Document Detail


Multiple vascular abnormalities and a paradoxical combination of vitamin B12 deficiency and thrombocytosis in a case with POEMS syndrome.
MedLine Citation:
PMID:  14673584     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
POEMS/Crow-Fukase syndrome is a rare multisystem disorder associated with elevated vascular endothelial growth factor (VEGF), which clinically presents with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes. We report a case of POEMS syndrome due to a gammopathy of undetermined significance with thrombocytosis, vitamin B(12) deficiency, highly elevated VEGF and in addition to glomeruloid angiomas two previously undescribed proliferative vascular lesions: a spinal arteriovenous fistula and a plexogenic pulmonary arteriopathy, which ultimately resulted in lethal pulmonary hypertension. We assume that the high VEGF levels caused the vascular abnormalities observed in our patient.
Authors:
J Lewerenz; A Gocht; P H Hoeger; P von den Driesch; B Eckert; K Lamszus; H-J Stuerenburg; A Methner
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of neurology     Volume:  250     ISSN:  0340-5354     ISO Abbreviation:  J. Neurol.     Publication Date:  2003 Dec 
Date Detail:
Created Date:  2003-12-15     Completed Date:  2004-02-24     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0423161     Medline TA:  J Neurol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  1488-91     Citation Subset:  IM    
Affiliation:
Research Group Protective Signaling, Zentrum für Molekulare Neurobiologie and Department of Neurology, University Hospital Hamburg-Eppendorf, Germany. lewerenz@zmnh.uni-hamburg.de
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MeSH Terms
Descriptor/Qualifier:
Adult
Arteriovenous Fistula / complications
Female
Humans
Lung / blood supply,  pathology
Lung Diseases / complications,  pathology
POEMS Syndrome / blood,  complications*,  pathology,  physiopathology
Paraproteinemias / complications,  physiopathology
Spinal Cord / blood supply,  pathology
Spinal Cord Diseases / complications,  pathology
Thrombocytosis / complications*
Vascular Diseases / complications*,  pathology
Vascular Endothelial Growth Factor A / blood
Vitamin B 12 Deficiency / complications*
Chemical
Reg. No./Substance:
0/Vascular Endothelial Growth Factor A

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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