Manickavachakan Narender, Ellur Sunderraj, Mattyoo Joseph Vijay Thomas, Victor Jonathan, Ross Cecil R
Department of Plastic Surgery, St. John's Medical College and Hospital, Bengaluru, Karnataka, India.
Department of Medicine, St. John's Medical College and Hospital, Bengaluru, Karnataka, India.
Indian J Plast Surg. 2017 May-Aug;50(2):213-216. doi: 10.4103/ijps.IJPS_214_16.
Haemophilia A is a rare haematological disorder due to deficiency of Factor VIII, causing an abnormal coagulation response to injury. In severe haemophilia A, Factor VIII level is < 1%, often manifesting with spontaneous bleeding into joints. Judicious use of recombinant Factor VIII therapy to maintain adequate levels in the intraoperative, immediate and late post-operative periods, together with adjuvant pro-coagulants, can ensure a safe outcome following surgery. We describe the successful management of one such patient suffering from Marjolin's ulcer of the right gluteal region, who needed wide local excision followed by flap cover. A protocol for management of such patients is also suggested. This is the first such case report from the Indian subcontinent, with only a few such published reports from the West.
甲型血友病是一种罕见的血液系统疾病，由于缺乏凝血因子VIII，导致对损伤的凝血反应异常。在严重的甲型血友病中，凝血因子VIII水平<1%，常表现为关节自发性出血。在术中、术后即刻和后期明智地使用重组凝血因子VIII疗法以维持足够的水平，同时使用辅助促凝剂，可以确保手术有安全的结果。我们描述了一名患有右臀区域Marjolin溃疡的此类患者的成功治疗，该患者需要进行广泛局部切除并随后进行皮瓣覆盖。还提出了此类患者的管理方案。这是印度次大陆的首例此类病例报告，西方仅发表了少数此类报告。
