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Washington University Experience | PERIPHERAL NEUROPATHY | 13 POEMS SYNDROME | 1A0 Case 1 History - Copy
Case 1 History ---- The patient is a 74-year-old female presenting in October 2022 with bilateral foot pain, progressive proximal and distal weakness, loss of sensation, areflexia and, shortly thereafter, severe bilateral pitting edema. EMG/NCS in 6/2023 showed evidence of a mixed axonal and demyelinating peripheral polyneuropathy. VEGF was 32, repeated a month later was 95/pg/ml (typical normal 30-90 but quite variable). Computed Tomography of the Chest, Abdomen, and Pelvis showed no organomegaly. An X ray survey was negative for osseus lesions although there was a chondroid abnormality. She underwent muscle and sural nerve biopsy which showed evidence of moderate chronic loss of myelinated axons. In 1/2024, her exam had worsened. CT chest/abdomen/pelvis showed splenomegaly, possible hepatomegaly, new retroperitoneal lymphadenopathy, and an unchanged chondroid lesion in the right iliac area. PET CT showed intense FDG uptake by the lesion in the right iliac wing. A bone marrow biopsy was performed with a target of the right iliac lesion, which showed a lambda restricted plasma cell neoplasm. Serum immunotyping revealed IgG lambda paraprotein, IgA <50 mg/dL, IgG 2670 mg/dL and IgM 159 mg/dL She was treated with Rituximab in 11/2023. Radiation therapy (40 Gy in 20 fractions) was delivered to the right iliac lesion over 4 weeks beginning at the end of March 2024
