Danesh F R, Klinkmann J, Yokoo H, Ivanovich P
Northwestern University Medical School and VA Chicago Health Care System, Lakeside Divison, IL 60611, USA.
Am J Kidney Dis. 1999 Mar;33(3):563-6. doi: 10.1016/s0272-6386(99)70195-2.
Destructive spondyloarthropathy is a serious complication in patients with end-stage renal disease. We report a case of fatal cervical spondyloarthropathy in a patient on hemodialysis who presented with severe pain in the cervical area. Magnetic resonance imaging (MRI) of the cervical spine showed a soft tissue mass at the cervico-occipital hinge with spinal cord compression and destructive lesions of the cervical vertebrae. The patient became quadriplegic during the MRI procedure and died a few days later. Postmortem examination showed deposition of beta2-microglobulin in the cervico-occipital hinge. A unique feature of this case was the documented presence of systemic beta2-microglobulin amyloid deposits involving the spleen that to our knowledge has not been reported previously. Clinical suspicion and early detection of lesions caused by dialysis-related amyloidosis (DRA) may help to prevent significant morbidity and mortality in long-term dialysis patients.
破坏性脊柱关节病是终末期肾病患者的一种严重并发症。我们报告一例接受血液透析的患者发生致命性颈椎关节病,该患者出现颈部严重疼痛。颈椎磁共振成像(MRI)显示颈枕关节处有一软组织肿块,伴有脊髓受压及颈椎破坏性病变。患者在MRI检查过程中出现四肢瘫痪,数日后死亡。尸检显示颈枕关节处有β2-微球蛋白沉积。该病例的一个独特特征是记录到系统性β2-微球蛋白淀粉样沉积物累及脾脏,据我们所知,此前尚未有过相关报道。临床怀疑并早期发现透析相关淀粉样变性(DRA)所致病变,可能有助于预防长期透析患者出现严重的发病和死亡情况。