Similar bleeding phenotype in young children with haemophilia A or B: a cohort study

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Similar bleeding phenotype in young children with haemophilia A or B: a cohort studyN. Clausen1,*, P. Petrini2, S. Claeyssens-Donadel3, S. C. Gouw4, R. Liesner5 andPedNet and Research of Determinants of Inhibitor development (RODIN) Study Group†Article first published online: 3 JUN 2014

DOI: 10.1111/hae.12470

© 2014 John Wiley & Sons Ltd

Issue

HaemophiliaEarly View (Online Version of Record published before inclusion in an issue)

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How to CiteClausen, N., Petrini, P., Claeyssens-Donadel, S., Gouw, S. C., Liesner, R. and PedNet and Research of Determinants of Inhibitor development (RODIN) Study Group (2014), Similar bleeding phenotype in young children with haemophilia A or B: a cohort study. Haemophilia. doi: 10.1111/hae.12470

Author Information1

Department of Pediatrics, University Hospital of Aarhus at Skejby, Aarhus, Denmark

2

Department of Pediatrics, Clinic of Coagulation Disorders, Karolinska Hospital, Stockholm, Sweden

3

Centre Regional d'Haemophilie, Centre Hospitale Universitaire, Toulouse, France

4

Department of Pediatric Hematology, Emma Children's Hospital, Academic Medical Center, Amsterdam, The Netherlands

5

Haemophilia Comprehensive Care Centre, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK

†

A complete list of the members of the PedNet and Rodin Study Group appears in the “Appendix”.

* Correspondence: Niels Clausen, Department of Pediatrics, University Hospital of Aarhus at Skejby, Brendstrupgaardsvej 100, DK-8200 Aarhus, Denmark.
Tel.: +45 7845 1664; fax: +45 7845 1710; e-mail: niecla@rm.dk

Publication HistoryArticle first published online: 3 JUN 2014Manuscript Accepted: 4 MAY 2014Funded byBaxter Healthcare IncBayer Healthcare SEARCH Search Scope All contentPublication titlesIn this journalIn this issue Search String Advanced >Saved Searches > SEARCH BY CITATION Volume: Issue: Page: ARTICLE TOOLSGet PDF (146K)Save to My ProfileE-mail Link to this ArticleExport Citation for this ArticleGet Citation AlertsRequest Permissions AbstractArticleReferencesCited By View Full Article (HTML) Enhanced Article (HTML) Get PDF (146K) Keywords:bleeding phenotype;children;haemophilia A;haemophilia B;haemorrhageSummary

The bleeding phenotype has been suggested to differ between haemophilia A and B. More knowledge on the bleeding phenotype at initiation of treatment is important to optimize patient care. The aim of this study was to investigate the severity of the bleeding phenotype and the variation in bleeding in children with severe or moderate haemophilia A and B. Consecutive, previously untreated patients with severe or moderate haemophilia A and B (factor VIII or IX activity <0.01 or 0.01–0.05 IU mL-1 respectively) born between January 1st 2000 and January 1st 2010 were included. Primary outcome was severity of bleeding tendency. Secondary outcome was variation in bleeding pattern. A total of 582 patients with severe haemophilia A and 76 with severe haemophilia B did not differ in age at first exposure to clotting factor (0.81 vs. 0.88 years, P = 0.20), age at first bleed (0.82 vs. 0.88 years, P = 0.36), and age at first joint bleed (1.18 vs. 1.20 years, P = 0.59). Patients with moderate haemophilia were older compared to patients with severe haemophilia. In patients with moderate haemophilia there were no clear differences between haemophilia A and B. Severity and variation in bleeding phenotype are similar during the early stage of treatment in patients with severe and moderate haemophilia A and B respectively. The findings imply that children with haemophilia B should be observed and treated as vigilantly as those with haemophilia A.

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