Linked Life Data

20673674

Source:http://linkedlifedata.com/resource/pubmed/id/20673674

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1-2
pubmed:dateCreated
2010-9-13
pubmed:abstractText
A POEMS syndrome is a rare disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin abnormalities including hyperpigmentation and hypertrichosis. Here we report a 55-year-old female case of a POEMS syndrome associated with Waldenstrom macroglobulinemia. The patient had bed-bound polyneuropathy, splenomegaly, IgM-? type monoclonal (M) protein, elevated ?-type free light chain (FLC), infiltration of CD20-positive lymphoplasmacytic cells in bone marrow, edema and hypertrichosis, and was diagnosed to have an 'atypical' POEMS syndrome associated with macroglobulinemia. Nerve conduction studies and a sural nerve biopsy confirmed a demyelination and axonal degeneration without IgM deposition on myelin sheathes. None of neuron-related auto-antibodies characteristic of IgM paraproteinemic neuropathies was detected in her serum and cerebrospinal fluid. Weekly administration of rituximab (375mg/m(2)) combined with thalidomide (50mg/day) was initiated. By eight weeks of the treatment, the ambulation activity of the patient was restored and her polyneuropathy completely disappeared as determined by clinical symptoms and electrophysiological examinations. This is the first case report presenting a POEMS syndrome associated with WM treated with rituximab and thalidomide. The further examinations of the present case should shed light on the pathogenesis of the 'atypical' POEMS syndrome.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Oct
pubmed:issn
1878-5883
pubmed:author
pubmed:copyrightInfo
Copyright 2010 Elsevier B.V. All rights reserved.
pubmed:issnType
Electronic
pubmed:day
15
pubmed:volume
297
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
101-4
pubmed:meshHeading
pubmed:year
2010
pubmed:articleTitle
Successful treatment with rituximab and thalidomide of POEMS syndrome associated with Waldenstrom macroglobulinemia.
pubmed:affiliation
Department of Hematology, Kumamoto University Hospital, 1-1-1 Honjo, Kumamoto 860-8556, Japan.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't