title: Multiple vascular abnormalities and a paradoxical combination of vitamin B12 deficiency and thrombocytosis in a case with POEMS syndrome.
abstract: 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.affiliation: Research Group Protective Signaling, Zentrum für Molekulare Neurobiologie and Department of Neurology, University Hospital Hamburg-Eppendorf, Germany. lewerenz@zmnh.uni-hamburg.de
authors.forename: J
authors.initials: J
authors.lastname: Lewerenz
authors.forename: A
authors.initials: A
authors.lastname: Gocht
authors.forename: P H
authors.initials: PH
authors.lastname: Hoeger
authors.forename: P
authors.initials: P
authors.lastname: von den Driesch
authors.forename: B
authors.initials: B
authors.lastname: Eckert
authors.forename: K
authors.initials: K
authors.lastname: Lamszus
authors.forename: H-J
authors.initials: HJ
authors.lastname: Stuerenburg
authors.forename: A
authors.initials: A
authors.lastname: Methner
mesh_terms: D000328:Adult; D001164:Arteriovenous Fistula / Q000150:complications; D005260:Female; D006801:Humans; D008168:Lung / Q000098:blood supply / Q000473:pathology; D008171:Lung Diseases / Q000150:complications / Q000473:pathology; D016878:POEMS Syndrome / Q000097:blood / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D010265:Paraproteinemias / Q000150:complications / Q000503:physiopathology; D013116:Spinal Cord / Q000098:blood supply / Q000473:pathology; D013118:Spinal Cord Diseases / Q000150:complications / Q000473:pathology; D013922:Thrombocytosis / Q000150:complications*; D014652:Vascular Diseases / Q000150:complications* / Q000473:pathology; D042461:Vascular Endothelial Growth Factor A / Q000097:blood; D014806:Vitamin B 12 Deficiency / Q000150:complications*
chemical_list: D042461:Vascular Endothelial Growth Factor A