POEMS SYNDROME - A RARE CASE REPORT

POEMS syndrome (also known as CrowFukase syndrome, Takatsuki disease, or PEP syndrome) is a rare medical syndrome. It is defined as the combination of a plasma-cell proliferative disorder (typically myeloma), polyneuropathy, and effects on many other organ systems. It begins in middle age the average age at onset is 50 and affects up to twice as many men as women Criteria The most recent criteria for the diagnosis of POEMS syndrome require that all of the following be present polyneuropathy, a monoclonal plasma-cell proliferative disorder one or more of the following major criteria sclerotic bone lesions ,Castleman's disease ,elevated VEGF levels ,one or more of the following minor criteria organomegaly (enlarged spleen, liver or lymph nodes)extravascular volume overload (oedema, pleural effusion or ascites)endocrinopathy (adrenal, pituitary, gonadal, parathyroid) diabetes or hypothyroidism alone are insufficient, skin changes papilloedema thrombocytosis or p o l y c y t h a e m i a . P O E M S Polyneuropathy,Organomegaly,Endocrinopathy,Mprotein, Skin changes .99 cases reported ,Mean survival rate 165 months .OUR CASE REPORT ,50 yr old male patient named muthu presented to us with clinical features p o l y n e u r o p a t h y , p e r i p h e r a l e d e m a , b a c k pain ,organomegaly,blurring vision,hypertension,lab findings favouring POEMS SYNDROME diagnosis .He was diagnosed to have L5 vertebral fracture without deficit.posterior stabilisation was done at L3,L4,S1 level.,On evaluation he showed features of this rare syndrome.,Patient was operated for solitary plasmacytoma of left scapula for which custom mega prosthesis done in 8 years back.usually in POEMS there is a preceding solitary plasmacytoma before manifestation of multiple myeloma,Other incidental findings Rectalpolyp(colonoscopy done in 2007), polypectomy done ,Biopsy reported as submucosal lipoma .With all the classical findings this patient showed features and fits into rare POEMS SYNDROME.