Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin abnormalities (POEMS) syndrome in a Filipino nurse returning to work following bortezomib treatment: A case report

ABSTRACT

@#<p style="text-align justify;" data-mce-style="text-align justify;"><span style="color #525a63; font-family Lato, sans-serif; font-size 16px; font-style normal; font-variant-ligatures normal; font-variant-caps normal; font-weight 400; letter-spacing normal; orphans 2; text-align start; text-indent 0px; text-transform none; white-space normal; widows 2; word-spacing 0px; -webkit-text-stroke-width 0px; background-color #ffffff; text-decoration-thickness initial; text-decoration-style initial; text-decoration-color initial; display inline !important; float none;" data-mce-style="color #525a63; font-family Lato, sans-serif; font-size 16px; font-style normal; font-variant-ligatures normal; font-variant-caps normal; font-weight 400; letter-spacing normal; orphans 2; text-align start; text-indent 0px; text-transform none; white-space normal; widows 2; word-spacing 0px; -webkit-text-stroke-width 0px; background-color #ffffff; text-decoration-thickness initial; text-decoration-style initial; text-decoration-color initial; display inline !important; float none;">POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) also known as Crow-Fukase syndrome or Takatsuki syndrome is a rare and disabling paraneoplastic syndrome that frequently occurs in the fifth or sixth decade of life without a known standard first-line therapy. A 34-year-old Filipino male nurse, who presented with gradually progressive distal lower extremity weakness and sharp shooting pain in bilateral legs accompanied by mild joint effusion on the left knee, hypertrichosis, bilateral conjunctival injection, and gynecomastia underwent extensive workup and was diagnosed with POEMS syndrome. Complete blood count revealed erythrocytosis and thrombocytosis with elevated serum VEGF (vascular endothelial growth factor) and elevated monoclonal serum free lambda light chains. The electrophysiologic studies revealed chronic demyelinating sensorimotor polyneuropathy while bone marrow core biopsy and bone marrow aspirate smear immunohistochemical staining showed it to be positive for lambda and CD138. He had an initial unsuccessful treatment course with melphalan and prednisone. Hence, bortezomib and dexamethasone were given which gave significant improvement in symptoms from the overall neuropathy limitation score of 5 to 1.</span></p>