Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia

Real‐world comparative analysis of bleeding complications and health‐related quality of life in patients with haemophilia A and haemophilia B

“…Patients with HA and HB in the CHESS study experienced comparable haemophilia‐related clinical and HRQoL impacts. These study findings support the recommendations of current clinical guidelines that efforts to promote the same standard of prophylaxis treatment for patients with HA and HB are warranted …”

“…A “target joint” as defined in the CHESS study encompasses any joint with known chronic synovitis. To incorporate the nuanced definitions and diagnostic options that exist within registries, trials and guidelines, study investigators were given discretion as to any further criteria they might use to define target joints with respect to bleed frequency and period of observation.…”

“…promote the same standard of prophylaxis treatment for patients with HA and HB are warranted. 17,[22][23][24][25][26][27] The difference in clinical severity of severe HB compared with HA has long been a topic of controversy, with some studies indicating that HB had less impact on HRQoL, with lower rates of bleeding, less joint damage and reduced requirement for surgical joint treatment. 7,8,10,13 However, the findings of this study have shown that although HB is less prevalent than HA, both HA and HB have important clinical and HRQoL impacts on patients: including the effects of bleeding into target joints, that results in pain; long-term effects, such as reduced range of movement (ROM), with up to 90% of patients developing chronic arthropathy due to haemophilia by 30 years of age.…”

Blood-induced bone loss in murine hemophilic arthropathy is prevented by blocking the iRhom2/ADAM17/TNF-α pathway

“…Depending on the severity of the symptoms that develop, HA can have a devastating impact on patients. 1,2 Current efforts to prevent HA are mainly focused on management of acute bleeds and optimizing the schedule for prophylactic factor replacement, 5 which has significant advantages over episodic factor replacement following bleeding. [6][7][8][9] However, breakthrough bleeds occur even on the best prophylaxis protocols, 10 and HA remains a persistent problem and challenge for hemophilia patients.…”

“…Therefore, improving the understanding of its pathogenesis and devising novel treatments remain major unmet needs in this area. 5,11 Patients suffering from hemophilia frequently develop osteopenia or osteoporosis, [12][13][14][15][16][17][18] raising questions about whether the underlying mechanism might be related to the presence of blood in the joint. Bleeding episodes are proposed to cause toxicity for articular chondrocytes by overloading them with iron, 19 resulting in damage to articular cartilage.…”