POEMS증후군 1예 보고

Abstract

This is a case report of unusual multisystem disorder first described as a unique syndrome in 1968, the POEMS syndrome. The acronym POEMS stands for Polyneuropathy, Organomegaly, Endocrinopathy, M-Protein, and Skin changes. The POEMS syndrome is also known as Crow-Fukase, or Takatsuki syndrome. Since 1968, more than one hundred cases have been reported in Japan and also several similar cases in other countries. As a as auther's knowledge, this may be the first case of the POEMS syndrome in Korea. The patient was a 32 years old male who had been admitted to the Severance hospital because of subacutely progressive quadriparesis and sensory changes. The patient was markedly emaciated and the skin was dark. On examination, the muslce bulk was markedly decreased with moderate to severe general weakness and the sensory functions were impaired in all modalities. The trunk and face were also involved, but worse in distal part of the extremities. Hepatomegaly and testicular hypotrophy was noticed on ;alpation. Laboratory studies disclosed hepatomegaly, hypothyroidism, hypogonadism, IgG monoclonal gammopathy, and sclerotic bone lesion at the thoracic spine. There was only transient symptomatic improvement on steroid, but followed by intractable progression. Complete atrio-ventricular conduction block with orthopnea occurred as a terminal event and the patient was expired at 10 months of the course."