POEMS Syndrome

POEMS syndrome is a rare blood disorder that occurs when an overgrowth of plasma cells (a specific type of cell in the bone marrow) results in production of compounds that damage other parts of the body. POEMS syndrome most often occurs in those over 50 years old and slightly more often in men than in women. Although patients sometimes confuse POEMS syndrome with multiple myeloma, the two diseases differ greatly in their symptoms and potential complications, and the average predicted survival is better for patients with POEMS than for those with multiple myeloma. POEMS syndrome is a chronic, progressive disease, but the symptoms can improve with treatment. The reported 5-year survival rate for POEMS syndrome is 60%.

Clinical Features and Symptoms

The name POEMS is an acronym for the five main features of the disease (detailed below): polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. A diagnosis of POEMS syndrome requires both polyneuorpathy and monoclonal gammopathy, in addition to either sclerotic bone lesions or Castleman disease. Usually, at least one of the other main features is also present. Symptoms vary based on the organ systems involved.

Polyneuropathy: nerve disease (numbness, tingling, and weakness of the hands and feet). The polyneuropathy associated with POEMS syndrome involves both motor and sensory nerves. It begins at the extremities and progressively spreads inward toward the trunk.

Organomegaly: organ enlargement (most often liver, lymph nodes, or spleen). Enlargement of the liver is not associated with any defined cellular or physiological changes, but enlargement of the lymph nodes and spleen usually is consistent with changes resulting from Castleman disease. Approximately 15% of patients with POEMS syndrome also have Castleman disease.

Endocrinopathy: disease affecting hormone-producing glands. Most patients with POEMS syndrome have more than one endocrinopathy. Diabetes mellitus and glucose intolerance are present in many patients with POEMS syndrome, as is improper functioning of the ovaries or testes. Hypothyroidism, hyperprolactinemia, and hypoparathyroidism are also among the many endocrinopathies associated with POEMS syndrome.

Monoclonal gammopathy or monoclonal plasma proliferative disorder: a condition with abnormal blood cells. All cases of POEMS syndrome are associated with a plasma cell disorder. Many such disorders have been reported in patients with POEMS syndrome, but the most common are osteosclerotic myeloma or monoclonal gammopathy of unknown significance. Classic multiple myeloma has not been associated with POEMS syndrome. The M proteins (hallmarks of plasma cell disorders) most frequently found with POEMS syndrome are the immunoglobulin A (IgA)–gamma and immunoglobulin G (IgG)–gamma light chains.

Skin changes: increased skin pigment, increased body hair, thickening of the skin, etc. Many dermatologic changes have been associated with POEMS syndrome. The most common changes include abnormal darkening of the skin, thickening and hardening of the skin, and excessive hair growth. Other changes, including whitening of the nails, excessive sweating, clubbing of the fingers, Raynaud phenomenon, and angiomas, are also observed.

Other common signs and symptoms of POEMS syndrome:

  • Fatigue and weakness
  • Generalized aches and pains
  • Edema (swelling of the ankles and legs)
  • Ascites (fluid collection in the abdomen)
  • Pleural effusion (fluid collection in the lungs)
  • Enlarged breast tissue (occurs in both men and women)
  • Increased blood cell counts (platelets or red cells)
  • Thromobosis (formation of blood clot within a vessel)
  • Changes in vision
  • Renal insufficiency

Treatment

The treatment of POEMS syndrome depends on treating the underlying plasma cell disorder. Most patients are treated with a combination of medical, surgical, and adjuvant therapies. When the plasma cell disorder responds to treatment, all other symptoms of POEMS syndrome usually improve or resolve completely. In general, the prognosis is worse for those with a plasma cell disorder that involves the bone marrow.

Prognosis

If untreated, POEMS syndrome grows progressively worse, and the associated symptoms depend on the systems involved (ranging from altered skin pigmentation to debilitating weakness and loss of function). The median survival period for patients with POEMS syndrome is 8 years, and the 5-year survival rate is 60%. Overall shorter survival has been associated with effusions, edema, and ascites and with clubbing of the fingers. The most common causes of death are cardiorespiratory failure, kidney failure, infection, and progressive exhaustion resulting from lack of nourishment. Approximately 50% of the patients with POEMS syndrome become bedridden as a result of the polyneuropathy, and the resultant inactivity can cause bedsores and blood clots, which can lead to death, as can organomegaly and endocrinopathy.

Learn More about POEMS Syndrome

More information about POEMS syndrome is available from the National Organization of Rare Diseases and from Medscape.