POEMS syndrome with vascular transformation of the lymph node sinuses: A case report.

Abstract

POEMS syndrome is a rare multisystem disorder associated with the clinical signs of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. However, there is often a delay in the diagnosis due to a lack of overall consideration of the symptoms collectively. For this reason, POEMS syndrome is frequently mistaken for other diseases, such as chronic inflammatory demyelinating polyneuropathy. The present study reports the case of a 40-year-old female patient, who presented with a progressive lack of strength in the lower limbs and a unilateral cervical lump. The patient's enlarged cervical lymph nodes were mistaken for local hemangioma. However, subsequently POEMS syndrome with vascular transformation of the lymph node sinuses (VTS) was diagnosed. The patient received glucocorticoid treatment (20 mg prednisone acetate, daily), which is ongoing. The most recent follow-up examination revealed that the patient's strength had improved and at the time of writing the patient remained alive. The study discusses the clinical manifestations, auxiliary examinations and reason for the misdiagnosis. Hematoxylin and eosin staining and cluster of differentiation 31 immunostaining were adopted to identify the VTS. To the best of our knowledge, this is the first report of POEMS syndrome with VTS.