Korean J Med.
2004 Apr;66(4):425-431.
A case of POEMS syndrome associated with amyloidosis
- Affiliations
-
- 1Department of Internal Medicine, Dong-A University College of Medicine, Busan, Korea. kimkh@daunet.donga.ac.kr
- 2Department of Pathology, Dong-A University College of Medicine, Busan, Korea.
- 3Department of Clinical Pathology, Dong-A University College of Medicine, Busan, Korea.
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.
Keyword
MeSH Terms
-
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
Amyloid
Colchicine
Fludrocortisone
Melphalan
Prednisolone
