A case of POEMS syndrome associated with amyloidosis.
- Author:
Eun Ju LEE
1
;
Heui Ryn JU
;
Seo Heui RA
;
Jin Yoeng HAN
;
Won Suk AN
;
Seong Eun KIM
;
Ki Hyun KIM
Author Information
1. Department of Internal Medicine, Dong-A University College of Medicine, Busan, Korea. kimkh@daunet.donga.ac.kr
- Publication Type:Case Report
- Keywords:
POEMS syndrome;
Amyloidosis
- MeSH:
Amyloid;
Amyloidosis*;
Ascites;
Axons;
Biopsy;
Birefringence;
Capillaries;
Cell Nucleus;
Colchicine;
Demyelinating Diseases;
Edema;
Extremities;
Female;
Fludrocortisone;
Humans;
Hyperprolactinemia;
Hypoaldosteronism;
Korea;
Melphalan;
Middle Aged;
Paraproteinemias;
POEMS Syndrome*;
Prednisolone;
Regeneration;
Sensation;
Skin
- From:Korean Journal of Medicine
2004;66(4):425-431
- CountryRepublic of Korea
- Language:Korean
-
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 lambda 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.
