Knobe Karin, Berntorp Erik
Lund University, Malmö Centre for Thrombosis and Haemostasis, Skåne University Hospital, Malmö, Sweden.
J Comorb. 2011 Dec 27;1:51-59. doi: 10.15256/joc.2011.1.2. eCollection 2011.
In patients with haemophilia, regular replacement therapy with clotting factor concentrates (prophylaxis) is effective in preventing recurrent bleeding episodes into joints and muscles. However, despite this success, intra-articular and intramuscular bleeding is still a major clinical manifestation of the disease. Bleeding most commonly occurs in the knees, elbows, and ankles, and is often evident from early childhood. The pathogenesis of haemophilic arthropathy is multifactorial, with changes occurring in the synovium, bone, cartilage, and blood vessels. Recurrent joint bleeding causes synovial proliferation and inflammation (haemophilic synovitis) that contribute to end-stage degeneration (haemophilic arthropathy); with pain and limitation of motion severely affecting patients' quality of life. If joint bleeding is not treated adequately, it tends to recur, resulting in a vicious cycle that must be broken to prevent the development of chronic synovitis and degenerative arthritis. Effective prevention and management of haemophilic arthropathy includes the use of early, aggressive prophylaxis with factor replacement therapies, as well as elective procedures, including restorative physical therapy, analgesia, aspiration, synovectomy, and orthopaedic surgery. Optimal treatment of patients with haemophilia requires a multidisciplinary team comprising a haematologist, physiotherapist, orthopaedic practitioner, rehabilitation physician, occupational therapist, psychologist, social workers, and nurses. Journal of Comorbidity 2011;1:51-59.
在血友病患者中，定期使用凝血因子浓缩物进行替代治疗（预防性治疗）可有效预防关节和肌肉反复出血发作。然而，尽管取得了这一成功，关节内和肌肉内出血仍是该疾病的主要临床表现。出血最常发生在膝盖、肘部和脚踝，且往往在儿童早期就很明显。血友病性关节病的发病机制是多因素的，滑膜、骨骼、软骨和血管都会发生变化。反复的关节出血会导致滑膜增生和炎症（血友病性滑膜炎），进而导致终末期退变（血友病性关节病）；疼痛和活动受限严重影响患者的生活质量。如果关节出血得不到充分治疗，往往会复发，导致恶性循环，必须打破这种循环以防止慢性滑膜炎和退行性关节炎的发展。血友病性关节病的有效预防和管理包括早期积极使用因子替代疗法进行预防性治疗，以及选择性手术，包括恢复性物理治疗、镇痛、抽吸、滑膜切除术和矫形外科手术。血友病患者的最佳治疗需要一个多学科团队，成员包括血液科医生、物理治疗师、矫形外科医生、康复医生、职业治疗师、心理学家、社会工作者和护士。《合并症杂志》2011年；1：51 - 59。
