Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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March 2, 2015
POEMS Syndrome
- POEMS syndrome is a poorly understood entity characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein (monoclonal plasma cell proliferative disorder), and skin changes.
- Etiology not established, likely secondary to imbalance of inflammatory cytokines.
- Clinical Presentation: Symmetrical and ascending peripheral neuropathy most common manifestation, pulmonary manifestations (PAH, restrictive lung disease), endocrinopathy (most commonly gonadal axis). Skin changes include scleroderma, hyperpigmentation, hemangioma/telangiectasia.
- Key Diagnostic Features:
- Radiographic findings of POEMS syndrome are nonspecific.
- Almost always associated with sclerotic bone lesions
- Organomegaly (hepatomegaly, splenomegaly) may be noted.
- Lymphadenopathy (including Castleman's disease) is a common feature.
-
- Lymphoma
- Osteoblastic metastatic disease
- Multiple myeloma
- Rx:
- Multisystem disease, no standardized therapy.
- Radiation may be utilized in a solitary bone lesion.
- High-dose chemotherapy with autologous stem cell support.
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