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Rare disease
Polyneuropathy: a poetic diagnosis
  1. Reena Pattani1,
  2. Dmitry Rozenberg2,
  3. Lee Mozessohn1,
  4. Lisa Hicks3
  1. 1Department of Medicine, University of Toronto, Toronto, Canada
  2. 2Department of Medicine, Division of Respirology, University of Toronto, Toronto, Canada
  3. 3Department of Medicine, Division of Hematology and Oncology, St. Michael’s Hospital, University of Toronto, Toronto, Canada
  1. Correspondence to Dr Reena Pattani, reena.pattani{at}utoronto.ca

Summary

A 60-year-old woman presented with subacute progressive lower extremity weakness. This was associated with a 40lb weight loss and fevers. She was previously healthy, aside from renal transplantation 9 years earlier for end stage renal disease resulting from IgA nephropathy. On examination, she had clubbing, a single lymph node in the right cervical chain, and objective findings of lower extremity weakness with lower motor neuron findings. Investigations revealed a thrombocytosis and elevated CSF protein. Electromyographic evidence of diffuse polyradiculopathy with demyelination was documented. On imaging, she had a large lytic/sclerotic lesion in the right scapula measuring 8 cm, a pericardial effusion, and borderline splenomegaly. Pathological assessment of the excised lymph node revealed Castleman-like changes; biopsy of the right scapular lesion revealed λ-restricted plasma cells, in the absence of a monoclonal protein in the bone marrow or periphery. A diagnosis of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) with plasmacytoma was established; a post-transplant lymphoproliferative disorder was suspected.

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Footnotes

  • Competing interests None.

  • Patient consent Obtained.