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| 신생검으로 확진된 전신성 형 경쇄침착질환 |
| 이규백 , 이윤하 , 장세호 , 김윤구 , 오하영 , 김미경 |
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| Abstract |
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The light chain related renal and systemic diseases include myeloma(cast) nephropathy, light chain de- position disease, and AL amyloidosis. Light chain deposition 4sease is characterized by the deposition of the sujtance lacks staining properties of congo- red and a fibrillar amyloid ultrastructure, and it con- tains monoclonal light-chain determinants, either z or ㅅ. These light-chain deposits ultimately result in the impairment of renal and other organ function and account for much of the morbidity and mortality found in patients with these disorder. We experienced a case of 46-year-old man who showed renal failure, paresthesia and weakness of lower extremities. The immunoelectrophoresis of urine and serum revealed A -type M protein. Electromyo- gram and nerve conduction velocity showed sensory motor neuropathy. EKG and echocardiogram showed atrial fibrillation-flutter and infiltrating cardiac lesion. The histologic findings showed widespread il -light chain deposits including the kidney and small vessels of muscular tissue. Bone marrow aspiration showed atypical plasma cell infiltration consistent with multiple myeloma. We recommanded intensive chemotherapy but he refused it. One month later, he died. We report tbe case of light chain deposition disease associated with multiple myeloma with brief review of related literature. |
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