POEMS syndrome, also known as Crow-Fukase syndrome or Takatsuki syndrome, is a very rare symptom complex that accompanies neoplastic disease of the hematopoietic system, which is the osteosclerotic form of multiple myeloma. What are the causes and symptoms of POEMS? What is the treatment?

POEMS team , otherwiseCrow-Fukase teamorTakatsuki team , it belongs to the group a rare disease and is a symptom complex that accompanies the osteosclerotic variety of multiple myeloma. This disease is based on the proliferation of plasma cells, which are responsible for the production and secretion of antibodies.

POEMS is very rare in the population. Men are sick more often than women. The mean age of onset of osteosclerotic multiple myeloma is 70 years.

POEMS syndrome - symptoms

The namePOEMS syndromecomes from the first letters of the symptoms characteristic of this syndrome:

P-polyneuropathy , a disease process that damages peripheral nerves, both sensory and motor. Symptoms of this condition include:

  • all sensory disorders
  • paresthesias
  • muscle weakness
  • paresis of limbs
  • abolition of deep reflexes
  • autonomic disorders incl. excessive sweating

Patients often report pain in the limbs and hyperalgesia. Initially, symptoms appear on the lower limbs, with time also on the upper limbs.

O-organomegaly , i.e. enlargement of internal organs. Most often it affects the parenchymal organs, such as:

  • liver (hepatomegaly)
  • spleen (splenomegaly)
  • lymph nodes (lymphadenopathy)

These organs are easily accessible by palpation of the abdomen, they extend beyond the costal arch, the liver beyond the right, and the spleen beyond the left costal arch. Physiologically, they are not palpable through the abdominal wall.

E-endocrinopathies , i.e. disorders of hormone secretion by endocrine glands. Hypogonadism is most frequently observed. It is a disorder consisting in a deficiency of sex hormones produced by the gonads (testes and ovaries), such as progesterone, estrogens andtestosterone. This results in :

  • lack of puberty
  • lack of typical male armpit or perineal hair
  • reducing libido
  • menstrual disorders in women

Other endocrinopathies are less frequently observed, such as:

  • gynecomastia (male mammary gland enlargement)
  • hypothyroidism
  • adrenal insufficiency
  • diabetes
  • hyperparathyroidism
  • hyperprolactinemia

M-monoclonal gammapathyis a disease involving the growth of a single clone of plasmocytes. Plasmocytes are the only cells in the immune system that produce and secrete antibodies. In this disease, a clone of plasmocytes produces a homogeneous (monoclonal) antibody - the M protein. It consists of two heavy chains of the same class and two identical light chains. The production of the M protein usually causes a reduction in the production of residual antibodies. This can reduce the body's resistance and make it more prone to infections.S-skin changesmost often include:

  • discoloration
  • increased skin pigmentation
  • excessive hair growth
  • hardening of the skin
  • skin thinning

The changes may also apply to nails.

POEMS team - reasons

The etiology of the POEMS team is unknown. VEGF, or vascular endothelial growth factor, is suspected to be involved in the development of POEMS. Patients with this symptom complex also have elevated serum levels of other cytokines, such as TNF alpha (tumor necrosis factor), interleukins IL-1 and IL-6.

POEMS team - diagnostics

Disease is most often diagnosed on the basis of symptoms, a physical examination by a doctor, and the results of laboratory tests of blood and urine. It may also be helpful to perform radiographic examinations of the skeletal system to show osteosclerotic changes and to perform a bone marrow biopsy.

Important

POEMS is very rare, so it is necessary to carefully analyze the test results and symptoms reported by the patient in order to make a correct diagnosis. There are no binding criteria for the diagnosis of this condition. It is considered necessary to have at least 3 of the 5 common symptoms, including 2 large (polyneuropathy and M protein) and 1 small (skin lesions, endocrinopathy, or organomegaly).

POEMS team: treatment

Treatment of POEMS and multiple myeloma is basedon the treatment of disorders of the hematopoietic system and removal of a pathological clone of plasma cells. Radiotherapy is the most frequently used method, less often systemic treatment such as chemotherapy and steroid therapy. After intensive treatment, the doctor may decide to transplant the patient with stem cells obtained from peripheral blood.

POEMS team: prognosis

The prognosis for a good response to treatment is good for osteosclerotic multiple myeloma.

Category:

Oncology