INTRODUCTION:

POEMS syndrome is linked to monoclonal gammopathies, also known as plasma cell dyscrasias. The aberrant buildup of M-proteins (also known as immunoglobulins) in the blood occurs from the uncontrolled proliferation of a single clone (monoclonal) of plasma cells. Immunoglobulin is a protein found in the body that helps to fight infection. The exact role of M-proteins in POEMS syndrome, as well as the exact cause, are unknown. A molecule called VEGF (vascular endothelial growth factor) may play a role in this condition, according to research. Because it was first described by Crow in 1956 and then by Fukase in 1968, the syndrome was dubbed Crow Fukase Syndrome. Osteosclerotic myeloma, Crow–Fukase syndrome, Takatsuki disease, and PEP syndrome are all terms for the same condition².

What is POEMS Syndrome?

POEMS syndrome is a multisystem condition that affects a small number of people. Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin Changes are all acronyms for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes.

POEMS syndrome is a multisystem illness that is highly rare. POEMS stands for polyneuropathy (disease of many nerves), organomegaly (abnormal enlargement of an organ), endocrinology (disease of certain hormone-producing glands that help regulate sexual function and metabolic function), Monoclonal gammopathy or M protein (skin abnormalities), and skin abnormalities.

POEMS syndrome is a paraneoplastic condition characterised by plasma cell dyscrasia linked to cytokine or growth factor alterations³.

Etiology:

· The cause of POEMS syndrome is Unknown

· Genetics

· Environmental and developmental factors

· Race-no specific racial association has been identified, although a preponderance of cases have been reported in the Japanese literature.

· Sex- Slightly more prevalent among men than women, with a male-to-female ratio of 2.5:1.

· Age-The onset of POEMS syndrome occurs most frequently in the fifth or sixth decade of life, with a mean patient age at onset of 48 years for men and 59 years for women⁴.

Etiology:

· POEMS syndrome has an unknown aetiology.

· Genetics, environmental, and developmental factors all have a role.

· Race: Although a large number of instances have been documented in the Japanese literature, no definite racial link has been found.

· Sex- Men are slightly more likely than women to have it, with a male-to-female ratio of 2.5:1.

· Age—POEMS syndrome usually strikes in the fifth or sixth decade of life, with a mean patient age of 48 years for males and 59 years for women at the time of onset⁵.

Pathophysiology:

POEMS syndrome's pathogenesis is not well understood. The development of the condition is caused by a plasma cell dysfunction; however, the mechanism by which this occurs is unknown. Interleukin (IL)-1beta, IL-6, and tumour necrosis factor (TNF)–alpha levels have all been found to be elevated. Significant increases in vascular endothelial growth factor (VEGF) levels have lately been observed. Increased VEGF levels are thought to increase vascular permeability, resulting in edoema, increased endo neural pressure, and the deposition of plasma cell–derived material. Demyelization can occur when myelin is exposed to serum cytokines and complement.

Clinical Manifestation:

1. Polyneuropathy - Numbness, tingling, and weakness in your legs — and, over time, possibly in your hands — as well as difficulty breathing. This is a crucial element in determining whether or not someone has POEMS syndrome.

2. Organomegaly - is characterised by an enlarged spleen, liver, or lymph nodes.

3. Endocrinopathy/edema - An underactive thyroid (hypothyroidism), diabetes, sexual issues, weariness, swelling in your limbs, and problems with metabolism and other important activities can all be symptoms of endocrinopathy/edema.

4. Monoclonal-protein - producing abnormal bone marrow cells (plasma cells) that create a protein (monoclonal protein) that is present in the bloodstream. This is a crucial element in determining whether or not someone has POEMS syndrome. Unusual bone stiffening or thickening is frequently linked to monoclonal protein.

Other symptoms and signs -

1. Extravascular fluid overload with ascites, peripheral oedema, and pleural effusion are some of the other symptoms and indicators (accumulation of fluid in the abdominal cavity, limbs and lung lining respectively)

2. Thromboses of the arterial and venous systems (blood clots)

3. Restrictive lung disease,

4. pulmonary hypertension, and

5. respiratory muscle weakness are all examples of pulmonary disease⁶.

Diagnostic Evaluation: To diagnose POEMS, need:

· Both mandatory major criteria,

· At least 1 of the other major criteria,

· AND at least 1 of the minor criteria.

