A Review-Poems Syndrome

 

Mrs Purohit Saraswati1*, Mrs. Rashmi P2, Mrs. Sunith PS3, Mrs.Vidya M4

1MSc (Psychiatric Nursing), Asst. Lecturer, Dept Mental Health Nursing,

J.S.S College of Nursing, Ramanuja Road. Mysuru-570004

2Asst Lecturer, J.S.S College of Nursing, Ramanuja Road. Mysuru-570004

3Asst Lecturer, J.S.S College of Nursing, Ramanuja Road. Mysuru-570004

*Corresponding Author E-mail: saruswati28@gmail.com

 

 

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 a rare paraneoplastic syndrome that is caused by an underlying plasma cell disorder. The diagnosis of POEMS syndrome: The two mandatory criteria PLUS ≥ 1 major AND ≥ 1 minor criterion. The treatment of POEMS syndrome depends on the treatment of the underlying plasma cell disorder. Most patients are treated with a combination of 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 one or more of the following features: osteosclerotic myeloma, Castleman's disease (angiofollicular lymph node hyperplasia), increased levels 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 and weakness in your legs — and over time, maybe in your hands — and difficulty breathing.

 

Organomegaly: Enlarged spleen, liver or lymph nodes.

 

Endocrinopathy: Abnormal hormone levels that can result in underactive thyroid (hypothyroidism), diabetes, sexual problems, fatigue, swelling in your limbs, and problems with metabolism and other essential functions.

 

Monoclonal plasmaproliferative disorder: Abnormal bone marrow cells (plasma cells) that produce a protein (monoclonal protein) that can be found in the bloodstream.

 

Skin changes: More color than normal on your skin, possibly thicker skin and increased facial or leg hair.

 

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-proliferative disorder (almost always λ)

 

Major criteria:

·      Castleman disease1

·      Sclerotic bone lesions

·      Vascular endothelial growth factor (VEGF) elevation

 

Minor criteria:

·      Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy)

·      Extravascular volume overload (edema, pleural effusion, or ascites)

·      Endocrinopathy (adrenal, pituitary, gonadal, parathyroid, thyroid2 and pancreatic2

·      Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata,

·      plethora, acrocyanosis, flushing, and white nails) Papilledema

·      Thrombocytosis/polycythemia3

 

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, other tests can give abnormal results supporting the diagnosis of POEMS syndrome. These included raised blood levels of VEGF, thrombocytes, and/or erythrocyte parameters. (6)

 

Treatment:

The treatment of POEMS syndrome depends on the treatment of the underlying plasma cell disorder. Most patients are treated with a combination of medical, surgical, and adjuvant therapies.

 

The current mainstays of treatment for patients with diffuse disease include combinations of corticosteroids, low-dose alkylators, and peripheral blood stem cell transplantation following high-dose chemotherapy. Some caution should be used in selecting the chemotherapeutic regimen to avoid worsening of the  Widespread osteosclerotic lesions may benefit from systemic therapy.

 

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:

1. 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 opioids 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.

 

REFERENCE:

1.       https://www.uptodate.com/contents/poems-syndrome

2.       https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3425020/

3.       https://en.wikipedia.org/wiki/POEMS_syndrome

4.       https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

5.       https://www.orpha.net/data/patho/Pro/en/Diagnostic_criteria_POEMS_EN.pdf

6.       https://en.wikipedia.org/wiki/POEMS_syndrome

7.       https://emedicine.medscape.com/article/1097031-treatment

8.       http://www.philly.com/philly/columnists/elizabeth_wellington/lisa-thomas-laury-shares- story-of-illness-and-recovery-in-new-book-20171013.html

 

 

 

 

 

 

Received on 27.01.2018        Modified on 18.03.2018

Accepted on 22.04.2018 ©A&V Publications All right reserved

Int. J. of Advances in Nur. Management. 2018; 6(3): 253-254.

DOI: 10.5958/2454-2652.2018.00056.2