血友病B伴腹盆腔假肿瘤一例

A Case of Hemophilia B with Abdominal and Pelvic Pseudotumor

摘要: 血友病B是一种基因突变导致的凝血因子Ⅸ(FⅨ)缺乏性疾病，主要表现为关节、肌肉及深部组织出血。血友病假肿瘤是反复出血导致液化坏死的软组织形成的肿块，大部分假肿瘤发生在骨骼及肌肉处。本文报道了一例血友病B伴盆腹腔假肿瘤形成的患者，病变位置相对罕见，对患者进行积极有效的止血后，患者血尿症状逐渐好转。该病例提示血友病患者早期及时止血治疗是至关重要的。

Abstract: Hemophilia B is a genetic disorder caused by coagulation factor Ⅸ(FⅨ) deficiency, mainly manifesting as joint, muscle and deep tissue bleeding. Hemophilia pseudotumor is a mass formed by soft tissue liquefaction and necrosis caused by repeated bleeding. Most pseudotumors occur in the bone and muscle. We report a case of hemophilia B with pseudotumor formation in the pelvis and abdomen, where lesion location is relatively rare. After active and effective hemostasis, the patient's hematuria symptom gradually improved. This case suggests that early and timely hemostatic treatment is crucial for patients with hemophilia.