Amyloidosis를 동반한 POEMS 증후군 1예
A case of POEMS syndrome associated with amyloidosis
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초록
POEMS 증후군은 다발성 신경병증(polyneuropathy), 장기 종대(organomegaly), 내분비장애(endocrinopathy), 단일클론성 단백질(monoclonal gammopathy), 피부 병변(skin change)을 주 양상으로 발현되는 드문 증후군으로, 본 예에서는 신조직 소견상 아밀로이드 침착이 관찰된 POEMS 증후군 환자 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.
Abstract
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal M protein, and skin lesion) is a rare multisystemic disease of unknown cause with varying clinical manifestations. Amyloidosis-associated POEMS Syndrome is also rare condition. We experienced a 63-year-old female who had been suffered from edema and tingling sensation of low extremities. She also had a marked demyelination, axonal degeneration and regeneration of the nerve, hypoaldosteronism, hyperprolactinemia, decreased sexual hormone, monoclonal gammopathy (IgG λ type), skin change and ascites. The renal biopsy revealed homogenous mesangial widening with a few cell nuclei at the glomerular capillary, and it showed positive apple-green birefringence in Congo-red stain. It's the first report that shows the deposition of amyloid in patients with POEMS syndrome in Korea. Edema and M-spike were improved by use of prednisolone, melphalan, fludrocortisone and colchicine.
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