Pure Motor Axonal Neuropathy, Organomegaly, Impaired Glucose Tolerance, M Protein, Skin Changes, Multiple Plasmacytomas & Acute Interstitial Nephritis in Osteolytic Myeloma: Beyond POEMS!

溶骨性骨髓瘤中的纯运动轴索神经病变、器官肿大、葡萄糖耐量受损、M蛋白、皮肤改变、多发性浆细胞瘤和急性间质性肾炎:超越POEMS综合征!

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Abstract

The authors describe a case of 52-year-old male who presented with sudden onset deterioration of weakness of both lower limbs and retention of urine. He had 1 month history of gradually progressive weakness of legs. On examination, there were lower motor neuron signs in lower extremity, digital clubbing and a lump over left iliac fossa. Routine blood tests showed impaired glucose tolerance, confirmed by oral glucose tolerance test while renal parameters were normal. Magnetic resonance imaging of spine documented osteolytic lesions, long segment epidural mass in thoracic spine and a mass overlying the left iliac bone, both were revealed to be plasmacytoma following cytology. Ultrasonography of abdomen showed splenomegaly. Nerve conduction studies showed gross axonal, motor, asymmetric polyneuropathy with conduction block involving all the four extremities, mainly lower limbs with sensory sparing. Serum protein electrophoresis showed M spike, and bone marrow showed diffuse neoplastic plasma cell proliferation. Osteolytic lesion was present in skull radiograph. Then in the course of illness the patient developed acute renal failure due to acute interstitial nephritis as evidenced from proteinuria and kidney biopsy, which improved with steroids and chemotherapy but unfortunately we lost the patient after 2 weeks of initiation of chemotherapy.

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