四肢血友病假瘤及血友病关节炎的外科治疗意义  

Surgical treatment for hemophilic pseudotumors and arthritis of limbs

作　　者：张伟滨[1] 徐建强[1] 万荣[1] 沈才伟[2] 杨庆铭[2] 

机构地区：[1]上海交通大学医学院附属瑞金医院骨科,上海200025 [2]上海市伤骨科研究所,上海200025

出　　处：《中国骨与关节外科》2009年第6期448-452,共5页Chinese Journal of Bone and Joint Surgery

摘　　要：目的探讨外科手术在四肢肌肉骨骼系统血友病假瘤及血友病关节炎治疗中的意义。方法四肢肌肉骨骼血友病假瘤及血友病关节炎患者43例,均为男性,年龄11～51岁,平均(28.00±11.54)岁,其中甲型血友病39例,乙型血友病4例,分别于浓缩抗血友病球蛋白(AHG)和凝血酶原复合物制剂(PCC)的严格替代治疗保护下,接受假瘤切除、截肢、关节置换术、关节融合等手术。结果在正规替代疗法保护下,43例患者中40例术后2周伤口一期愈合,另3例甲型血友病患者术后伤口感染、窦道形成,经伤口换药、再清创,最终愈合,愈合时间:1例为3周,1例为6周,另1例伤口未完全愈合即出院,4个月后告知完全愈合。伤口愈合不良率为6.98%,未发现此3例患者术后伤口愈合不良与血浆Ⅷ因子浓度过低相关。2例血友病假瘤复发,再次手术切除,复发率为4.65%。因诊断不明,在无替代疗法情况下误行病灶活检术3例,病灶刮除植骨术1例,血肿引流术2例,最后均经血浆凝血因子Ⅷ浓度测定确诊为甲型血友病。结论在严格的替代疗法保护下,对生长迅速、压迫重要血管和神经、肢体缺血性改变、反复关节出血伴慢性滑膜炎、病理性骨折、窦道出血感染且替代疗法无效的四肢血友病假瘤,外科手术是避免假瘤进行性增大、溃破感染、防止骨关节进一步损坏、挽救有功能肢体、抢救患者生命的最有效治疗手段。Objective To discuss the scientific significance of surgical treatment for hemophilic pseudotumors and arthritis of limbs.Methods 43 hemophiliacs with pseudotumors or hemophilic arthritis in limbs,in which 39 was hemophilia A and 4 hemophilia B,were surgically treated under the protection of strict factor-deficient replacement therapy using antihemophilic globulin（AHG） for hemophilia A or prothrombin complex concentrate（PCC） for hemophilia B.The types of surgery were pseudotumor resection,amputation,arthrodeses,total knee replacement,and so on.Results Under the protection of the factor-deficient replacement therapy,40 of 43 hemophiliacs had I stage incision healing in 2 weeks postoperatively.The other 3 hemophilia A patients who had suffered wound infection and sinus formation postopera- lively were eventually restored by continued supplement of factorⅧand wound care and debridement.The wound healing failure rale was 6.98%.No relationship could be found between wound healing problem and lower plasma concentration of factorⅧ.2 cases recurred hemophilic pseudotumors at previous sites.The recurrent rate was 4.65%.3 biopsies,1 curettage of the lesion and 2 drainage for heamatomas were done without the protection of factor-deficient replacement therapy because of preoperative misdiagnoses.Increased bleeding occurred during operation in 4 of them.All of those 6 patients were finally diagnosed hemophilia A by testing their plasmatic concentrations of factorⅧ.Conclusion Under cover of factor-deficient replacement therapy,for hemophilic pseudotumors which expanded rapidly,compressed on vital vessels and nerves,repeated uncontrollable hemarthroses and chronic synovitis,combined pathological fracture and sinus bleeding, surgical management was the most effective way in avoiding progressive expanding of pseudotumor and infected sinus formation,preventing from further destruction of involved bone and joint,saving a functional limb and even the patients life.

关 键 词：血友病 假瘤 替代疗法 手术治疗 

分 类 号：R554.1[医药卫生—血液循环系统疾病] R687.4[医药卫生—内科学]