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Semin Thromb Hemost 2013; 39(07): 723-731
DOI: 10.1055/s-0033-1354422
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Arthropathy in Patients with Moderate Hemophilia A: A Systematic Review of the Literature

Matteo Nicola Dario Di Minno   *
1   Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, “Federico II” University, Naples, Italy
,
Pasquale Ambrosino   *
1   Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, “Federico II” University, Naples, Italy
,
Massimo Franchini
2   Immunohematology and Transfusion Medicine, Carlo Poma Hospital, Mantova, Italy
,
Antonio Coppola
1   Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, “Federico II” University, Naples, Italy
,
Giovanni Di Minno
1   Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, “Federico II” University, Naples, Italy
› Author Affiliations
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Publication History

Publication Date:
10 September 2013 (online)

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Abstract

Chronic arthropathy is a major complication in severe hemophilia A (Factor [F] VIII < 1%). Almost all adults with severe hemophilia, who have not received prophylaxis with FVIII since their early childhood, suffer from chronic arthropathy. Patients with moderate hemophilia (FVIII activity 1–5%) usually experience fewer joint bleeds than those with severe hemophilia and are thought to rarely develop a significant degree of chronic arthropathy. However, some patients with moderate hemophilia behave like those with the severe form of the disorder, reporting several joint bleeds per year and significant joint impairment. Currently, only little data are available about the prevalence of arthropathy, the degree of quality of life impairment, and the need for orthopedic care/aids in patients with moderate hemophilia. In this systematic review of literature, the prevalence of overt arthropathy ranges between 15 and 77% in patients with moderate hemophilia and prophylactic replacement treatment is prescribed in approximately 30% of these patients, usually after diagnosis of clinically overt arthropathy. Moreover, because of the lack of imaging studies (magnetic resonance and/or ultrasound), the prevalence of subclinical arthropathy cannot be determined. These data confirm that severity of hemophilia should not be defined only according to FVIII levels and that a relevant proportion of patients with nonsevere hemophilia might benefit from a “tailored early prophylaxis.”

Keywords

moderate hemophilia - arthropathy - prophylaxis

* Matteo Nicola Dario Di Minno and Pasquale Ambrosino contributed equally to this article.


 

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