Multiple vascular abnormalities and a paradoxical combination of vitamin B12 deficiency and thrombocytosis in a case with POEMS syndrome

• Published in: Journal of neurology Vol. 250; no. 12; pp. 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: Journal Article
• Language: English
• Published: Berlin Springer 01.12.2003; Springer Nature B.V
• 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; Endocrinopathy; Skin disease; pulmonary hypertension; Vitamin; Cardiovascular disease; Hemopathy; Vascular disease; Immunoglobulinopathy; arteriovenous malformations; Adult; Female; Thrombocytosis; Diagnosis; Arteriovenous malformation; Human; Immunopathology; Nervous system diseases; Deficiency; VEGF; Congenital disease; Cyanocobalamin; POEMS syndrome; Case study; Concomitant disease; Platelet; Vascular endothelium growth factor
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-003-0261-7

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.