Monoclonal Gammopathy of Undetermined Significance (MGUS)와 관련된 경쇄침착질환 1예 = A Case of Idiopathic Light Chain Deposition Disease

다국어 초록 (Multilingual Abstract)

Light chain deposition disease (LCDD) is a systemic disorder characterized by the deposition of monoclonal immunoglobulin light chains (LCs) in various organs. As LCs are overproduced by an abnormal clone of B cells, LCDD is usually described in the course of plasma cell dyscrasias or other lymphoproliferative disorders. However, it can occur in the absence of any detectable hematological disorder even during prolonged follow-up. We experienced a case of 62-year-woman who presented generalized edema, massive proteinuria and renal insufficiency. The histologic findings showed nodular glomerular mesangial expansion and prominent ribbon like immunohistochemical staining for kappa-light chain in the glomerular capillary wall. There was no evidence of multiple myeloma in bone marrow biopsy specimen. Because she refused the intensive chemothrapy, low dose of prednisolone and cyclophosphamide were prescribed for 6 months. Her renal function was maintained relatively well without renal replacement therapy for 7 months. We report a case of idiopathic LCDD not associated with multiple myeloma or other plasma cell dysclasia. (Korean J Nephrol 2005;24(1):146-151)

국문 초록 (Abstract)

경쇄침착질환 (light chain deposition disease, LCDD)은 단일클론성 면역글로부린이 신장을 비롯한 여러 장기에 침착되어 다양한 임상상을 나타나는 전신성 질환으로 Randall 등1)에 의하여 처음 기술되었다. 이러한 LCDD는 형질세포질환 (plasma cell dysclasia) 및 림프증식질환 (lymphoproliferative disorder)의 임상 경과 중에 발견되는 예가 많지만 혈액학적 질환과 관련이 없는 특발성 LCDD도 드물게 나타날 수 있다고 한다2-5). 경쇄는 신장을 비롯한 여러 장기에 침착될 수 있으며 신장조직에 과도하게 침착되면 심한 단백뇨 및 신부전증 등의 임상상을 보인다2). LCDD에 대한 국내보고는 매우 드물어 다발성골수종과 동반된 증례가 몇 예5,6) 있었으나 다발성골수종과 관련되지 않은 LCDD에 대한 보고는 아직 없었다. 저자들은 최근 전신부종 및 신부전증으로 입원하여 골수검사상 다발성골수종이 없었던 환자에서 발생한 LCDD 1예를 경험하여 문헌고찰과 함께 보고한다.

참고문헌 (Reference)

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