R. S. Crow, workign in Bristol, was the first one to describe POEMS syndromein 1956 and was subsequently described by Fukase in 1969. The acronym POEMS was coined by Bardwick in 1980. The syndrome was also referred to as Crow–Fukase syndrome by Nakanishi in 1984 based on a Japanese study of 102 cases, and this name is more widely known in Japan than POEMS syndrome.
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What makes POEMS syndrome so unique? Keep on reading!
What Is POEMS Syndrome?
POEMS syndrome is a rare blood disorder that can damage your nerves and affect other body parts as well. The syndrome is characterised by a huge number of abnormal plasma cells multiplying out of control. These cells release a substance called a monoclonal protein (or M-protein) into your blood. Having too many abnormal plasma cells and too much M-protein can damage your nerves and organs.
POEMS is an acronym based on its most common symptoms:
P – Polyneuropathy
O – Organomegaly
E – Endocrinopathy
M – Monoclonal protein (M-protein)
S – Skin changes
What Are The Symptoms Of POEMS Syndrome?
The most common signs of POEMS syndrome are polyneuropathy, organomegaly, endocrinopathy, having too many M-proteins and skin changes. Each common sign has its own set of symptoms, such as:
P – Polyneuropathy
- Weakness.
- Tingling.
- Numbness.
- Prickling.
- Burning.
- A pins-and-needles sensation.
O – Organomegaly
Imaging procedures may show that you have an enlarged:
- Liver (hepatomegaly).
- Spleen (splenomegaly).
- Lymph nodes (lymphadenopathy).
E – Endocrinopathy
Low sex hormone levels are a common sign of POEMS endocrinopathy, which affects testosterone and estrogen. Additional symptoms include:
- Blood sugar regulation issues, like diabetes.
- Thyroid problems, such as hypothyroidism.
- Addison’s disease, involving adrenal insufficiency.
In individuals assigned male at birth (AMAB), fertility problems and gynecomastia (enlarged breasts) may occur. For those assigned female at birth (AFAB), expect amenorrhea (missed periods) and breast growth. Elevated prolactin levels may lead to nipple discharge, even if not pregnant.
M – Monoclonal protein (M-protein)
High level of M-protein in your body can cause tumor formation in your blood and your bones to become thickened or harden.
S – Skin changes
A variety of skin changes may take place, including:
- Darker skin.
- Thicker skin.
- White fingernails.
- Increased hair growth on your face and legs.
- Growth of blood vessels (particularly on your chest) that look like small cherries.
Other symptoms may include:
- Swelling in your arms and legs.
- Unexplained weight loss.
- Bone and joint pain.
- Vision problems.
- Trouble breathing.
- Fatigue.
- Fever.
Who Treats POEMS Syndrome?
A medical team consisiting of specialists such as, myeloma specialists, radiologists, cardiologists, endocrinologists, neurologists, dermatologists, etc. treats POEMS syndorme.
The disorder requires a holistic treatment approach as many parts and organs of the body gets affected with the disease. The syndrome also has many complications that can further complicate the situtaion, if even one aspect of the disease is ignored.
Which Body Systems Are Affected By POEMS Syndrome?
POEMS syndorme is a complicated disease that can affect many bodily systems, such as:
- Hematological System: Involves an increase in the levels of WBCs, monoclonal plasma cells and certain proteins.
- Nervous System: Includes peripheral neuropathy, weakness, numbness, and tingling in the extremities.
- Endocrine System: Hormonal imbalance can lead to low sex drive and disruption in functioning of the glands like thyroid.
- Skeletal System:
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What Is The Treatment For POEMS Syndrome?
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