Joint protection in haemophilia
Joint protection in haemophilia
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DOI：
10.1111/j.1365-2516.2011.02615.x
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发表时间：
2011-11-01
期刊：
影响因子：
3.9
通讯作者：
Caffarini, A.
中科院分区：
文献类型：
--
作者：
Rodriguez-Merchan, E. C.;Jimenez-Yuste, V.;Caffarini, A.
关节出血（关节内出血）是血友病患者常见的结果。必须尽早诊断和治疗这些出血事件。此外，理想情况下，治疗应集中管理（加强按需治疗），直到症状消失。关节抽吸在急性和大量关节积血中起着重要作用，因为关节中血液的存在导致软骨细胞凋亡和慢性滑膜炎，最终导致关节退行性变（血友病性关节病）。尽管就成像技术而言，磁共振成像仍然是金标准，但超声检查（US）是评估血友病急性关节积血演变的适当诊断技术。有些患者会出现亚临床关节积血，最终导致一定程度的关节病，尤其是踝关节。如今，保护这些患者的最有效方法是一级预防，在实践中将重度血友病转变为中度血友病，预防或至少最大限度地减少关节积血的发生。如果一级预防，无论出于何种原因，不是一种选择，二级预防和加强按需治疗应考虑。抑制物患者有两种替代方案：（i）根据与非抑制物患者相同的基本原则，使用旁路药物（rFVIIa或aPCC）控制止血，作为加强按需治疗或二级预防（如适用），以及（ii）免疫耐受诱导（ITI）以根除抑制物。
Haemarthroses (intra-articular haemorrhages) are a frequent finding typically observed in patients with haemophilia. Diagnosis and treatment of these bleeding episodes must be delivered as early as possible. Additionally, treatment should ideally be administered intensively (enhanced on-demand treatment) until the resolution of symptoms. Joint aspiration plays an important role in acute and profuse haemarthroses as the presence of blood in the joint leads to chondrocyte apoptosis and chronic synovitis, which will eventually result in joint degeneration (haemophilic arthropathy). Ultrasonography (US) is an appropriate diagnostic technique to assess the evolution of acute haemarthrosis in haemophilia, although magnetic resonance imaging remains the gold standard as far as imaging techniques are concerned. Some patients experience subclinical haemarthroses, which eventually tend to result in some degree of arthropathy, especially in the ankles. Nowadays, the most effective way of protecting these patients is primary prophylaxis, which in practice changes severe haemophilia into moderate haemophilia, preventing or at least minimizing the occurrence of haemarthrosis. If primary prophylaxis is, for whatever reason not an option, secondary prophylaxis and enhanced on demand treatment should be considered. Two alternatives are available for inhibitor patients: (i) control of haemostasis using by-passing agents (rFVIIa or aPCCs) either as enhanced on demand treatment or secondary prophylaxis, as appropriate, following the same basic principles used for non-inhibitor patients and (ii) immune tolerance induction (ITI) to eradicate the inhibitor.