POEMS Syndrome

What is POEMS Syndrome?

This syndrome is a rare disorder of the blood affecting many of the body systems and causing damage to the nerve. POEMS can occur due to the escalation of certain plasma cells in the bone marrow which create an atypical quantity of blood proteins that damage body organs.

POEMS is an acronym for this syndrome’s more frequent symptoms and signs.

  • Polyneuropathy : Damage to the nerve causing tingling, numbness as well as weakness of the feet and hands.
  • Organomegaly : Enlargement of organs such as lymph node, liver or spleen
  • Endocrinopathy : Hormone levels that are abnormal
  • Monoclonal protein : An assortment of blood proteins (Immunoglobulins) that are abnormal and made from cells of bone marrow known as plasma cells; also called “monoclonal immunoglobulin”
  • Skin alterations : Skin pigment increases, body hair increases, thickening of the whitening of the skin and nails.

Other features might also be present in POEMS syndrome, such as blood clots and high platelet counts.

Symptoms

Symptoms and signs of this syndrome include:

  • Nerve numbness, tingling and weakness especially in the hands and feet
  • Enlarged liver
  • Enlarge spleen
  • Castleman disease – lymph node disorder
  • Changes in hormone production
  • Diabetes
  • Impotence
  • Amenorrhea
  • Hypothyroidism
  • Development of abnormal blood protein
  • Development of breast in males
  • Increase in skin pigment
  • Skin thickening and tightening
  • Increase in body hair on the face, chest and limbs
  • Whitening of nails
  • Swelling or edema of the feet and legs
  • Fatigue easily
  • Generalized pains and aches
  • Changes in vision
  • Enlarged breast area tissue in both women and men
  • Failure of testes and ovaries to function
  • Fluid retention in the abdomen
  • Fluid retention in ankles and legs

Causes

The cause of this syndrome is not known, however it is normally connected with disorders of plasma cells for instance myeloma. It is not inherited or contagious. To receive a diagnosis of POEMS, an individual need not have all 5 symptoms. This syndrome is most common in individuals in their 50s with male slightly more at risk then women. It is most frequently found in Japan, where it is referred to as “Crow-Fukase syndrome”.

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Treatment

In order to diagnosis this syndrome some of the following tests will need to be done:

  • Neurological exam
  • Electromyography
  • Nerve conduction velocity test
  • Blood tests – such as immunoglobulin electrophoresis, hormone studies
  • Urine tests
  • Bone x-rays
  • Biopsy of the bone marrow

The aim of any treatment is the alleviation of the symptoms that are worse which is normally from nerve damage that causes progressive weakening in the extremities. Treatment also tries to halt the production of cells of bone marrow that cause problems in other areas of the body. Forms of treatment include:

  • Radiation therapy
  • Physical therapy
  • Chemotherapy
  • Corticosteroids
  • Hormone replacement – when needed

With neuropathy in the peripheral areas caused by this syndrome do the following:

  • Stop any drugs that cause peripheral neuropathy
  • Start non-drug therapy for the reduction of pain, such as:
    • Avoiding expanded episodes of walking or standing
    • Wearing shoes that are looser
    • Pain drugs
    • Take safety procedures to balance loss of sensation
    • Ask your physician about therapeutic shoes (which might be covered by Medicare as well as other insurance)

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