POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) treated with autologous hematopoietic stem cell transplantation: a case report and literature review

The American Journal of Case Reports
Carlos AranaTomás Martín Hernández

Abstract

POEMS syndrome is a rare systemic pathology of paraneoplastic origin that is associated with plasma cell dyscrasia. It is characterized by the presence of sensorimotor polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes, and other systemic manifestations. The pathogenesis of the syndrome is unknown but over-production of vascular endothelial growth factor is probably responsible for most of the more characteristic symptoms. There is no standard treatment for POEMS syndrome and no randomized controlled clinical trials of treatment exist in the available literature. High-dose melphalan with autologous hematopoietic stem cell transplantation should be considered for younger patients with widespread osteosclerotic lesions, and for patients with rapidly progressive neuropathy. This is the case of a 62-year-old Caucasian man who was admitted to our center presenting pretibial edema accompanied by significant weight loss and difficulty walking. POEMS criteria were present and an immunofixation test confirmed the presence of a monoclonal plasmaproliferative disorder. After autologous hematopoietic stem cell transplantation, the monoclonal component disappeared and the patient's clinical status improved mar...Continue Reading

References

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Nov 29, 2002·Blood·Angela DispenzieriMorie A Gertz
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Aug 29, 2009·European Journal of Internal Medicine·Olivier DecauxBernard Grosbois
Jan 28, 2014·Clinical Lymphoma, Myeloma & Leukemia·Chiaki Nakaseko

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Citations

Sep 4, 2017·Journal of Neurology, Neurosurgery, and Psychiatry·Joachim BurmanHåkan Askmark

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Methods Mentioned

BETA
biopsy

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