Management of acute haemarthrosis in haemophilia A without inhibitors: literature review, European survey and recommendations

Abstract

Summary. Acute haemarthrosis is a frequent type of bleeding in individuals with haemophilia. Delayed and/or inadequate treatment can trigger a series of pathological changes within the joint, leading to a painful and disabling arthropathy. The early management of intra‐articular bleeding has the potential to prevent chronic joint disease and may include a combination of factor replacement, rest, ice, rehabilitation and, in certain cases, joint aspiration. Little data are, however, available regarding the optimal management of acute haemarthrosis, especially with respect to replacement therapy and the use of adjunctive therapies (aspiration, avoidance of weight bearing and immobilization, as well as the use of anti‐inflammatory medication and embolization). To provide more insight into the management of acute haemarthrosis in patients with haemophilia, a literature review was conducted. Concomitantly, current management was surveyed in 26 European haemophilia comprehensive care centres representing 15 different countries. The review highlights the need for future robust studies to better define the appropriate replacement therapy and the role of adjunctive therapies such as aspiration. The survey reveals much heterogeneity in the management of acute haemarthrosis across the EU. Within the constraints discussed, treatment recommendations are presented that reflect the literature, current practice and the clinical experience of the European Haemophilia Therapy Standardisation Board (EHTSB).

Nacionalna priporočila za obravnavo bolnikov s hemofilijo

“…»punkcije«) je smiselen, kadar je sklep napet in boleč zaradi krvavitve in ne kaže izboljšanja po 24 urah nadomestnega zdravljenja, če bolečine v sklepu ne uspemo zmanjšati, če so izraženi znaki prizadetosti živca in žile pripadajočega uda, ob nenavadnem dvigu lokalne ali telesne temperature ter ob drugih znakih okužbe (99,100).…”

“…Pri hemofiliku z že okvarjenim sklepom so primerni fizioterapevtski postopki ob nadomestnem zdravljenju s koagulacijskimi faktorji zato, da se sklep vrne v stanje pred krvavitvijo in se prepreči nastanek novih krvavitev. Zato je med rehabilitacijo sklepov potrebna trajnejša redna obravnava pri izkušenem fizioterapevtu in/ali fiziatru (99,174).…”

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“…Those with little or no circulating factor (<1% of normal activity within the blood) generally experience the most severe symptoms. The syndrome is characterised by spontaneous bleeding [2] commonly affecting the musculoskeletal system, particularly the joints [3][4][5]. Historically, severe forms of haemophilia carried a very poor prognosis.…”

Haemophilia: A Race for Cure

“…The syndrome is characterised by spontaneous bleeding 2 commonly affecting the musculoskeletal system, particularly the joints. [3][4][5] Historically, severe forms of haemophilia carried a very poor prognosis. Fortunately advances in pharmacotherapy and clinical care have improved both morbidity and mortality.…”