PB1129 - Experience in rural areas in the evaluation of the joint health status of patients with non-severe hemophilia A not treated with prophylaxis.

Background: Although clinical and/or radiological joint involvement is clearly documented in moderate or severe forms of hemophilia, its hidden presentation makes its presence underestimated in mild forms.

Aims: The objective is to document the presence of clinical and/or radiological alterations in mild hemophiliac patients without prophylactic treatment.

Methods: Prospective study that analyzed 5 patients diagnosed with mild hemophilia A (FVIIIc: 10-40%) and asymptomatic from the joint clinical point of view and without the need for prophylactic treatment. The radiological assessment was carried out by ultrasound using the HEAD-US scale.

Results: The median age was 11 years (range: 5-32 years) and FVIIIc levels of 23% (range: 20%-40%) without previous exposure to treatment and with physical activity according to age (even 40% patients performed physical activity with high joint impact, agriculture for example). In this same population, joint damage was evidenced through the HEAD-US scale (minimal synovitis of the left ankle in the first patient and in the second patient there was established bilateral knee synovitis + osteochondral involvement < 50% in the right knee). This same population reports presenting more than one episode of joint pain per year (sometimes disabling).

Conclusion(s): 40% of the sample, presented radiological alterations compatible with hemophilic arthropathy, so it is essential to jointly evaluate mild hemophiliac patients (especially those who perform physical activity with great joint impact) to optimize their therapeutic management, regardless of the quantification of FVIIIc levels.