POEMS 증후군에서 단독 α Heavy Chain Monoclonal Gammopathy 증례 보고

초록

POEMS syndrome has been known as a rare multisystemic disease associated with plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. These patterns of M protein were identified that IgA λ or IgG λ is occupied with nearly almost portions and IgM λ IgG κ or isolated light chain is with minor portions. The 45-year-old Korean man presented with abdominal distension, progressive paresthesia, and motor weakness of both lower extremities. Neurological evaluation revealed the motor of lower extremities was significantly weak and tendon reflex was lost. The electromyogram demonstrated the chronic inflammatory demyelinating polyneuropathy. M-band on serum PEP and isolated IgA heavy chain on serum immunofixation electrophresis were demonstrated. but light chain not detected. Endocrine study shows thyroid stimulating hormone 8.819 uIU/ml, free thyroglobulin 0.86 ng/dl, HbA1C 7.6%. The radiology investigation showed bone marrow hyperplasia on bone marrow scan using Tc-99m tincolloid, suggesting systemic hematologic disorder. Bone marrow aspiration and biopsy result in normocellular marrow, consisting of about 3% of plasma cell and increased number of megakaryocytes with hyperlobulated muclei