Agapidou Alexandra, Stavrakis Thomas, Vlachaki Efthymia, Anagnostis Panagiotis, Vakalopoulou Sophia
Aristotle University, Hippokration Hospital, Second Propaedeutic Department of Internal Medicine, Thessaloniki, Greece, E-mail :
Turk J Haematol. 2016 Jun 5;33(2):88-93. doi: 10.4274/tjh.2016.0031.
Haemophilia is an inherited bleeding disorder that can lead to degenerative joint arthropathy due to recurrent bleeding episodes affecting the musculoskeletal system of the patient. The cause of bleeding can be either traumatic or spontaneous. The pathogenesis of haemophilic arthropathy is unclear as many factors like iron, inflammatory cytokines, and angiogenic factors contribute to this process. Blood into joints can deteriorate the bone to such an extent that the patient experiences pain, reduction of the range of movement, and deformity of the joint, conditions that could have a great impact on quality of life. Over the years, management of haemophilic arthropathy has changed. Nowadays, early diagnosis with high resolution imaging like magnetic resonance imaging along with application of prophylaxis regimens can reduce the extent of damage to the joints. However, not all haemophilia patients have access to these interventions as cost may be prohibitive for some of them. The need for new, easy, and cost-effective strategies with the ability to identify early changes could be beneficial and could make a difference in the management of haemophilic arthropathy. Understanding the mechanism of processes like angiogenesis in the mechanism of developing arthropathy could be innovative for these patients and could help in the detection of new early diagnostic and therapeutic markers.
血友病是一种遗传性出血性疾病,由于反复出血发作影响患者的肌肉骨骼系统,可导致退行性关节病。出血原因可以是创伤性的,也可以是自发性的。血友病性关节病的发病机制尚不清楚,因为铁、炎性细胞因子和血管生成因子等多种因素都参与了这一过程。关节内出血会使骨骼恶化到患者出现疼痛、活动范围减小和关节畸形的程度,这些情况会对生活质量产生很大影响。多年来,血友病性关节病的治疗方法已经发生了变化。如今,通过磁共振成像等高分辨率成像进行早期诊断,并应用预防方案,可以减少关节损伤的程度。然而,并非所有血友病患者都能获得这些干预措施,因为费用可能对他们中的一些人来说过高。需要新的、简便且具有成本效益的策略来识别早期变化,这可能有益,并可能在血友病性关节病的管理中产生重要影响。了解关节病发生机制中血管生成等过程的机制,对这些患者来说可能具有创新性,并有助于检测新的早期诊断和治疗标志物。