Overview
POEMS syndrome is a rare plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein (M protein), and skin changes 167.Diagnosis
Key Diagnostic Criteria: Polyneuropathy, presence of M protein, organomegaly, endocrinopathy, and skin changes 167.
Recommended Tests:
- Bone marrow biopsy to identify plasma cell dyscrasia 14.
- Electrophoresis and immunofixation for M protein detection 1.
- Imaging studies (e.g., MRI) to assess organomegaly and spinal involvement 1.
- Electrodiagnostic studies (e.g., electroneuromyography) for polyneuropathy 1.
- Endocrinological evaluation to identify endocrine abnormalities 15.Management
First-Line Treatments:
- Corticosteroids for symptom management 14.
- Radiation therapy for localized plasmacytomas 14.
Adjunctive Treatments:
- Immunomodulatory drugs (e.g., thalidomide, lenalidomide) 1 (specific doses not detailed).
- Stem cell transplantation in selected cases 14.Special Populations
Comorbidities: Concurrent cervical disc herniation may complicate diagnosis and require surgical intervention alongside POEMS syndrome management 1.
Diverse Presentations: POEMS syndrome can present atypically in younger patients or those with systemic lupus erythematosus 45.Key Recommendations
Evaluate patients with atypical neurological signs and systemic symptoms for concurrent POEMS syndrome, especially in the presence of cervical spondylopathy 1 (Evidence: Moderate).
Consider bone marrow biopsy and comprehensive endocrine evaluation in suspected POEMS syndrome cases 15 (Evidence: Moderate).
Aggressive treatment including corticosteroids, radiation, and potentially stem cell transplantation may improve outcomes in POEMS syndrome 14 (Evidence: Weak).References
1 Ling T, Liu L, Song Y, Zhou S, Zhou C. Neurological impairment in a patient with concurrent cervical disc herniation and POEMS syndrome. European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society 2019. link
2 Thakral S, Issa NP, Barboi AC, Lee JM. Prominent dysautonomia in a patient with POEMS syndrome. Clinical autonomic research : official journal of the Clinical Autonomic Research Society 2016. link
3 Ho KK. Endocrinology: the next 60 years. The Journal of endocrinology 2006. link
4 Wong VA, Wade NK. POEMS syndrome: an unusual cause of bilateral optic disk swelling. American journal of ophthalmology 1998. link00106-8)
5 Murphy N, Schumacher HR. POEMS syndrome in systemic lupus erythematosus. The Journal of rheumatology 1992. link
6 Prasad K, Ahuja GK, Goulatia RK, Sood A, Dawar R, Behari M. POEMS syndrome. The Journal of the Association of Physicians of India 1991. link
7 Walters JP, Irvine RA, Mohammed H. The POEMS syndrome. The West Indian medical journal 1991. link