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POEMS neuropathy: optimising diagnosis and management
Stephen Keddie1,2,3, Shirley D'Sa4, David Foldes4
1MRC Centre for Neuromuscular Diseases, National Hospital for Neurology and Neurosurgery, London, UK.
Practical Neurology
|March 8, 2018
Summary
POEMS syndrome, a rare autoinflammatory disorder, presents with neuropathy and plasma cell issues. Early diagnosis is crucial to prevent debilitating symptoms and ineffective treatments for this multisystem condition.
Area of Science:
- Neurology
- Immunology
- Hematology
Background:
- POEMS syndrome is a rare, disabling autoinflammatory condition.
- It is characterized by peripheral neuropathy and a monoclonal plasma cell disorder.
- The acronym POEMS encompasses its multisystem features: polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin disease.
Purpose of the Study:
- To present typical features of POEMS syndrome.
- To highlight diagnostic challenges and clinical conundrums.
- To provide an investigative guide and treatment algorithm for clinicians.
Main Methods:
- Review of a large POEMS cohort at University College London Hospitals.
- Analysis of clinical presentations and diagnostic variations.
- Case studies to illustrate diagnostic difficulties.
Main Results:
- POEMS diagnosis is challenging due to heterogeneous presentations.
- Patients often face misdiagnosis and delayed treatment.
- Ineffective immunomodulatory therapies may be administered before correct diagnosis.
Conclusions:
- Accurate and timely diagnosis of POEMS syndrome is critical.
- A structured investigative approach is needed for suspected cases.
- Treatment should be tailored to the monoclonal plasma cell proliferation.
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