Mandatory criteria

Major criteria

Minor criteria

· Peripheral neuropathy

· Monoclonal plasma cell disorder

· Osteosclerotic bone lesions

· Castleman disease (giant cell or Angio follicular lymph node hyperplasia)

· Increased vascular endothelial growth factor (VEGF)

· Organomegaly

· Endocrinopathy (excluding diabetes or hypothyroidism)

· Extravascular volume overload (most commonly peripheral oedema; also pleural effusion, ascites)

· Skin changes

· Papilloedema

· Thrombocytosis / polycythaemia – arterial and venous thrombosis, strokes

Other diagnostic test:

· Blood tests are performed.

Ø A complete blood count, which may reveal thrombocytosis and/or polycythaemia.

Ø Abnormalities in thyrotropin, hyperglycemia, and oestrogen may be detected on endocrine panels.

Ø Electrophoresis of serum or urine to look for monoclonal immunoglobulin

· X-ray of bones in almost all patients show osteosclerotic lesions, which are often multiple.

· Biopsy of an enlarged lymph node may show Castleman disease.

· Lumbar puncture in those with neuropathy may show increased protein in the cerebrospinal fluid.

· Nerve conduction studies may show changes of demyelination and axonal degeneration.

· Bone marrow examination may show involvement with plasma cells.

· Lymph node biopsy of enlarged nodes.

· Skin biopsy results are usually nonspecific-

· Nonspecific hyperpigmentation of the basal layer with inflammatory infiltration or dermal fibrosis is seen in Scleroderma-like lesions. It differs from scleroderma because sweat glands and collagen are normal.

· Inflammatory infiltration of lymphocytes and plasma cells is seen in hyperpigmented lesions.

· Strawberry naevus, lobular capillary angioma, and glomeruloid haemangioma are examples of angiomas (this is especially characteristic of POEMS syndrome and shows enlarged vascular spaces filled with coiled capillaries surrounded by pericytes, which look like kidney glomerulus)⁸.

MANAGEMENT:

POEMS syndrome is treated in the same way that the underlying plasma cell dysfunction is treated. Medical, surgical, and adjuvant therapy are used to treat the majority of patients. Combinations of corticosteroids, low-dose alkylators, and peripheral blood stem cell transplantation after high-dose chemotherapy are currently the cornerstones of treatment for patients with diffuse illness.

Prognosis:

POEMS Syndrome is a long-term illness. Patients with multiple myeloma have a median survival of about a decade (8-14 years), which is three times longer. However, due to neuropathy, many patients are confined to their beds (50 percent)⁹.

CONCLUSION:

POEMS Syndrome is an uncommon clinical condition that manifests polyneuropathy, organomegaly, endocrinopathymonogammppathy, and skin abnormalities as a result of plasma dyscariasis. The cause is unknown, however this disorder has a strong family history. Because each patient is different, the person or family should work with a doctor to develop a treatment plan that is right for them.

REFERENCES:

1. S Vincent Rajkumar. POEMS syndrome. UpToDate. Waltham, MA: UpToDate; May 15, 2015;

2. Veerabhadrappa G Mendagudli, Tushar S Kedar. POEMS Syndrome- An Overview. International Journal of Nursing Education and Research. 2021; 9(4):505-8. doi: 10.52711/2454-2660.2021.00119

3. YP Mundal, SK Sharma et al. API Textbook of medicine, 9th edition, published by Jaypee Brothers, Volume-1 page no.982.

4. Jameson Fausi, Kapser, Harrison`s Textbook of principles of internal medicine, 20th edition volume -2 , page no. 346,498,802-803, 2167,2759-2760,3231.

5. Dr Tina Tian, Medical Oncology Registrar, Hawke's Bay Hospital, Hastings, New Zealand, 2013. https://dermnetnz.org/topics/poems-syndrome/

6. POEMS syndrome. DermNet NZ. September 13, 2014; http://dermnetnz.org/systemic/poems.html.

7. https://rarediseases.org/rare-diseases/poems-syndrome/

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

9. Angela Dispenzieri Hematology Am Soc Hematol Educ Program (2005) 2005 (1): 360–367.https://doi.org/10.1182/asheducation-2005.1.360 https://ashpublications.org/hematology/article/2005/1/360/19304/POEMS-Syndrome.

Received on 10.02.2022 Modified on 07.03.2022

Int. J. of Advances in Nur. Management. 2022; 10(2):148-150.

DOI: 10.52711/2454-2652.2022.00038