Multiple vascular abnormalities and a paradoxical combination of vitamin B12 deficiency and thrombocytosis in a case with POEMS syndrome.
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.
Lewerenz
J
J
Research Group Protective Signaling, Zentrum für Molekulare Neurobiologie and Department of Neurology, University Hospital Hamburg-Eppendorf, Germany. lewerenz@zmnh.uni-hamburg.de
Gocht
A
A
Hoeger
P H
PH
von den Driesch
P
P
Eckert
B
B
Lamszus
K
K
Stuerenburg
H-J
HJ
Methner
A
A
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*
D042461:Vascular Endothelial Growth Factor A