Victor Babes University of Medicine and Pharmacy, Timisoara, Romania.
Hamostaseologie. 2009 Oct;29 Suppl 1:S39-41.
Treatment of haemophiliacs with inhibitors is of great concern in low-income countries confronting shortage in substitutive treatment. Invasive interventions on these patients represent a major challenge due to the fact that costs are significantly higher in comparison to similar procedures conducted on patients without inhibitors.
In the context of insufficient availability of clotting factor, we aimed at highlighting the experience of surgical treatment in inhibitor patients. We analyzed the indications, types of performed interventions and outcomes.
PATIENTS, METHODS: This single center, retrospective analysis has been conducted on 7 inhibitor patients registered and treated in Haemophilia Center of Timisoara over ten years (1997-2007): six patients with severe hemophilia A (3 - high titer, 3 - low titer), one patient with von Willebrand disease (low titer).Three patients developed inhibitors only after 2-5 days post surgery.
A total of 15 invasive procedures were carried out: 2 orthopedic interventions (1 arthrodesis, 1 arthroscopic synovectomy), 2 urogenital interventions (1 surgical testicular detorsion, 1 orchiectomy), 4 limb amputations (2 bilateral upper and 2 lower limb amputation), 2 pseudotumour (PT) surgery interventions, 5 drainages (2 massive pyohaemothorax, 1 drainage of shank haematoma, 1 drainage of compressive forearm haematoma, 1 drainage of thigh haematoma). Haemostasis was achieved in patients with low level inhibitors (< 5 BU/ml) with high doses of FVIII concentrates; in those with high inhibitor level (> 5 BU/ml), surgery was managed using by-passing agents. Supplementation with local fibrin glue and intravenous or local antifibrinolytic agents was given in 68.75% of interventions. Postoperative complications consisted of haemorrhagic shock in 13.33% of interventions and infection in 6.66%. Haemostatic outcome was evaluated by blood loss and duration of treatment, compared to expectations for non-inhibitor patients. The outcome was excellent and good in 66.66% of interventions, and fair in 33.33%. Discussion, conclusion: Indication of invasive procedures in haemophiliacs with inhibitors was limited to life and/or limb-threatening situations. In low-income countries, inhibitor and recovery of FVIII monitoring is mandatory in the postoperative follow-up of patients with low or no substitution prior to surgery due to false negative results at the preoperative investigation.
在凝血因子供应不足的情况下，我们旨在强调在抑制剂患者中进行外科治疗的经验。我们分析了适应证、所进行干预的类型和结果。
这是一项单中心回顾性分析，研究对象为蒂米什瓦拉血友病中心登记并治疗的 7 名抑制剂患者：6 名重型血友病 A 患者（3 名高滴度，3 名低滴度），1 名血管性血友病患者（低滴度）。其中 3 名患者仅在手术后 2-5 天出现抑制剂。
共进行了 15 次侵入性手术：2 次矫形手术（1 次关节融合术，1 次关节镜滑膜切除术），2 次泌尿生殖系统手术（1 次手术睾丸扭转复位术，1 次睾丸切除术），4 次肢体截肢术（2 次双侧上肢和 2 次下肢截肢术），2 次假性肿瘤（PT）手术，5 次引流术（2 次大量血胸，1 次胫骨血肿引流术，1 次前臂压迫性血肿引流术，1 次大腿血肿引流术）。低水平抑制剂（<5BU/ml）患者采用高剂量 FVIII 浓缩物实现止血；高水平抑制剂（>5BU/ml）患者采用旁路剂进行手术管理。68.75%的干预措施中给予局部纤维蛋白胶和静脉或局部抗纤维蛋白溶解剂补充。术后并发症包括 13.33%的干预措施发生出血性休克和 6.66%的感染。通过失血和治疗持续时间评估止血效果，并与非抑制剂患者的预期进行比较。66.66%的干预措施结果为优秀和良好，33.33%为一般。讨论、结论：抑制剂血友病患者的侵入性手术适应证限于危及生命或肢体的情况。在低收入国家，由于术前检查存在假阴性结果，因此在手术前，对于低替代或无替代的患者，术后随访中必须进行抑制剂和 FVIII 恢复监测。
