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 undetermin...

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Bibliographic Details
Published in:Journal of neurology 2003-12, Vol.250 (12), p.1488-1491
Main Authors: LEWERENZ, J, GOCHT, A, HOEGER, P. H, VON DEN DRIESCH, P, ECKERT, B, LAMSZUS, K, STUERENBURG, H.-J, METHNER, A
Format: Article
Language:English
Subjects:
Adult
Arteriovenous Fistula - complications
Biological and medical sciences
Female
Humans
Immunodeficiencies. Immunoglobulinopathies
Immunoglobulinopathies
Immunopathology
Lung - blood supply
Lung - pathology
Lung Diseases - complications
Lung Diseases - pathology
Medical sciences
Paraproteinemias - complications
Paraproteinemias - physiopathology
POEMS Syndrome - blood
POEMS Syndrome - complications
POEMS Syndrome - pathology
POEMS Syndrome - physiopathology
Spinal Cord - blood supply
Spinal Cord - pathology
Spinal Cord Diseases - complications
Spinal Cord Diseases - pathology
Thrombocytosis - complications
Vascular Diseases - complications
Vascular Diseases - pathology
Vascular Endothelial Growth Factor A - blood
Vitamin B 12 Deficiency - complications
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Summary: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.
ISSN:0340-5354
1432-1459
DOI:10.1007/s00415-003-0261-7