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Review Article Volume 5 Issue 4
A review: crow–fukase syndrome/poems syndrome
Purohit Saraswati
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Department of Mental Health Nursing, JSS College of Nursing, India
Correspondence: Purohit Saraswati, Department of Mental Health Nursing, JSS College of Nursing, India, Tel 7204256844
Received: December 19, 2017 | Published: July 19, 2018
Citation: Saraswati P. A review: crow–fukase syndrome/poems syndrome. Nurse Care Open Acces J. 2018;5(4):229-230. DOI: 10.15406/ncoaj.2018.05.00152
Abstract
POEMS syndrome is a paraneoplastic syndrome whose acronym stands for less than half of the defining features of the disease, that is, polyradiculoneuropathy, organomegaly, potentially including coexisting Castleman disease, endocrinopathy, monoclonal plasma cell neoplasm, and skin changes. POEMS syndrome is caused by an underlying plasma cell disorder. The diagnosis of POEMS syndrome: The two mandatory criteria PLUS ≥ 1 major AND ≥ 1 minor criterion. Patients with POEMS syndrome are treated with medical, surgical and adjuvant therapies.
Keywords: paraneoplastic, castleman disease, endocrinopathy, organomegaly, polyneuropathy
Introduction
POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes) is characterized by the presence of a monoclonal plasma cell disorder, peripheral neuropathy, and Disease features are: osteosclerotic myeloma, Castleman's disease, elevation of serum vascular endothelial growth factor (VEGF), organomegaly, endocrinopathy, edema, typical skin changes, and papilledema.1
Definition: POEMS syndrome is a paraneoplastic syndrome whose acronym stands for less than half of the defining features of the disease, that is, polyradiculoneuropathy, organomegaly, potentially including coexisting Castleman disease, endocrinopathy, monoclonal plasma cell neoplasm, and skin changes.2
Causes: POEMS syndrome is a rare paraneoplastic syndrome that is caused by an underlying plasma cell disorder.3
Signs and symptoms:4
- Polyneuropathy. Numbness, tingling in hands and difficulty in breathing
- Organomegaly. Spleenomegaly, nodes in lymph or liver.
- Endocrinopathy. Hypothyroidism, diabetes, sexual problems weakness and metabolic problems.
- Skin changes. More pigmentation, thicker skin and hair over body.
Diagnosis
The diagnosis of POEMS syndrome: The two mandatory criteria PLUS ≥ 1 major AND ≥ 1 minor criterion.5
Mandatory criteria
- Polyneuropathy (typically demyelinating)
- Monoclonal plasma cell proliferation.
Major criteria
- Castleman disease
- Sclerotic bone lesions
- Vascular endothelial growth factor (VEGF) elevation
- Minor criteria
- splenomegaly, hepatomegaly, or lymphadenopathy
- edema, pleural effusion, or ascites
- adrenal, pituitary, gonadal, parathyroid, thyroid and pancreatic
- Skin changes
- Papilledema
- Thrombocytosis
In addition laboratory tests such as EMG for neuropathy, CT scan, bone marrow biopsy to detect clonal plasma cells, plasma or serum protein electrophoresis to myeloma proteins, Raised blood levels of VEGF, thrombocytes, and/or erythrocyte parameters these are the supportive diagnosis for POEMS syndrome.6
Treatment
The treatment of POEMS syndrome depends on the treatment of the underlying plasma cell disorder. And also treated with medical, surgical and adjuvant therapies. Patients are treated with combination of corticosteroids, low-dose alkylators, and peripheral blood stem cell transplantation following high-dose chemotherapy. Caution should be taken in selecting chemotherapeutic regimen to avoid worsening of the disease. A 2014 multicenter retrospective study in Japan has shown positive results for autologous stem cell transplantation (ACST) in the treatment of patients with POEMS syndrome in terms of long-term survival and quality of life.7
POEMS Syndrome at current News
NBC 10 philadelphia-15-Nov-2017: Action News anchor Thomas-Laury suffered from a rare condition called POEMS syndrome that paralyzed her vocal cords, bound her to a wheelchair and required two bone marrow transplants. Then, she became addicted to the opioid prescribed to treat her chronic pain.8
Conclusion
By this review article I got an opportunity to review about POEMS Syndrome which is a paraneoplastic syndrome whose acronym stands for less than half of the defining features of the disease, that is, polyradiculoneuropathy, organomegaly, potentially including coexisting Castleman disease, endocrinopathy, monoclonal plasma cell neoplasm, and skin changes.
Acknowledgments
None
Conflict of interest
The author declares that there is no conflict of interest.
References
- S Vincent Rajkumar, Robert A Kyle, Rebecca F Connor. POEMS syndrome.
- Dispenzieri A. How I treat POEMS syndrome. Blood. 2012;119(24):5650–5658.
- https://en.wikipedia.org/wiki/POEMS_syndrome
- https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678
- Angela Dispenzieri. Diagnostic criteria for POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes). Am J Hematol. 2014;89:214–223.
- https://en.wikipedia.org/wiki/POEMS_syndrome
- Joanna L Chan, Matthew N Kubicki. POEMS Syndrome Treatment & Management. Medspace. 2018.
- http://www.philly.com/philly/columnists/elizabeth_wellington/lisa-thomas-laury-shares-story-of-illness-and-recovery-in-new-book-20171013.html
©2018 Saraswati. This is an open access article distributed under the terms of the, which permits unrestricted use, distribution, and build upon your work non-commercially.