POEMS SYNDROME - A RARE CASE REPORT

Abstract

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 polycythaemia. POEMS 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 polyneuropathy,peripheral
edema,back 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 POE