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.
Highlights
POEMS syndrome, as a rare multi-systemic disease, is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes [1]
There are several clinical features that not represented in its acronym, such as sclerotic bone lesions, Castleman disease, vascular endothelial growth factor (VEGF) elevation, extravascular volume overload, papilledema, thrombocytosis/polycythemia, clubbing, weight loss, hyperhidrosis, pulmonary hypertension/restrictive lung disease, thrombotic diatheses, diarrhea and low vitamin B12 values [2,3,4]
A young man with arterial thrombosis and skin changes as the onset manifestations of POEMS syndrome was reported and similar cases recorded in literature were reviewed
Summary
POEMS syndrome, as a rare multi-systemic disease, is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes [1]. A 33-year-old man was admitted to our hospital due to intermittent amaurosis of his left eye that suddenly occurred one month before He had a one-year history of skin blackening, fatigue, impotence and emaciation. Laboratory tests including blood counts, a biochemistry panel and coagulation function were performed. Immunofixation electrophoresis test did not detect monoclonal protein in the serum and urine for the first time. We repeated the serum protein and immunofixation electrophoresis in another laboratory and this time the presence of monoclonal protein (λ-light chain in the γ region ) was detected (Figure 3).
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