ORIGINAL COMMUNICATION

Journal of Neurology

, Volume 250, Issue 12, pp 1488-1491

First online:

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

  • J. LewerenzAffiliated withResearch Group Protective Signaling, Zentrum für Molekulare Neurobiologie and Department of Neurology, University Hospital Hamburg-Eppendorf Email author 
  • , A. GochtAffiliated withDepartment of Pathology, University Hospital
  • , P. H. HoegerAffiliated withDepartment of Dermatology, University Hospital
  • , P. von den DrieschAffiliated withDepartment of Dermatology, University Hospital
  • , B. EckertAffiliated withDepartment of Neuroradiology, University Hospital
  • , K. LamszusAffiliated withDepartment of Neurosurgery, University Hospital
  • , H.-J. StuerenburgAffiliated withDepartment of Neurology, University Hospital
  • , A. MethnerAffiliated withResearch Group Protective Signaling, Zentrum für Molekulare Neurobiologie and Department of Neurology, University Hospital Hamburg-Eppendorf

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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, Mprotein, and skin changes. We report a case of POEMS syndrome due to a gammopathy of undetermined significance with thrombocytosis, vitamin B12 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.

Key words

POEMS syndrome VEGF thrombocytosis arteriovenous malformations pulmonary hypertension