The First College of Clinical Medicine, Zhejiang Chinese Medical University, Hangzhou, Zhejiang Province, China.
Department of Orthopedic Surgery, The First Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou, Zhejiang Province, China.
Mol Biol Rep. 2021 Jan;48(1):969-974. doi: 10.1007/s11033-020-06052-8. Epub 2020 Dec 8.
Haemophilic arthropathy (HA), caused by intra-articular haemorrhage, is one of the most common complications in patients with haemophilia. Factor replacement therapy provides missing coagulation factors to prevent children with haemophilia from joint bleeding and decreases their risk for HA. However, haemophilia patients in developing countries are still suffering from HA due to insufficient replacement therapy. Symptoms such as pain and activity limitations caused by HA seriously affect the functional abilities and quality of life of patients with HA, causing a high disability rate in the haemophilia cohort. The pathological mechanism of HA is complicated because the whole pathological mainly involves hypertrophic synovitis, osteopenia, cartilage and bone destruction, and these pathological changes occur in parallel and interact with each other. Inflammation plays an important role in the whole complex pathological process, and iron, cytokines, growth factors and other factors are involved. This review summarizes the pathological mechanism of HA to provide background for clinical and basic research.
血友病性关节病（HA）是由关节内出血引起的，是血友病患者最常见的并发症之一。因子替代疗法提供缺失的凝血因子，以防止血友病儿童关节出血，并降低他们发生 HA 的风险。然而，发展中国家的血友病患者仍因替代疗法不足而遭受 HA 的困扰。HA 引起的疼痛和活动受限等症状严重影响 HA 患者的功能能力和生活质量，导致血友病患者的残疾率较高。HA 的病理机制很复杂，因为整个病理主要涉及肥厚性滑膜炎、骨质疏松、软骨和骨破坏，这些病理变化同时发生并相互作用。炎症在整个复杂的病理过程中起着重要作用，涉及铁、细胞因子、生长因子等因素。本文综述了 HA 的病理机制，为临床和基础研究提供背景。
