Without treatment, the rare blood condition known as POEMS syndrome can become very serious. Having an excessive number of aberrant plasma cells might harm several organs and bodily systems. The acronym “POEMS” refers to a number of frequent symptoms, such as polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin abnormalities. The majority of neoplasms have mass effects that result from their cells invading and destroying tissues, which generate their signs and symptoms. POEMS syndrome cannot be prevented, although early diagnosis and treatment can lead to better results.

 

Introduction:

 

An uncommon blood condition called POEMS syndrome can have an effect on various physiological systems. Your body produces aberrant plasma cells that multiply out of control when you have POEMS. These cells discharge a chemical into your blood known as a monoclonal protein (or M-protein). Your nerves and organs can be harmed if you have too many aberrant plasma cells and too much M-protein. The most typical symptoms and indicators of POEMS syndrome are:

• Polyneuropathy: Damage to your body’s nerves occurs during polyneuropathy. Your legs, arms, feet, or hands may experience pain or other uncomfortable sensations as a result of POEMS.
• Organomegaly: Large organs are referred to as organomegaly. Your lymph nodes, spleen, and liver may grow as a result of POEMS.
• Endocrinopathy: A hormone issue is endocrinopathy. Your endocrine glands might emit an excessive amount of hormones if you have POEMS.
• Monoclonal protein: When you have POEMS, monoclonal protein (M-protein) accumulates in your blood. When a single plasma cell divides excessively, or clones, itself, M-proteins build up. You gradually develop an excess of M-proteins because each plasma cell discharges them into your blood.
• Skin: Darkening, thickness, and other changes in the skin can result from POEMS.

Primary and secondary POEMS syndromes are two different subtypes. While secondary POEMS syndrome is linked to other underlying illnesses like Castleman disease, lymphoma, or other malignancies, primary POEMS syndrome is a rare disorder that develops spontaneously. Monoclonal gammopathy, an aberrant protein created by just one clone of plasma cells, is a feature of primary POEMS syndrome. The skin, organs, and nerves may all be harmed by this protein. The excessive production of cytokines, which are immune system-regulating proteins, results in secondary POEMS syndrome. These cytokines have the potential to inflame tissues and harm skin, organs, and nerves.

 

Causes:

 

It is unclear what specifically causes POEMS syndrome. But it’s thought to be brought on by an unusual immunological response. The aberrant protein that the plasma cells create in primary POEMS syndrome can cause an immunological reaction that harms the skin, organs, and nerves. The overproduction of cytokines in secondary POEMS syndrome can also set off an immunological response that harms the skin, nerves, and organs.

Studies have shown that people with POEMS syndrome have abnormally high amounts of vascular endothelial growth factor (VEGF) and pro-inflammatory cytokines such as interleukin-6, interleukin-1, and TNF-alpha in their blood. The immune system of the body produces cytokines, which are proteins, in reaction to inflammation. To precisely understand how cytokines and VEGF contribute to development, more study is required.

 

Symptoms:

 

An uncommon multisystem paraneoplastic condition is called POEMS syndrome. Some of the main symptoms and indicators of the disease are abbreviated as POEMS. Polyneuropathy, monoclonal gammopathy, osteosclerotic lesion, Castleman disease, or increased levels of vascular endothelial growth factor (VEGF) are the major criteria for POEMS syndrome, together with at least one of the minor criteria given below.

 

Polyneuropathy

An illness called polyneuropathy affects the nerves all over the body. Polyneuropathy symptoms include:

• weakness, discomfort, numbness, or tingling in the hands, feet, legs, and/or toes
• Paresthesia, or burning, tickling, numbness, and prickling of the skin
• reduced sensitivity of the muscles to stimuli (areflexia)

Organomegaly

The unusual expansion of an organ or system in the body is known as organomegaly. Individuals with POEMS syndrome may have the following organomegaly symptoms:

• Hepatomegaly, or abnormally big liver
• Lymphadenopathy, or abnormally swollen lymph nodes
• Angiofollicular lymph node hyperplasia or Castleman disease-related lymph node swelling

Endocrinopathy

Endocrinopathy is a condition in which the endocrine system, which consists of glands that discharge hormones into the blood, produces aberrant hormone levels. In people with POEMS syndrome, endocrinopathy symptoms include:

