Arias Manuel, Villalba Carmen, Requena Ignacio, Vázquez-Veiga Hugo, Sesar Angel, Pereiro Inmaculada
Services of Neurology, Hospital de Conxo (CHUS), Santiago de Compostela, Spain.
Spine (Phila Pa 1976). 2004 Apr 15;29(8):E161-3. doi: 10.1097/00007632-200404150-00025.
Systemic mastocytosis is a mast cell proliferative disorder affecting many organs that is rarely associated with internal bleeding.
To describe a case of spinal epidural hematoma in a patient with past medical history of urticaria pigmentosa and osteoporosis diagnosed with systemic mastocytosis.
A 63-year-old woman with urticaria pigmentosa was admitted to hospital for severe back pain after minor trauma. Physical examination showed pain on pressing T12 and L1 spinous processes, bilateral Lasègue sign, absent ankle jerk, and extensor plantar response. Computed tomography disclosed L3 fracture, and magnetic resonance imaging revealed spinal epidural hematoma and T2 hyperintensive scattered vertebral foci that suggested malignancy. The 24-hour urine histamine was very high. Mast cell infiltration was found in bone marrow biopsy. Because power was normal and there was no clinical sphincter disorder, the patient was successfully treated with conservative care.
To our knowledge, acute intraspinal epidural hematoma has never been associated with mastocytosis. The hematoma was likely related to the vertebral fracture as well as a hemorrhagic diathesis due to anticoagulants released by local mast cells.
系统性肥大细胞增多症是一种影响多个器官的肥大细胞增殖性疾病，很少与内出血相关。
描述一例既往有色素性荨麻疹和骨质疏松症病史且诊断为系统性肥大细胞增多症的患者发生脊柱硬膜外血肿的病例。
一名患有色素性荨麻疹的63岁女性因轻微外伤后严重背痛入院。体格检查显示T12和L1棘突压痛、双侧拉塞格征阳性、跟腱反射消失及跖伸反射阳性。计算机断层扫描显示L3骨折，磁共振成像显示脊柱硬膜外血肿及T2高信号散在椎体病灶，提示恶性病变。24小时尿组胺水平非常高。骨髓活检发现肥大细胞浸润。由于患者凝血功能正常且无临床括约肌功能障碍，因此成功接受了保守治疗。
据我们所知，急性脊髓硬膜外血肿从未与肥大细胞增多症相关联。该血肿可能与椎体骨折以及局部肥大细胞释放的抗凝剂导致的出血素质有关。
