Rodriguez-Merchan E Carlos
Department of Orthopaedic Surgery, La Paz University Hospital, Madrid, Spain.
Cardiovasc Hematol Disord Drug Targets. 2016;16(1):21-24. doi: 10.2174/1871529x16666160613114506.
Hematologic primary prohylaxis is the gold standard of treatment in persons with hemophilia (PWH). The goal is to reduce or prevent joint bleeds and subsequent joint degeneration (hemophilic arthropathy). In acute hemarthroses, early treatment with factor (VIII or IX) replacement and rest of the joint (4 to 5 days) are paramount. In patients with inhibitors (antibodies against factor VIII or IX) we can use bypassing agents such as activated prothrombin complex concentrate (aPCC) and recombinant factor VIIa (rFVIIa). The goal is to get the rapid resolution of the joint bleed that must be confirmed by means of ultrasonography (US). This way the risk of long-term complications will be minimized. Ice therapy could help, although its current role in hemophilia remains controversial. Pain killers (paracetamol) may also be needed. Arthrocentesis (joint aspiration) should be performed in very tense and painful joints. The procedure should always be performed under factor coverage and in aseptic conditions. Rehabilitation (physiotherapy) will help recovering the pre-bleeding full range of motion of the joint. In recurrent joint bleeds, radiosynovectomy (RS) and arthroscopic synovectomy (AS) can break the vicious cycle of hemarthrosis-synovitis-hemarthrosis. If joint damage is not avoided, it will compromise the health-related quality of life (HRQoL) of PWH.
血液学一级预防是血友病患者（PWH）治疗的金标准。目标是减少或预防关节出血及随后的关节退变（血友病性关节病）。在急性关节积血时，早期使用凝血因子（VIII 或 IX）替代治疗并让关节休息（4至5天）至关重要。对于有抑制剂（针对凝血因子VIII或IX的抗体）的患者，我们可以使用旁路制剂，如活化凝血酶原复合物浓缩剂（aPCC）和重组凝血因子VIIa（rFVIIa）。目标是使关节出血迅速消退，这必须通过超声检查（US）来确认。这样可以将长期并发症的风险降至最低。冰敷疗法可能有帮助，尽管其目前在血友病中的作用仍存在争议。可能还需要使用止痛药（对乙酰氨基酚）。对于非常紧张和疼痛的关节，应进行关节穿刺术（关节抽吸）。该操作应始终在凝血因子覆盖及无菌条件下进行。康复治疗（物理治疗）将有助于恢复关节出血前的全范围活动。在复发性关节出血中，放射性滑膜切除术（RS）和关节镜下滑膜切除术（AS）可以打破关节积血 - 滑膜炎 - 关节积血的恶性循环。如果不避免关节损伤，将会损害血友病患者的健康相关生活质量（HRQoL）。
