Coon W W, Penner J A
Surgery. 1981 Oct;90(4):735-40.
The abdominal hemophilic pseudotumor is a rare but frequently disabling and life-threatening complication in patients with severe hemophilia. Our patients were observed for a considerable period of time to document progressive enlargement of the pseudotumor and increasing disability from severe pain, nerve compression, or leg swelling. The decision to operate was made on the basis of incapacitating symptomatology or fear of impending rupture. Although the complications resulting from operation may be major, with the current availability of large amounts of factor VIII and activated prothrombin complex concentrate, excision of this lesion can be performed without concern for the hazard of uncontrollable hemorrhage. Late recurrence of the pseudotumor may necessitate further operative management.
腹部血友病性假肿瘤是严重血友病患者中一种罕见但常导致功能障碍和危及生命的并发症。我们对患者进行了相当长一段时间的观察,以记录假肿瘤的逐渐增大以及因剧痛、神经受压或腿部肿胀导致的功能障碍加剧。手术决策基于症状严重影响功能或担心即将破裂。尽管手术可能会引发重大并发症,但鉴于目前可获得大量的凝血因子 VIII 和活化凝血酶原复合物浓缩剂,切除该病变时无需担心不可控出血的风险。假肿瘤的晚期复发可能需要进一步的手术治疗。