Farrugia Daniel, Camilleri David James, Azzopardi Janice, Camilleri Franco
Haematology, Mater Dei Hospital, Msida, Malta
Haematology, Mater Dei Hospital, Msida, Malta.
BMJ Case Rep. 2019 Dec 3;12(12):e230284. doi: 10.1136/bcr-2019-230284.
We present the case of a previously well, 70-year-old lady who presented with gait deterioration and a clinicoradiological picture of myositis with T2-enhancement on MRI of the distal lower limb muscles. A muscle biopsy pointed towards neurogenic amyotrophy and a mixed demyelinating and axonal sensorimotor polyneuropathy was confirmed on nerve conduction studies. This was initially thought to be secondary to a myeloproliferative disorder but a positron emission tomography scan showed uptake in the left hip which corresponded to what was previously reported as a subchondral cyst on CT. A biopsy showed this to be a plasmacytoma in the context of a normal serum protein electrophoresis and a polyclonal increase in light chains on serum-free light chain estimation. The patient was started on treatment 6 months after the presentation for a diagnosis of POEMS syndrome, by which time, the patient had become bed-bound and fully dependent.
我们报告一例既往健康的70岁女性病例，该患者出现步态恶化，下肢远端肌肉MRI显示T2增强的临床放射学表现，提示为肌炎。肌肉活检提示神经源性肌萎缩，神经传导研究证实存在脱髓鞘和轴索性感觉运动性多神经病混合表现。最初认为这是继发于骨髓增殖性疾病，但正电子发射断层扫描显示左髋部有摄取，与之前CT报告的软骨下囊肿相符。活检显示这是一个浆细胞瘤，而血清蛋白电泳正常，血清游离轻链估计显示轻链多克隆增加。患者在出现症状6个月后开始接受治疗，诊断为POEMS综合征，此时患者已卧床不起，完全依赖他人。