• Low sex hormone production (hypogonadism)
• Blood sugar regulation issues (diabetes or decreased glucose tolerance)
• Hypothyroidism is an underactive thyroid.
• Adrenal glands not functioning normally (primary adrenal insufficiency)
• Men may also go through:
  • Gynecomastia, or breast enlargement
  • Erectile dysfunction (impotence)
• Women may also go through:
  • Menstruation not occurring (amenorrhea)
  • Increased milk production and prolactin circulation in the breasts Abnormal milk discharge from the nipples (galactorrhea)

M-Protein

• POEMS is indicated by elevated levels of M-protein. Blood tumors might develop if you have an excessive amount of M-protein. Overproduction of M-protein can lead to osteosclerosis, a thickening or hardening of the bones.

Skin alterations

Skin modifications could include any of the following:

• lighter skin.
• larger skin.
• fingernails are white.
• increased facial and leg hair growth.
• Blood vessel growth resembles little cherries, especially on the breast.

 

Diagnosis:

 

In addition to tests that match the aforementioned symptoms, other procedures that can give abnormal results and support the diagnosis of POEMS syndrome include EMG for neuropathy, a CT scan, bone marrow examination to look for clonal plasma cells, and plasma or serum electrophoresis of protein to look for myeloma proteins. These included increased VEGF, thrombocyte, and/or erythrocyte parameter levels in the blood.

Patients with Castleman disease who also show many of the symptoms and signs of POEMS syndrome but who are not also found to have clonal plasma cells or peripheral neuropathy should not be labeled as having POEMS syndrome. They are more appropriately diagnosed as having POEMS syndrome with the Castleman disease variant. When compared to individuals with POEMS syndrome, these patients may have lower overall survival rates and elevated blood levels of the cytokine interleukin-6.

 

Treatment for POEMS Syndrome:

 

Although POEMS syndrome is not curable, treatment may help your symptoms. Over the past ten or so years, the prognosis for persons with POEMS syndrome has significantly improved.

• Phosphorus: The drug is quite successful in treating both the disease’s symptoms and its side effects. Cold food cravings are the defining symptom.
• Agaricus muscarius: Patients who benefit from this medicine experience a sense of being pierced by unnecessary things. The hurting area feels chilly and numb.
• People who have excruciating pain along with numbness and nocturnal sweats are prescribed chamomilla. The symptoms include restlessness, a stiff neck, paralysis-like sensations in the feet during the night, etc.
• Capsicum annum: – Hip-to-foot pain, insomnia, pain in distant body areas after coughing, and knee pain.
• Calcarea Phosphorica: This treatment is effective in treating numbness. It is administered to kids who are grumpy, chubby, and have a tendency to have cold arms and legs.

Although there is no known treatment for POEMS syndrome, some approaches can assist manage its symptoms, which include:

• Radiation therapy: Radiation kills aberrant plasma cells by directing strong radiation doses at them.
• Chemotherapy: Cancer-fighting medications such as chemotherapy kill or inhibit the formation of abnormal plasma cells. Similar to cancer therapies for multiple myeloma, chemotherapy is used to treat POEMS. These therapies are quite successful and frequently have minor adverse effects.
• Autologous stem cell transplant: In this treatment, healthy stem cells are used to replace your defective plasma cells. Stem cells transform into fully grown cells, such as plasma cells. Your doctor takes healthy stem cells from your body during an autologous stem cell transplant. The aberrant plasma cells are then eliminated using radiation or chemotherapy. Your healthcare professional will reintroduce the stem cells into your body after treatment so they can develop into healthy plasma cells.
• Physical therapy: You can benefit from physical therapy if you’re having movement problems as a result of polyneuropathy.

 

Precautions for POEMS Syndrome:

 

POEMS cannot be stopped. It’s crucial to receive POEMS treatment. POEMS syndrome can be fatal if left untreated. However, a treatment that successfully eradicates aberrant plasma cells can lengthen your life. Additionally, treatment can reduce symptoms and enhance your quality of life. If you have any POEMS syndrome symptoms, particularly weakness in your arms and legs, get in touch with your doctor very away.