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( Jeong Hwan Youk ),( Hyunk Yung Park ),( Young Il Koh ),( In Ho Kim ),( Seon Yang Park ),( Sung Soo Yoon ) 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1
Background: POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonality and skin changes) is a very rare monoclonal disorder. Except for a few case reports, clinical and laboratory characteristics of Korean patients with POEMS syndrome have not been uncovered yet. Methods: We reviewed the medical records of patients with POEMS syndrome in Seoul National University Hospital from January 2000 to June 2014. Patients who met the mandatory criteria of POEMS syndrome ((1) the evidence of monoclonality; (2) the evidence of polyneuropathy that is not explained by other causes ) were included. Comparison with historical control of multiple myeloma was performed. (Mayo Clin Proc. 2003 Jan;78(1):21-33. ) Results: A total of twenty-four patients (Male/Female = 15/9, median age = 52. 6 years old) fulfi lled the mandatory criteria. Osteosclerotic bone lesions and castleman`s disease were found in 45% and 12. 5% of patients, respectively. All of light chains in patients with systematic disease were λ. (IgA/IgG=12/9, light-chain disease=2) Only one patient had a plasmacytoma. The median overall survival (OS) was 64. 4 months. Six patients had autologous stem cell transplantation and they were all alive. Not a single clinical or laboratory factors predicted OS in these population. Interestingly, hemoglobin level(14. 3 g/dL vs 12. 4 g/dL; p=0. 012) and quantity of serum M-protein(0. 95 g/dL vs 0. 17 g/dL; p=0. 015) were significantly different between IgG and IgA subgroups. Compared to multiple myeloma, age at diagnosis was younger and hemoglobin and platelet level tended to be higher in POEMS syndrome. The median OS was longer than that of multiple myeloma. Conclusions: Patients with POEMS syndrome have unique features compared to those of multiple myeloma. In addition, it has been fi rst revealed that the laboratory fi ndings between immunoglobulin subgroups in POEMS syndrome are different.
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