POEMS syndrome is a syndrome of plasmacytoma or plasma cell proliferation resulting in multisystem damage. It presents with progressive multiple peripheral neuropathies, hepatosplenomegaly, endocrine disorders, increased M-protein and skin pigmentation, and may present with generalized sunken edema, thoraco-abdominal fluid, pestle finger and heart failure. POEMS syndrome is a rare group of multi-system damage syndrome with polyneuropathy as the main manifestation. Due to the complex and variable manifestations of the first symptoms and different periods of the disease course, it is easy to misdiagnose and miss the diagnosis. There is no specific treatment for POEMS syndrome. Immunotherapy, surgery and radiotherapy are the main treatments. Chemotherapy, radiotherapy or surgical removal of plasmacytoma can be used to relieve symptoms in patients with myeloma, but not in non-myeloma patients. Treatment with adrenocorticotropic hormones may result in partial symptom relief. Patients who have failed to respond with prednisone and cyclophosphamide have improved various symptoms after switching to triamcinolone (Tamoxfen). It has been reported that in some cases where hormone therapy is ineffective, the application of plasma exchange, 2500-3000 ml each time for a total of 5 times, resulted in improvement of polyneuropathy and skin lesions. The use of blood paddle exchange method therapy is relatively narrow, the therapy is expensive, the treatment effect is relatively short maintenance time, usually can be maintained in about 7-15 days, so it is also not an effective method of treatment of this disease. Chinese medicine believes that POEMS syndrome is caused by body weakness and sweating, fluid depletion, and loss of tendons and veins in the moistening, Chinese medicine treatment should be and camping and muscle relief, soothing the Xiangqiang, through the meridians and live genera. In 1958, it was first described by Crow, and in 1968, Fukase presented it as an independent syndrome. 1980, Bardwick PA believed that the main manifestations of this syndrome are: Polygneuropathy, Organomegaly, Endocrinopathy, and Endocrinopathy. Endocrinopathy, M-protein, and Skin-changes. The syndrome is called POEMS syndrome after its initials. It is also known as Shimpos syndrome, PEP syndrome, Taktsuti syndrome, or PASEDOO syndrome. or PASEDOO syndrome. Currently, POEMS syndrome or Crow-Fukase syndrome is more commonly used. 2, clinical manifestations of this syndrome onset age 26-80 years, about 45 years old, the ratio of men to women is 2-3:1, is a chronic course, often involving multiple systems. 2.1. Chronic polyneuropathy is seen in all patients and is the most common first symptom. It is often progressive, symmetrical sensory and motor damage, developing from distal to proximal extremities, with numbness, pain, weakness, tenderness, gradual muscle atrophy, paralysis, and loss of tendon reflexes. Sensory involvement is often first, motor damage is second, and cranial nerve damage is less common. Individual patients have only motor impairment. In some patients, cerebrospinal fluid pressure is increased, protein is increased (>0.5g/L), sugar, chloride, and cell count are normal, suggesting separation of protein and cells. Optic papilledema, hyperhidrosis, hypotension, impotence, diarrhea, constipation, intestinal paralysis, and other vegetative neurological dysfunction are seen. Peripheral nerve biopsy showed varying degrees of axonal degeneration and/or segmental demyelination changes. Electromyography showed significant slowing of motor and sensory nerve conduction. 2.2. Organ enlargement Hepatosplenomegaly is more common, ranging from 62% to 82% and 39% to 62%, respectively, followed by diffuse lymph node enlargement. Other organs may also show changes, such as pulmonary fibrosis, pulmonary hypertension, cardiomyopathy and renal insufficiency. 2.3. Endocrine disorders often affect the gonads and thyroid gland. Inadequate secretion of gonadal hormones may lead to impotence and feminized breasts in men; amenorrhea, painful breast enlargement, breast overflow in women, increased estrogen, increased lactogen, decreased testosterone, hypothyroidism, partially hyperthyroidism, abnormal glucose tolerance, increased blood sugar, and changes in adrenal cortical function. M protein and bone marrow abnormalities are found in the blood of 75%-87% of patients, mostly IgG type, followed by IgA, and rarely IgM; M protein can also be found in the cerebrospinal fluid. 90% of M protein light chain measurements are λ type, rarely K type. 10% of patients have this-period protein in the urine. The bone marrow picture shows plasma cell hyperplasia, of which 50% are mildly hyperplastic, 8.5% are moderately hyperplastic, and 5% are severely hyperplastic; in a few patients, although no plasma cell hyperplasia is seen in the bone marrow, extramedullary plasmacytoma may be present. 