Young patient with arterial thrombosis and skin changes as the onset manifestations: POEMS syndrome

Abstract

POEMS syndrome is a rare multi-systemic disease characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes. Arterial thrombosis is a distinctively unusual feature in patients with POEMS syndrome. We report a 33-year-old man with intermittent amaurosis of left eye and skin changes as the onset manifestations, who was finally confirmed as having POEMS syndrome. Most notably, this was a young man without high risk factors of arterial thrombosis and no monoclonal protein was detected until the repeated measurement later. This case evokes the need to consider the diagnosis of POEMS syndrome for young patients with symptoms of arterial thrombosis but no high risk factors of thrombosis.

Ischemic stroke in patients with POEMS syndrome: a case report and comprehensive analysis of literature

“…However, the frequencies of stroke risk factors such as diabetes mellitus, hypertension and atrial fibrillation occurred in our study are similar to or even lower than those in stroke registries [ 54 , 55 ]. In accordance with previous literature, the preponderance of multiple cerebral vessel lesions and coexistence of extracerebral vessel disease in patients of POEMS without stroke risk factors are also more common than those reported in stroke registries [ 13 - 14 , 22 - 24 , 27 - 28 , 38 ]. This phenomenon may suggest that POEMS syndrome has a predisposition towards large-vessel vasculopathy which could only be confirmed by biopsy.…”

“…The recommended treatments for POEMS syndrome include autologous stem cell transplantation, radiotherapy and melphalan-based chemotherapy [ 1 ]. It is worth noting that recurrent ischemic stroke events were interrupted after chemotherapy or corticosteroids therapy in several patients [ 12 , 25 , 27 ]. Immunomodulatory drugs including thalidomide and lenalidomide represent a new treatment option for the patients with POEMS syndrome, not only because of their excellent suppression of monoclonal plasma cell proliferation, but also because of the modulation of VEGF secretion and upregulated cytokines suppressed by these drugs [ 1 , 5 , 6 , 68 ].…”