Thakker S, McGehee W, Quismorio F P
Arthritis Rheum. 1986 Jun;29(6):808-11. doi: 10.1002/art.1780290617.
A 47-year-old man with congenital factor XIII deficiency developed recurrent hemarthrosis of the right ankle. He had radiologic evidence of permanent joint damage. A review of the literature revealed that hemarthrosis is not a rare manifestation of factor XIII deficiency; however, destructive changes in the joints are relatively uncommon in this disorder, in contrast with those changes seen in patients with classic hemophilia and arthropathy. The bleeding manifestations of factor XIII deficiency are readily correctable by plasma or cryoprecipitate infusions; this may account for the preservation of joint function and structure.
一名患有先天性因子 XIII 缺乏症的 47 岁男性出现了右踝关节复发性关节积血。他有永久性关节损伤的影像学证据。文献回顾显示，关节积血并非因子 XIII 缺乏症的罕见表现；然而，与经典血友病和关节病患者所见的变化相比，该疾病中关节的破坏性变化相对不常见。因子 XIII 缺乏症的出血表现可通过输注血浆或冷沉淀轻松纠正；这可能是关节功能和结构得以保留的原因。
