Department of Thrombosis and Hemostasis, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China.
Clin Appl Thromb Hemost. 2011 Jun;17(3):279-82. doi: 10.1177/1076029610366433. Epub 2010 May 11.
Pseudotumor is an uncommon but severe complication in patients with hemophilia. To our knowledge, although China has large population of persons with hemophilia, there is rare information on the incidence, clinical feature, image finding, and management of pseudotumor among Chinese patients. This study aimed at improving our knowledge on clinical diagnosis and management of hemophiliac pseudotumor. In this retrospective study, the medical records of 1248 patients with hemophilia diagnosed between January 1983 and October 2004 at our hospital were reviewed. The clinical feature, imaging finding, management, and outcome of 14 patients with pseudotumor among these patients with hemophilia were analyzed. All patients have hemophilia A (8 severe cases and 6 moderate cases). Eight patients sustained an injury prior to the development of pseudotumor. Main image findings included osteolysis lesion, soft tissue swelling, or lump. Surgical therapy was carried out in 7 patients and 6 achieved remission, with fistula formation remaining in 1. One patient underwent radiotherapy together with replacement therapy achieved remission. Three patients accepted replacement therapy as only management and only 1 patient achieved improvement of swelling. Our study showed that the incidence of pseudotumor in our enrolled patients with hemophilia is 1.12%. Hemophilic history of patients can contribute to the right diagnosis of pseudotumor. Surgical therapy together with sufficient replacement therapy is safe and effective.
假瘤是血友病患者中一种不常见但严重的并发症。据我们所知，尽管中国有大量的血友病患者，但关于中国患者假瘤的发病率、临床特征、影像表现和治疗的信息很少。本研究旨在提高我们对血友病性假瘤临床诊断和治疗的认识。在这项回顾性研究中，我们回顾了 1983 年 1 月至 2004 年 10 月期间在我院诊断的 1248 例血友病患者的病历。分析了其中 14 例假瘤患者的临床特征、影像学表现、治疗和转归。所有患者均为血友病 A（8 例重型，6 例中型）。8 例患者在假瘤发生前有外伤史。主要影像学表现包括溶骨性病变、软组织肿胀或肿块。7 例患者接受了手术治疗，6 例患者缓解，1 例患者仍存在瘘管形成。1 例患者接受放疗和替代治疗缓解，3 例患者仅接受替代治疗，仅 1 例患者肿胀改善。我们的研究表明，纳入的血友病患者中假瘤的发病率为 1.12%。患者的血友病病史有助于假瘤的正确诊断。手术治疗联合充分的替代治疗是安全有效的。
