King Joseph J, Crawford Eileen A, Iwenofu O Hans, Fox Edward J
Department of Orthopaedic Surgery, University of Pennsylvania, Philadelphia, PA 19106-6192, USA.
Clin Orthop Relat Res. 2007 Jun;459:263-9. doi: 10.1097/BLO.0b013e3180487ced.
Systemic mastocytosis is characterized by clonal proliferation of mast cells, which cause disease by accumulating in various organs and releasing their chemical products into tissues. We highlight a patient with systemic mastocytosis that caused a pathologic femur fracture in a 63-year-old man with minimal trauma. The typical presentation of bone involvement is a combination of mixed sclerotic and osteolytic lesions, but this patient also had osteopenia. Although bone involvement is common in patients with systemic mastocytosis, pathologic fractures outside the vertebral column are rare. The patient was treated with intramedullary fixation of his femur and retained adequate fixation at the time of his death 7 months postoperatively. We review the literature on pathologic fractures outside the vertebral column in patients with systemic mastocytosis.
系统性肥大细胞增多症的特征是肥大细胞的克隆性增殖，这些肥大细胞通过在各个器官中积聚并将其化学产物释放到组织中而引发疾病。我们重点介绍了一名患有系统性肥大细胞增多症的患者，该疾病在一名63岁男性身上导致了病理性股骨骨折，而其受伤程度极小。骨受累的典型表现是硬化性病变和溶骨性病变混合出现，但该患者还伴有骨质减少。虽然骨受累在系统性肥大细胞增多症患者中很常见，但脊柱外的病理性骨折却很罕见。该患者接受了股骨髓内固定治疗，术后7个月死亡时仍保持了足够的固定。我们回顾了关于系统性肥大细胞增多症患者脊柱外病理性骨折的文献。
