POEMS Syndrome

POEMS syndrome is a rare disorder. POEMS stand for:
Polyneuropathy – numbness, weakness of lower limbs, and tingling sensation
Organomegaly – enlarged liver, spleen, or lymph nodes
Endocrinopathy – abnormal levels of hormone leading to diabetes, fatigue, hypothyroidism, and metabolism problems
Monoclonal protein – abnormal bone marrow cells that produce a monoclonal protein leading to monoclonal plasma-proliferative disorder
Skin abnormalities – increased thickness of skin; increased hair growth on legs and face

This syndrome is seen mostly in men and elderly people. It is also known as shimpo syndrome, Crow-Fukase syndrome, or Takatsuki syndrome.

Signs and symptoms

Common signs and symptoms of POEMS syndrome include:

  • Weakness of nerves in the upper and lower limbs
  • Darkening of the skin (hyperpigmentation)
  • Thickening of the skin
  • Abnormal fluid retention
  • Increased hair growth (hypertrichosis)
  • Elevated platelet count
  • Shortness of breath
  • Fluid accumulation around the lungs (i.e. pleural effusion)

Causes

POEMS syndrome occurs due to uncontrolled growth of monoclonal cells (plasma cells) leading to the accumulation of M-proteins (which fight infections) in the blood. A protein called vascular endothelial growth factor (VEGF) also causes angiogenesis leading to POEMS syndrome.

Diagnosis

To diagnose the POEMS syndrome, your doctor may prescribe few blood tests which include measuring of vitamin B12 levels, VEGF levels, and hemoglobin levels.

POEMS syndrome diagnosis may be confirmed if your blood tests show:

  • Low vitamin B12 levels
  • Elevated VEGF levels
  • Decreased hemoglobin levels

A lumbar puncture is performed to determine protein levels in cerebrospinal fluid (CSF).

Your doctor may also recommend electromyography (EMG) for neuropathy, computed tomography (CT) scan and bone marrow biopsy to diagnose the condition.

Treatment

The treatment for POEMS helps to relieve the symptoms but may not cure the condition. The treatment depends on underlying plasma cell disorder. The treatment usually involves a combination of medical, surgical, and adjuvant therapies. Treatment options may also include radiation therapy, chemotherapy, and peripheral blood stem cell transplant.

Corticosteroids and low-dose alkylating agents are given followed by chemotherapy. Systemic therapy can be given in the case of widespread osterosclerotic lesions.

If VEGF is the underlying cause, a monoclonal antibody such as bevacizumab may be prescribed by your doctor.

POEMS syndrome is a rare disease and research is still ongoing to identify the appropriate treatment for it.

Prevention

Early diagnosis of POEMS syndrome is very important as it progresses rapidly and may be life-threatening at times. This condition is many times misdiagnosed as it mimics the symptoms of other conditions as well.