2.5, skin changes common skin pigmentation, mainly in the limbs and head and face, can spread throughout the body, brown-black, black areola; skin thickening, hardening, hairy, itchy skin, some patients appear vascular warts on the trunk, the diameter is like rice grain – soybean size, pestle finger, Raynaud’s sign, nail whitening, etc.. 2.6, other edema is quite common, often the first symptom, mostly in the lower extremities sunken swelling, but also can be systemic, some patients combined with pleural effusion, ascites. In addition, symptoms such as hypothermia, excessive sweating, and pestle finger are seen in more than half of the patients. Some patients have isolated or multifocal bone disease on skeletal X-ray, which is divided into osteosclerotic type, mixed osteosclerotic and fused bone type and fused bone type, with the trunk, distal extremities and pelvis being involved. In 1984, Nakanishi proposed the basic clinical features and diagnostic criteria for POEMS syndrome. (1) Chronic progressive peripheral neuropathy, optic papilla edema, and increased cerebrospinal fluid protein. (ii) Enlarged liver, spleen and lymph nodes. ③Skin changes: hyperpigmentation, thickening, and increased hair. ④Endocrine changes: sexual dysfunction, impotence, amenorrhea, breast enlargement, combined with diabetes mellitus. ⑤ Edema: limb edema, pleural effusion, ascites. ⑥Abnormal proteinemia: presence of M protein, bone damage, etc. Others may include fever, hyperhidrosis, pestle finger, etc. Laboratory tests: serum protein electrophoresis may show M protein, increased blood sedimentation, the appearance of this – week protein in the urine, positive rheumatoid factor. The diagnosis of the disease can be made by having three or more of the above six conditions. The syndrome involves multiple systems, and the main symptoms may not appear at the same time, and the manifestations may vary at different times. The frequency of M-protein and urine peri-protein is significantly higher than that of POEMS syndrome, and X-rays generally show osteoporosis and multiple osteolytic destruction, while osteosclerosis is rare. Endocrine changes are less frequent than in POEMS syndrome, and bone marrow aspiration plasma cells are significantly higher. (ii) Chronic Grin-Barre syndrome, mainly manifested by multiple peripheral neuropathy and increased cerebrospinal fluid protein, usually without skin damage and endocrine dysfunction, without skeletal damage and M protein and plasma cell infiltration. (iii) Connective tissue disease, which can also present multi-systemic damage, but its muscle damage is mostly myogenic, serum protein electrophoresis does not show M protein, and autoantibodies can be measured in blood to differentiate. However, the coexistence of scleroderma or SLE with POEMS syndrome has been reported. 4. Etiology and pathogenesis The etiology is not known. It is generally believed to be an autoimmune disease associated with plasma cell proliferation producing abnormal immunoglobulins. The presence of IgG, IgA, IgM, λ, and k proteins in the serum of the patient was detected by the technique of radiotracer, and the ultrastructural examination of nerve biopsy revealed a large number of missing and demyelinated nerve axons, immunoglobulin deposits in the nerve endothelium, especially under the nerve endothelium, and M protein deposits on the nerve myelin sheath. After clinical application of immunosuppressants, resection or chemotherapy of isolated plasmacytoma, the disease may improve, and the blood γ-globulin decreases and M-protein turns negative, which supports the above view. The pathogenesis is not well understood. It may be related to the toxic effects of M-protein or lymphotropin secreted by plasma cells on the peripheral nervous system, endocrine glands, bone, reticuloendothelial system, hematopoietic system and autoimmune system; it may also be related to hyperviscosity. The relationship with amyloid deposition has been viewed differently and is mostly considered unrelated. Since treatment of focal myeloma can lead to improvement of symptoms such as peripheral neuropathy and skin changes, this evidence is also considered to be a distant effect of the tumor. Some patients have a history of exposure to trichloroethylene, pesticides, organic solvents, etc., so it is thought to be related to poisoning. Recently, it has been reported that interleukin-6 (IL-6) was elevated in blood, pleural fluid, ascites, glomeruli and vascular endothelial cells of patients and decreased after treatment. It is known that IL-6 can stimulate plasma cell proliferation in addition to inducing terminal differentiation of B cells, so IL-6 is related to its pathogenesis. It has also been suggested that it is related to chromosomal variation of plasma cells in the bone marrow. It has been shown that the concentration of vascular endothelial growth factor (VEGF) decreases after patients’ symptoms improve with steroid treatment, suggesting that VEGF plays an important role in the pathogenesis. In conclusion, the etiology and pathogenesis of POEMS syndrome are still unclear. It has been suggested that the underlying disease of POEMS syndrome is not homogeneous and can be an immune-related disorder of different causes, or that POEMS syndrome is a malignant Castleman disease, while Tahus considers POEMS syndrome as a paraneoplastic syndrome. The reason for this is to be further studied. 5, treatment, prognosis For POEMS syndrome, there is no special treatment in Western medicine. The main treatment is immunotherapy, surgery and radiation therapy. Those with myeloma can be treated with chemotherapy, radiotherapy or surgical removal of plasmacytoma to relieve symptoms, but not for non-myeloma patients. In 1992, Enveoldson et al. reported that a patient who was treated with prednisone and cyclophosphamide, which were ineffective, had improved symptoms after switching to triamcinolone acetonide (Tamoxfen). The application of plasma exchange, 2,500-3,000 ml each time for a total of 5 times, has been reported to improve polyneuropathy and skin lesions in those who have failed to respond to certain hormonal treatments.