POEMS syndrome

ABSTRACT

POEMS syndrome is the syndrome of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonalprotein and typical Skin changes. A 65-year-old lady presented with the 2-day-history of inabilityto walk, 4-month-history of progressive worsening of muscle weakness of both lower limbs and1-year-history of progressive worsening of bilateral numbness of lower limbs. Nerve conduction studyrevealed generalized sensorimotor demyelinating polyneuropathy. She was initially treated as chronicinflammatory demyelinating polyradiculoneuropathy with intravenous immunoglobulin (IVIG) andhigh-dose prednisolone. However, she had no significant neurological improvement despite gettingstandard therapy. In addition to peripheral neuropathy, the presence of hepatosplenomegaly, skinchanges, polycythaemia and thrombocytosis prompted for further investigations. She was diagnosedas POEMS syndrome based on the presence of two mandatory major criteria [polyneuropathy,monoclonal plasma cell proliferative disorder (lambda)], one major criterion (sclerotic bone lesions)and three minor criteria (organomegaly, skin changes and thrombocytosis/polycythaemia). Shereceived treatment with melphalan and prednisolone. She achieved clinical improvement and partialresponse (haematologic and radiological) after six cycles of therapy. We highlight the awarenessof this rare syndrome, for patients presenting with peripheral neuropathy and not responding to itsstandard therapy, by recognizing other associated clinical manifestations and proceeding furtherdiagnostic work-up.