What is POEMS syndrome and is it dangerous?

This is an automatically translated article.

POEMS syndrome is a condition associated with endocrine and metabolic disorders. POEMS syndrome affects men more than women and usually occurs in the 40s or 50s, although cases have been reported in the 20s.

1. Causes of POEMS syndrome

POEMS syndrome is a rare multiple system disorder. POEMS is an acronym for:
- (P) polyneuropathy, a disease that affects many nerves.
- (O) organomegaly, abnormal enlargement of some internal organs.
- (E) endocrinopathy, a disease that affects certain hormone-producing glands that help regulate sexual function and certain metabolic functions;
- (M) monoclonal gammopathy or M proteins.
- (S) skin, skin abnormalities.
The cause of POEMS syndrome is not well understood. POEMS syndrome is a paraneoplastic disorder, which means that patients present with signs and symptoms presenting as plasma cell disorders (most of these patients have multiple myeloma or abnormal M protein in blood). However, the mechanism of this disorder is unclear.
POEMS syndrome may be due to circulating immunoglobulins caused by plasma cell dysfunction. Circulating cytokines (IL-1-β, IL-6), vascular endothelial growth factor, and tumor necrosis factor-alpha were also increased.

2. Manifestations of POEMS . syndrome

Patients with POEMS syndrome may have the following:
Enlarged liver Enlarged lymph nodes Hypogonadism Type 2 diabetes Primary hypothyroidism Hyperparathyroidism Adrenal insufficiency (Addison's disease) Plasma cells produce too much IgA and Monoclonal IgG. Skin abnormalities (eg, hyperpigmentation, skin thickening, hirsutism, hair, angiomas) Other symptoms and signs of POEMS syndrome may include edema, ascites, pleural effusion, and macular edema spots and fever. Approximately 15% of patients with POEMS syndrome are associated with Castleman disease (lymphocyte life and function dysfunction, some forms associated with HIV infection or human herpesvirus 8).
Like other syndromes of uncertain pathophysiology, POEMS syndrome is diagnosed based on a cluster of symptoms and signs. Criteria included presentation of polyneuropathy and monoclonal hyperplasia plus any 2 other symptoms of the disorder.

3. Treatment of POEMS . syndrome

Timely treatment of POEMS syndrome is essential because it is a dangerous syndrome. Treatment is aimed at two purposes:
- The first is directed at treating the underlying plasma cell disorders (eg, fibromyalgia).
- The second is directed at improving the specific symptoms of each patient. Treatment may require the coordinated efforts of a team of specialists.
The use of radiation (radiotherapy) or surgical removal of localized fibrous lesions (eg, not widespread throughout the body) may provide temporary (sometimes permanent) relief of symptoms associated with POEMS syndrome.
In many cases, including those with extensive fibrotic or diffuse bone marrow involvement, treatment with certain anticancer drugs (chemotherapy), such as corticosteroids with cyclophosphamide or melphalan, can reduce the symptoms associated with POEMS syndrome. Many patients can be offered high-dose chemotherapy with a peripheral blood stem cell transplant.
Additional treatments used to treat POEMS syndrome include lenalidomide, thalidomide, and bortezomib. The role of the following treatments is less certain: anti-VEGF antibodies, interferon, intravenous immunoglobulin, plasmapheresis, and all forms of retinoic acid conversion.
For accurate diagnosis of POEMS syndrome, it is necessary to visit medical facilities with a team of experienced doctors and modern equipment. POEMS syndrome is a dangerous syndrome that needs early detection and treatment to prevent unwanted complications.
Vinmec International General Hospital is a high-quality medical facility in Vietnam with a team of highly qualified medical professionals, well-trained, domestic and foreign, and experienced.
A system of modern and advanced medical equipment, possessing many of the best machines in the world, helping to detect many difficult and dangerous diseases in a short time, supporting the diagnosis and treatment of doctors the most effective. The hospital space is designed according to 5-star hotel standards, giving patients comfort, friendliness and peace of mind.