Kijner C H, Yousem S A
Fairfax Hospital Department of Pathology, Falls Church, Virginia.
Am J Surg Pathol. 1988 May;12(5):405-13. doi: 10.1097/00000478-198805000-00009.
This case report describes the primary manifestation of systemic light chain deposition disease as bilateral nodules on chest radiographs. Although this case was initially classified as amyloidosis, a subsequent renal biopsy and ultrastructural, histochemical, and immunohistochemical studies allowed its distinction from amyloidosis. Kappa light chains were expressed with immunoperoxidase studies on paraffin-embedded tissue, and ultrastructural studies showed the dense, granular deposits characteristic of systemic light chain deposition disease. Serum and urine electrophoresis showed a monoclonal spike, but no plasmacytosis was identified at bone marrow biopsy and autopsy. The literature on this subject is also herein reviewed, with particular attention to the extrarenal manifestations of systemic light chain deposition disease.
本病例报告描述了系统性轻链沉积病的主要表现为胸部X线片上的双侧结节。尽管该病例最初被归类为淀粉样变性,但随后的肾活检以及超微结构、组织化学和免疫组织化学研究使其与淀粉样变性得以区分。通过对石蜡包埋组织进行免疫过氧化物酶研究发现κ轻链表达,超微结构研究显示出系统性轻链沉积病特有的致密颗粒状沉积物。血清和尿液电泳显示单克隆峰,但骨髓活检和尸检未发现浆细胞增多。本文还对该主题的文献进行了综述,特别关注系统性轻链沉积病的肾外表现。