What is POEMS syndrome?

  POEMS syndrome is a rare monoclonal plasma cell proliferative disease with a relatively benign course, but with a high disability rate and an extremely poor quality of life for patients. The main manifestations are, in alphabetical order: P polyneuropathy (including numbness and weakness of the extremities, predominantly distal weakness of the lower extremities), O organomegaly (including hepatosplenomegaly, lymph node enlargement, lymph node biopsy pathology often showing Castleman’s disease), E endocrine abnormalities (including gonadal-hypogonadism; thyroid-hypothyroidism; adrenal-cortical insufficiency; diabetes mellitus, etc.), M presence of serum M protein (confirmed by protein electrophoresis or immunofixation electrophoresis, usually IgG or IgA lamda type), S skin changes (skin color darkening and hardening, increased body hair hardening), other manifestations are ascites, pleural effusion and edema, pulmonary hypertension, optic papilledema and so on.  1.When should I suspect the possibility of POEMS syndrome?  Unexplained limb weakness or numbness; Unexplained ascites, pleural effusion or edema; Unexplained hepatosplenomegaly; Unexplained hypogonadism; Unexplained skin darkening and other changes 2.How to confirm the diagnosis of POEMS syndrome?  The final diagnosis requires the clinical judgment of experienced clinicians. 3. The efficiency is about 60%, the cost is about 100,000, but there is a possibility of relapse; our hospital has performed autologous stem cell transplantation for POEMS syndrome in more than 10 cases, the results are satisfactory; MD program: for patients who do not have the financial or physical conditions to perform autologous HSCT, they can choose MD program, our hospital currently treats 10 cases of POEMS syndrome with MD program, the efficiency is about 50%, the effect is about 3 months. The effect is about 3 months, but the MD regimen has a long course (usually 10 to 12 courses); other experimental treatments: for example, Avastin, etc., the efficacy is not certain.