POEMS Syndrome
What is POEMS Syndrome? POEMS syndrome is a very rare blood disease. It damages the nerves, following an abnormal immune system response to a neoplasm and affecting other body parts. Picture courtesy: medicine It is a paraneoplastic syndrome. It is due to an underlying plasma cell neoplasm. POEMS is a derived acronym and stands for […] Read More
Top Doctors For POEMS Syndrome Treatments
Top Hospitals For POEMS Syndrome Treatments
POEMS Syndrome
What is POEMS Syndrome?
POEMS syndrome is a very rare blood disease. It damages the nerves, following an abnormal immune system response to a neoplasm and affecting other body parts.
Picture courtesy: medicine
It is a paraneoplastic syndrome. It is due to an underlying plasma cell neoplasm. POEMS is a derived acronym and stands for this blood disorder’s major signs and symptoms.
Signs and symptoms are:
- Polyneuropathy
- Organomegaly
- Endocrinopathy or Edema
- Monoclonal Gammopathy
- Skin Changes.
- Osteosclerotic myeloma
- Crow-Fukase syndrome
- Takatsuki syndrome [1].
Picture Courtesy: Malacards
For POEMS syndrome, risk stratification is restricted to clinical phenotypes rather than specific molecular determinants because the syndrome’s pathogenesis is not well understood.
Risk factors may include:
- Pulmonary hypertension
- Low serum albumin
- Pleural effusion
- Reduced eGFR (estimated glomerular filtration rate)
- Age (above 40 years)
- Melphalan + Dexamethasone
- Lenalidomide + Dexamethasone
- Chemotherapy
- Stem cell transplantation
Picture Courtesy: healthjade
Symptoms
The major signs and symptoms of POEMS syndrome were earlier mentioned, reflected in its acronym, “POEMS.” They are:
Polyneuropathy
- Nerves outside the brain and spinal cords become damaged.
- Numbness, tingling, and weakness in the legs and hands.
- Burning and prickling of the skin (paresthesia)
- Breathing difficulty.
Organomegaly
- Enlargement of liver, spleen, or lymph nodes.
- Swollen lymph nodes (as seen in angiofollicular lymph node hyperplasia or Castleman disease)
Endocrinopathy /Edema - Abnormal hormone levels
- Diabetes
- Fatigue
- Metabolic problems
- Hypothyroidism
- Swollen limbs
- Sexual dysfunction
Monoclonal Gammopathy
- Plasma cells dyscrasias; produce a monoclonal protein that can be found in the bloodstream.
- Hardening and thickening of bones.
Skin Changes
- Hyperpigmentation (dark-colored skin).
- Skin becomes thicker (scleroderma).
- Red-spotted skin.
- Excessive sweating
- Clubbed fingernails
- More facial and leg hair.
Other symptoms
- Visual disturbance, headache, and nausea
- Pericardial effusion
- Fluid in arms and legs
- Weight loss
- Fatigue
Causes
The real cause of POEMS syndrome remains unknown. But it is observed that people with POEMS syndrome show an increased number of plasma cells that produce an abnormal amount of monoclonal protein, which can damage other parts of the body. Studies have demonstrated abnormal high levels of VEGF (vascular endothelial growth factor) and pro-inflammatory cytokines, interleukin-6 and 1, TNF-α in the blood of individuals with POEMS disease [3].
One should not misconstrue the signs and symptoms of POEMS syndrome for other disorders such as osteoporosis and arthritis. Early diagnosis is important because POEMS syndrome advancement can be very life-threatening.
FAQ
What is a plasma cell disorder?
Plasma cell disorder is a blood cancer situation in which plasma cells develop tumors and can cause damage to the bones, heart, kidneys, bone marrow, and immune system.
What causes POEMS syndrome?
The real cause of POEMS syndrome remains unknown. But studies have shown abnormal vascular endothelial growth factor (VEGF) levels in the blood of POEMS syndrome patients.
More research is needed to know the exact role of VEGF and cytokines in this disorder.
What are the diets that can improve the quality of life of people with POEMS syndrome?
There is nothing specific on the POEMS syndrome table, but eat well and be healthy. Therefore, some suggested healthy foods may include:
- A high healthy fat diet such as olives, avocado, walnuts, etc.
- Green leafy vegetables
- Fruits; berries, watermelon, grapes, apples, etc.
- Prebiotics; bananas, yogurt, tempeh, miso.
- Nuts
- Seafood, eggs, honey, fatty fish, lean meat
- Antioxidants rich foods
- Drink fluids, especially a lot of water.
It is important to seek your doctor’s advice on your diet.
Is there a difference between plasma cell myeloma and multiple myeloma?
Plasma cell myeloma is also known as multiple myeloma or myelomatosis. It is a type of cancer that starts in the white blood cells (WBCs).
Who is affected by POEMS syndrome?
Risk stratification for POEMS syndrome is restricted to clinical phenotype. The most common risk factors include people over the age of 40 years that fall under the following health disadvantage categories:
- Low serum albumin
- High blood pressure (Hypertension)
- Pleural effusion
- Reduced eGFR.
What is the prognosis of people with POEMS syndrome?
The prognosis of the syndrome varies from person to person. It depends on the part of the body affected and the degree to which it is affected. Early treatment can ease the symptoms to improve the quality of life [1].
What is the life expectancy of a POEMS syndrome patient?
POEMS syndrome is a chronic condition. The average survival time for a person suffering from POEMS syndrome is 8-14 years [4].
References
- https://my.clevelandclinic.org/health/diseases/17985-poems-syndrome
- https://emedicine.medscape.com/article/1097031-clinical
- https://rarediseases.org/rare-diseases/poems-syndrome/
- https://www.medicinenet.com/how_long_can_you_live_with_poems_syndrome/article.htm
