B Blood, November 26, 2009; 114(23): 4907 - 4908. [Full Text] [PDF] M. Makris Is VIII worse than IX? Blood, July 23, 2009; 114(4): 750 - 751. [Full Text] [PDF]

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