What is it?
POEMS syndrome is a rare multisystem disease defined by the coexistence of polyneuropathy (P) and monoclonal gammopathy (M) and their association with other changes, among which the most important that contribute to the definition of the syndrome are organogaly (O) (hepatomegaly, splenomegaly, lymphadenopathy), endocrinopathy (E) (adrenal glands, thyroid gland, pituitary gland, gonads, parathyroid gland and pancreas) and skin changes (S) (typical hyperpigmentation, hypertrichosis, cutaneous hemangiomas, acrocyanosis). The syndrome occurs more often in males (M / F: 2.5 / 1) and usually in the fifth to sixth decade of life, with an average onset age of 48 for males and 59 for females.
Which are the symptoms?
The onset symptoms are mainly associated with bilateral polyneuropathy, which affects both motor and sensory fibers of the lower extremities and manifests itself as muscle weakness in the arms and legs, tingling, numbness, burning pain and a lack of sensitivity. Organomegaly, that is, an increase in the size of some internal organs, most often lymph nodes, liver, spleen and, less often, heart, can be found in about half of patients. Among the endocrinological changes, the most common are hypothyroidism and adrenal insufficiency; Also, sexual function is often involved with an increase in the level of estrogen in the blood, which determines impotence and gynecomastia in men, that is, excessive development of the mammary glands, and in women - the absence of menstruation and hypertrichosis. Also common: diabetes / glucose intolerance.
How is it diagnosed?
In the blood of patients, there is usually an increase in the amount of some abnormal proteins called paraproteins or M proteins, which are parts of antibodies represented by light chains, or lambda, immunoglobulins A and G, or IgA lambda and IgG lambda, which are produced in excess due to abnormal proliferation of plasma cells. Other possible clinical manifestations include: skin changes such as hyperpigmentation, skin thickening, and excessive sweating; acrocyanosis, that is, the bluish color of the fingers and toes; Raynaud's phenomenon, a circulatory disorder characterized by periodic crises of vasospasm with discoloration of the skin; the development of angiomas, pathological formations consisting of blood or lymphatic vessels; vasculitic manifestations; alopecia, meaning hair loss. Swelling of the lower extremities and joint pain are also common. To confirm the clinical suspicion of POEMS syndrome, using immunoelectrophoresis, it is necessary to detect the presence in the blood and urine of paraprotein M associated with impaired proliferation of plasma cells. Radiographs allow identification of any lytic-sclerotic bone lesions due to proliferation of plasma cells. Electromyographic study of nerve conduction velocity, which serves to demonstrate the slowing down of the conduction rate of electrical impulses. The latest diagnostic criteria for the diagnosis of POEMS, published in 2011 (Dispenzieri 2011), require, in addition to the simultaneous presence of polyneuropathy and monoclonal gammopathy, the presence of one of three other main criteria (Castleman disease, sclerotic bone lesions and increased VEGF values) and one of six minor criteria (organogaly, effusion / edema / ascites, endocrinopathy, skin changes, papilledema, and thrombocytosis / polycythemia).
How is it treated?
Since the cause of POEMS syndrome is currently unknown, there is no standardized treatment for causation; however, the need for pretreatment of the plasma cell proliferative disorder is now being elucidated. In the case of a solitary plasmacytoma or isolated osteosclerotic lesion, the preferred treatment is radiation therapy or surgical removal, with frequent cure of the syndrome. However, in most patients, the disease of plasma cells is not a single, but a systemic lesion with diffuse infiltration of the bone marrow; in such cases, chemotherapy is indicated, which includes a combination of high doses of corticosteroids and immunosuppressants. The former are often used with benefit, but in and of themselves are not sufficient to control any relapses of the disease, if they are not associated with specific chemotherapy. Chemotherapy drugs must be carefully selected to rule out drugs that may have toxic effects on neuropathy. Therapy with high doses of intravenous immunoglobulin and plasmapheresis does not always give the desired effect. Some recent evidence suggests that Bevacizumab is effective against VEGF; however, the results are still under evaluation.
Where do we treat it?
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