Clinical and echographical control protocol of haemarthrosis in haemophilia patients with inhibitors: evaluation of the efficacy of recombinant factor VIIa in the evolution process (EFFISEVEN protocol)

Abstract

The intention of the 'clinical and echographical protocol of evaluation the efficacy of recombinant activated factor VII in the haemarthrosis' (EFFISEVEN protocol) was to provide an extensive study of the evolution of haemarthrosis, and second, of its repercussions on the degenerative process of joints. The clinical evaluation of haemarthroses and their evolution is based on a well-established methodology, although very few studies have sought to determine the correlation between pain, mobility and the objective data regarding the haemorrhage. We believe that it is necessary to unify criteria and that the EFFISEVEN protocol may contribute data that improve standards which, in turn, will influence the degenerative process of joints, and consequently affect the quality of life of haemophilia patients with inhibitors. Echographical control of haemarthrosis is an objective method that allows control over how the haemorrhage evolves and also helps in the identification of rebleeding. Therefore, its use adds a new dimension to patient management strategies. Techniques used to monitor recombinant activated factor VII (rFVIIa) treatment require further study, although preliminary results guarantee their efficacy.

A systematic review of ultrasound imaging as a tool for evaluating haemophilic arthropathy in children and adults

“…Of these 14 articles, four assessed children , three adults and seven both children and adults. In addition to those articles, three other articles reported different US scoring systems , two of which also provided information on US scanning protocols (Table ; Appendix ). Except for Martinoli et al .…”

“…Except for Martinoli et al . article which also provided information regarding the HEAD‐US scoring system, the remaining four articles (Table ; Appendix ) that described US scanning protocols were discussed in this review, but were not graded for reporting and methodological quality since they did not meet the inclusion criteria of this review.…”

Joint status in Spanish haemophilia B patients assessed using the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD‐US) score

“…The critical role of imaging techniques in the assessment and follow‐up of joint health in patients with haemophilic arthropathy is widely recognized . The development of MSK‐US in this field and the HEAD‐US protocol and scoring system have provided a unique opportunity to screen the joint status of HB patients at national level, as well as to observe the effect of treatment modality and HB severity on the establishment of haemophilic arthropathy.…”

“…In this regard, appropriate assessment with imaging tools plays a role, especially for detection of early joint changes, since clinical assessment may underestimate abnormalities. Recent studies have highlighted the importance of imaging techniques in the assessment of early arthropathy, revealing abnormalities in joints that are almost asymptomatic on physical examination . If we consider synovitis as a parameter indicating active disease due to either inappropriate treatment or to problems related to low therapeutic adherence (eg, missing appointments and/or non‐compliant patients), this was found in a considerable number of HB patients treated OD: severe (grade 2) synovitis was observed in 18% of cases and moderate to severe (grade 1 + grade 2) synovitis in up to 57%.…”

The role of ultrasonography in the diagnosis of the musculo‐skeletal problems of haemophilia

“…Querol et al. in 2008 described the protocol for the ultrasonographic control of haemarthrosis in patients with inhibitors, concluding the objective diagnosis of bleeding [26].…”