How much do you know about POEMS syndrome?

  POEMS syndrome, also known as Crow-Fucase syndrome, was first reported by Crow and Fucase in 1956 and 1968, respectively. It is a multisystemic disease with multiple peripheral neuropathies as the main clinical manifestation, and almost all cases are combined with myeloma or extramedullary plasmacytoma or present with M protein or polyclonal protein throughout the course of the disease.  POEMS syndrome: P (polyneuropathy) multiple peripheral neuropathy (numbness and weakness of the extremities, mainly distal weakness of the lower extremities), O organomegaly (normal liver and spleen, liver function, enlarged lymph nodes), E (endocrinopathy) endocrinopathy (gonadal-hypogonadism; thyroid-hypothyroidism; adrenal-cortical insufficiency; diabetes mellitus M(M-protein), M(M-protein) (confirmed by protein electrophoresis or immunofixation electrophoresis, usually IgG or IgA lamda type) and S(skinchanges) (diffuse skin pigmentation – darkening and hardening of the skin, increased and hardened body hair). . Other clinical manifestations: hypothermia, hyperhidrosis, ascites, pleural effusion and edema, pulmonary hypertension, optic papilledema, etc.  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. Patients with early onset of unexplained peripheral nerve damage edema (mainly in both lower limbs and face) and organ enlargement should think about the possibility of this disease.  Possible test abnormalities: serum immunofixation electrophoresis reveals M protein and the M protein is IgG or IgA lamda type; electromyography confirms peripheral neuropathy; endocrinological examination confirms endocrine abnormalities; serum VEGF level test, etc.