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POEMS syndrome.

W W T Nyunt1, R Remli, F A Abdul Muttlib

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POEMS syndrome, a rare condition causing Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, and Skin changes, requires specific diagnosis beyond typical neuropathy treatments. Early recognition of associated symptoms is key for effective management.

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Area of Science:

  • Neurology
  • Hematology
  • Rare Diseases

Background:

  • POEMS syndrome is a rare multisystem disorder characterized by Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, and Skin changes.
  • Diagnosis can be challenging due to overlapping symptoms with other conditions, often leading to delayed or incorrect initial treatment.

Observation:

  • A 65-year-old female presented with progressive sensorimotor demyelinating polyneuropathy unresponsive to standard treatments like IVIG and prednisolone.
  • Clinical presentation included hepatosplenomegaly, skin changes, polycythemia, and thrombocytosis, prompting further investigation beyond chronic inflammatory demyelinating polyradiculoneuropathy.

Findings:

  • The patient was diagnosed with POEMS syndrome based on established criteria, including polyneuropathy, lambda monoclonal plasma cell disorder, sclerotic bone lesions, organomegaly, skin changes, and thrombocytosis.
  • Treatment with melphalan and prednisolone resulted in significant clinical improvement and partial hematologic and radiologic response.

Implications:

  • This case underscores the importance of considering POEMS syndrome in patients with peripheral neuropathy refractory to standard therapies.
  • Recognizing the constellation of associated clinical manifestations is crucial for timely diagnosis and appropriate management of this rare but treatable condition.