High incidence of ankle arthropathy in mild and moderate haemophilia A

Abstract

A clinical study of patients with mild haemophilia A to document the frequency and severity of arthropathy has not been previously published. We studied ankle arthropathy in 34 patients with mild/moderate haemophilia A. The patients were assessed for the presence and severity of pain, and by the physical and radiological scoring systems for the evaluation of haemophilic arthropathy recommended by the World Federation of Haemophilia (WFH). Of the 34 patients, 16 (47%) had ankle pain, which was of moderate to severe degree in nine patients, and associated with limitation of physical activities in 13 patients. Of 33 patients examined by radiology 17 (52%) were positive for ankle arthropathy, and of these, 16 were also positive by the physical score. The presence and severity of ankle arthropathy was more common in patients with a one-stage factor VIII level of less than or equal to 11 IU/dl. There was a significant relationship between the presence of ankle arthropathy and a history of bleeds into the ankle joint as a child. We conclude that arthropathy of the ankle in these patients is common, is often severe and disabling, and is due to episodes of bleeding into the ankle joint during childhood.

Effect of radiosynoviorthesis on the progression of arthropathy and haemarthrosis reduction in haemophilic patients

“…Thus, it appears that the progression of arthropathy, following onset, is not only influenced by the type of substitution treatment received and by the number of bleeding episodes, [1][2][3][4][5]31,32 but it could also be largely conditioned by the subject's age.…”

Rationale for individualizing haemophilia care

“…However, a recent study has highlighted the prevalence of disabling arthropathy in patients with mild or moderate haemophilia [13]. In our opinion, these patients should receive more attention and individualized therapy, the rationale for which we outline below.…”

“…An early report showed that arthropathy may develop in patients who have reported few or no joint bleeds [14]. Subsequently, other studies have shown that although some patients bleed frequently but do not develop arthropathy, others develop arthropathy in the absence of overt bleeding [13,[15][16][17]. These studies suggest that arthropathy may develop from subclinical bleeding, and prophylaxis to prevent this was suggested many years ago [18].…”

Biomechanical markers and theoretical concepts related to haemophilic ankle and subtalar joint arthropathy: introducing the term ‘haemophilic tarsal pan‐arthropathy’

“…In CWH the ankle has more reported haemarthroses and worse joint scores compared with knees or elbows [2,[6][7][8]. The prevalence of ankle arthropathy has also been reported as high in mild and moderate PWH [9]. Therefore, this area requires specific attention.…”