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POEMS syndrome associated with polycythemia vera
1Department of Medicine, Loyola University Stritch School of Medicine, Maywood, Illinois 60153.
Cancer
|March 15, 1989
Summary
POEMS syndrome, a rare multisystem disorder, can present with polycythemia vera. This case highlights successful treatment of POEMS syndrome relapse with prednisone, improving neuropathy and skin symptoms.
Area of Science:
- Hematology
- Neurology
- Oncology
Background:
- POEMS syndrome is a rare multisystem disorder characterized by Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein (M-protein), and Skin changes.
- While myeloproliferative disorders are associated with multiple myeloma, their co-occurrence with POEMS syndrome is not well-documented.
Observation:
- A patient presented with POEMS syndrome, including polyneuropathy, splenomegaly, impotence, IgA-lambda M-protein, skin thickening, and notably, polycythemia vera.
- The patient initially responded to melphalan and prednisone but relapsed after 10 years.
Findings:
- The relapse of POEMS syndrome was successfully treated with pulse doses of prednisone alone.
- Treatment resulted in complete resolution of polyneuropathy and skin changes.
- The patient experienced a decrease in IgA levels and improvement in polycythemia vera.
Implications:
- This case suggests a potential link between POEMS syndrome and myeloproliferative disorders like polycythemia vera.
- Pulse prednisone therapy can be effective for POEMS syndrome relapse, impacting hematological parameters.
- Further research into the etiology of this association is warranted.