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What is POEMS Syndrome?

POEMS Syndrome is a rare disorder named for its main features: Polyneuropathy (peripheral nerve damage), Organomegaly (abnormal enlargement of organs), Endocrinopathy (damage to hormone-producing glands)/Edema, M-protein (an abnormal antibody) and Skin abnormalities (including hyperpigmentation and hypertrichosis).

Community Statistics

7 community members

1 community discussions

0 community resources

Description

Acknowledgement

Acknowledgement of POEMS Syndrome has not been added yet.

Prevalence

0.0

Synonyms

Synonyms for POEMS Syndrome has not been added yet.

Cause

A plasma cell disorder is linked to the development of the disease, but specific details about this are not known. Increased cytokinesis levels, especially vascular endothelial growth factor, may be a factor in the development of the disease as well.

Symptoms

There are varying presenting symptoms due to the wide range of organ systems affected. Frequently, patients complain of symmetric distal weakness and paresthesia, peripheral edema, and painful diarrhea. Other general symptoms include impotence, shortness of breath, gynecomastia, amenorrhea, hyperpigmentation, hypertrichosis, hyperhidrosis, Raynaud phenomenon, skin tightening, and edema. Crow-Fukase also has skin, neurological, cardiovascular, endocrine, pulmonary, and other extracutaneous manifestations. Skin thickening, hyperpigmentation, angiomas, nail changes, and hypertrichosis are some skin manifestations of the disease. Motor and sensory deficiencies, loss of temperature sensation and nociception, and diminished deep tendon reflexes are some neurological manifestations. Stroke may be a cardiovascular manifestation. Individuals will often experience endocrinopathy, hypothyroidism, abnormal glucose metabolism, adrenal insufficiency, and hypocalcemia. They may also develop restrictive lung disease, pulmonary hypertension, respiratory muscle weakness, and isolated diminished diffusing capacity. Hepatomegaly, splenomegaly, Castleman’s syndrome, and lymphadenopathy may occur.

Diagnosis

Both polyneuropathy and monoclonal plasma-proliferative disorder must be present, as well as sclerotic bone lesions, Castleman’s disease, organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy), oedema (peripheral oedema, pleural effusion, or ascites), endocrinopathy, skin changes, or papilloedema.

Diagnostic Test

Diagnostic tests of POEMS Syndrome has not been added yet

Treatment

The treatment of Crow-Fukase disease depends on the underlying plasma cell disorder and the nature of the symptoms. Most patients are treated with corticosteroids, low-dose alkylators, blood stem cell transplantation, and chemotherapy. Other treatments include surgery and radiation.

Prognosis

This disease is chronic and patients with Crow-Fukase survive an average of 8 years. However, survival times are dependent on the extent of the presentation of Crow-Fukase disease.

Tips or Suggestions

Name	Description
C	It is crucial for individuals with Cushing’s syndrome to not push themselves too hard physically, eat healthily, and monitor their mental health. Cognitive issues resulting from Cushing’s syndrome may improve through mental exercises such as Sudoku. Pain can be alleviated with low-impact exercise, hot baths, and massages.

References

References of POEMS Syndrome has not been added yet.

Other POEMS patients Created by rmarino
Last updated 18 Apr 2011, 08:50 PM

Posted by arobin
18 Apr 2011, 08:50 PM

Marino have you been diagnosed and if so by whom? Arobin

Posted by rmarino
13 Mar 2009, 10:35 PM

Looking to share and compare my experiences with POEMS with other POEMS patients.

View Full Thread

Community Resources

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Clinical Trials

CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access.

Enrolling is easy.

Complete the screening form.
Review the informed consent.
Answer the permission and data sharing questions.

After these steps, the enrollment process is complete. All other questions are voluntary. However, these questions are important to patients and their families to create awareness as well as to researchers to study rare diseases. This is why we ask our participants to update their information annually or anytime changes to their information occur.

Researchers can contact CoRDS to determine if the registry contains participants with the rare disease they are researching. If the researcher determines there is a sufficient number of participants or data on the rare disease of interest within the registry, the researcher can apply for access. Upon approval from the CoRDS Scientific Advisory Board, CoRDS staff will reach out to participants on behalf of the researcher. It is then up to the participant to determine if they would like to join the study.

Visit sanfordresearch.org/CoRDS to enroll.

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Community User List

val

My syndrome caused my addrenal glands to shut down and now I'm Adrenal Insuffient. My docs tell me without hydrocortisone I wont live.

LoriEllisforNate

I am the mother of a 39 year old son with POEMS syndrome. After 16 months of very debilitating symptoms and misdiagnosis we are now faced with researching what this disease is and what options and...

chelseac

elizabethkjaeger

arobin

suspected POEMS Syndrome can't get a dr. to dx.

Lynne

rmarino

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