What is POEMS Syndrome?
POEMS Syndrome is an extremely rare blood disorder which tends to significantly damage the nerves of the body and also affects various other body parts. POEMS stands for the signs and symptoms of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal proliferative Disorder, Skin changes which range from numbness and tingling along with weakness in the extremities to enlargement of the liver or spleen, to abnormal hormonal levels, and abnormal skin changes.
POEMS Syndrome often at times is misdiagnosed due to the similarity of symptoms with various other medical conditions. POEMS Syndrome is a condition which progresses at a fairly rapid pace and tends to become life threatening if not treated at the appropriate time. There is no cure for POEMS Syndrome but timely treatment can ease the symptoms to a significant degree. Some of the treatment options for treating POEMS Syndrome are radiation and chemotherapy or stem cell transplant.
What Causes POEMS Syndrome?
What exactly causes POEMS Syndrome is not well known; however, it is known to be a paraneoplastic disorder meaning that the symptoms of this condition appear as a manifestation of a plasma cell disorder but why this occurs is not known. POEMS Syndrome is also believed to be associated with excessive production of inflammatory cytokines which are known to promote inflammation. Another cause for POEMS Syndrome is the vascular endothelial growth factor or VEGF which is a vasodilator which tends to lead to leaky blood vessels and is often associated with POEMS Syndrome.
What are the Symptoms of POEMS Syndrome?
As POEMS Syndrome affects multiple systems in the body, hence the symptoms of this disorder is also extremely varied. The symptoms for POEMS Syndrome are:
Polyneuropathy: The symptoms of Polyneuropathy include weakness, pain, numbness or tingling generally starting in the lower extremities and then gradually progressing as the disease advances to the upper extremities.
Organomegaly: In general the liver and spleen are the organs enlarged in an individual with POEMS Syndrome and hence the symptoms are related to that.
Endocrine Abnormalities: Individuals with POEMS syndrome may show numerous abnormalities which affect the endocrine system like improper functioning of ovaries in females and testes in males along with development of diabetes, hypothyroidism, glucose intolerance, gynecomastia in males.
Monoclonal Gammopathy: Individuals with POEMS Syndrome will also have monoclonal gammopathy which is characterized by uncontrolled growth of plasma cell. This excessive production of plasma cells lead to development of tumors.
Skin Abnormalities: Individuals with POEMS Syndrome will also have significant skin abnormalities with hyperpigmentation of the skin, excessive hair growth on the skin, hard and thick skin, and hyperhidrosis.
Apart from these symptoms, individuals with POEMS Syndrome also tend to have papilledema with gradual loss of vision.
Some of the other symptoms of POEMS Syndrome are:
- Pleural effusion
- Frequent fever
- Clubbing of the fingers
- Renal dysfunction
- Pulmonary hypertension in some cases.
How is POEMS Syndrome Diagnosed?
Diagnosing POEMS syndrome is quite complex as the symptoms resemble so many other medical conditions but to diagnose POEMS Syndrome a detailed history and physical examination is done and a note is made of the symptoms experienced by the patient. A family history of the patient is also taken to inquire as to whether there are other family members who have had this problem but a definitive diagnosis of POEMS Syndrome is made usually by identifying certain immunologic abnormalities on laboratory testing.
These tests are done on the blood or the CSF or cerebrospinal fluid which will show elevated levels of M-proteins which is a clinching evidence of POEMS Syndrome. Additionally, analysis of blood plasma will show elevated levels of vascular endothelial growth factor which will virtually confirm the diagnosis of POEMS Syndrome.
How is POEMS Syndrome Treated?
There is no cure for POEMS Syndrome but treatment is basically two fold with the first treatment totally aimed at treating the plasma cell disorder and the second is to treat the specific symptoms that the patient experiences. The treatment for POEMS Syndrome requires a team of physician from various specialties working in a coordinated manner to try to give best possible treatment to the patient.
Radiation and chemotherapy or even surgical excision of localized osteosclerotic lesions and which have still not spread is quite effective in ameliorating the symptoms of POEMS Syndrome. In some cases chemotherapy, anti-cancer agents, and steroids have also some effectiveness towards treatment of POEMS Syndrome. Stem cell transplant is yet another way of attempting to treat symptoms of POEMS Syndrome. Otherwise, the treatment is purely supportive and symptomatic for POEMS Syndrome.
What is the Prognosis or Survival Rate of POEMS Syndrome?
POEMS Syndrome as of now cannot be cured completely and the symptoms tend to come and go. The condition advances at a fairly rapid pace to assume a life threatening condition if not treated at an appropriate time. POEMS syndrome with time becomes a chronic condition and in majority of cases renders an individual completely disabled due to the symptoms. On average the survival rate has been estimated to be around 14 years for an individual with POEMS Syndrome, although with timely treatment and a good response to the treatment received an individual can survive for a longer period of time with POEMS Syndrome.
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