POEMS syndrome

Common Name(s)

POEMS syndrome

POEMS syndrome is a rare blood disorder. It is characterized by Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma proliferative disorder, and Skin changes. Signs and symptoms may include progressive sensorimotor polyneuropathy, enlarged liver and spleen, swollen lymph nodes, and darkening of the skin. Further testing and evaluation may show plasma cell disorder and sclerotic bone lesions. Many people with POEMS syndrome have diabetes (20-50%) and primary gonadal failure (55-70%). Other general signs and symptoms may include increased body hair, thickening of the skin, whitening of the nails, fatigue, weakness, swelling of the ankles and legs, enlarged breast tissue, generalized aches and pains, fluid in the lungs, and vision changes. Sign and symptoms tend to begin in a person's 40's to 50's. POEMS syndrome is a rapidly progressive disorder that can become life threatening. Currently the exact cause of POEMS syndrome is unknown.  Researchers believe the syndrome involves an overgrowth of bone marrow cells (plasma cells), which produce chemicals that damage other parts of the body.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "POEMS syndrome" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "POEMS syndrome" returned 136 free, full-text research articles on human participants. First 3 results:

Rapidly Progressive Polyneuropathy in a Patient With Monoclonal Gammopathy: A Case Report of POEMS Syndrome and Beyond.
 

Author(s): Chen Wang, Yu-Zhou Guan, Qian-Qian Cai, Wei Su, Dao-Bin Zhou, Jian Li

Journal: Medicine (Baltimore). 2016 Apr;95(16):e3453.

 

Neuropathy, the dominant clinical feature of POEMS syndrome, is typically distal, symmetric, and slowly progressive with demyelinating changes. After a gradual proximal spread, it usually results in severe muscle weakness and functional disabilities. Cases characterized by acute onset ...

Last Updated: 22 Apr 2016

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Castleman disease variant of POEMS syndrome complicated with multiple cerebral infarction: a rare case report and review of literature.
 

Author(s): Hang Yu, Fang Yao, Yue Li, Jian Li, Quan-Cai Cui

Journal:

 

POEMS syndrome is a rare hematological disorder associated with plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. Castleman disease is a lymphoproliferative disorder that can be present in POEMS patients, which ...

Last Updated: 1 Jan 2016

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Uncompacted Myelin Lamellae and Nodal Ion Channel Disruption in POEMS Syndrome.
 

Author(s): Rina Hashimoto, Haruki Koike, Mie Takahashi, Ken Ohyama, Yuichi Kawagashira, Masahiro Iijima, Gen Sobue

Journal: J. Neuropathol. Exp. Neurol.. 2015 Dec;74(12):1127-36.

 

To elucidate the significance of uncompacted myelin lamellae (UML) and ion channel disruption at the nodes of Ranvier in the polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, we evaluated sural nerve biopsy specimens from 33 patients ...

Last Updated: 20 Nov 2015

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "POEMS syndrome" returned 18 free, full-text review articles on human participants. First 3 results:

POEMS syndrome and calciphylaxis: an unrecognized cause of abnormal small vessel calcification.
 

Author(s): Nobuyuki Araki, Sonoko Misawa, Kazumoto Shibuya, Satoshi Ota, Takashi Oide, Asuka Kawano, Minako Beppu, Yukio Nakatani, Satoshi Kuwabara

Journal:

 

Calciphylaxis is a syndrome consisting of vascular calcification, thrombosis, and skin necrosis. The syndrome develops often in chronic hemodialysis patients. However, there have been several case reports on calciphylaxis in patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, ...

Last Updated: 12 Apr 2016

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POEMS syndrome: update on diagnosis, risk-stratification, and management.
 

Author(s): Angela Dispenzieri

Journal: Am. J. Hematol.. 2015 Oct;90(10):951-62.

 

POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman ...

Last Updated: 21 Sep 2015

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

Author(s): Carlos Arana, José Antonio Pérez de León, Gerardo Gómez-Moreno, Ramón Pérez-Cano, Tomás Martín Hernández

Journal:

 

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. ...

Last Updated: 2 Mar 2015

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

POEMS Syndrome Treatment With Lenalidomide
 

Status: Recruiting

Condition Summary: POEMS Syndrome

 

Last Updated: 8 Mar 2015

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Ixazomib Citrate, Lenalidomide, and Dexamethasone in Treating Patients With POEMS Syndrome
 

Status: Recruiting

Condition Summary: Plasmacytoma; POEMS Syndrome

 

Last Updated: 1 Nov 2016

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