Rajagopal Meyyappa Devan, Nachiappa Ganesh Rajesh, Parameswaran Sreejith, Puthiyottil Dhanin
Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India.
Department of Nephrology, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India.
BMJ Case Rep. 2018 Dec 22;11(1):e225899. doi: 10.1136/bcr-2018-225899.
Light chain cast nephropathy is the most frequent form of renal disease in plasma cell neoplasm showing precipitation of monoclonal immunoglobulin light chains in the lumen of the distal tubules. This has a typical morphological feature characterised by the presence of a fractured cast. In this article, we report an unusual case of light chain cast nephropathy exhibiting amyloidogenic potential with lamellated, spiculated appearance. These casts were positive for periodic acid-Schiff and Jones' silver stain, fuchsinophilic in Masson trichrome stain and showed apple-green birefringence under polarised light in Congo red stain. Complete haematological evaluation confirmed the presence of underlying plasma cell myeloma. The connotation of intratubular amyloid cast lies in the fact that this may represent an early phenomenon during the development of light chain cast nephropathy-associated systemic amyloidosis and may precede the formation of light chain amyloid in renal or extrarenal location.
轻链管型肾病是浆细胞肿瘤中最常见的肾脏疾病形式,表现为单克隆免疫球蛋白轻链在远端肾小管管腔内沉淀。它具有典型的形态学特征,以断裂管型的存在为特点。在本文中,我们报告了一例不寻常的轻链管型肾病病例,其具有淀粉样变潜能,呈现层状、针状外观。这些管型对过碘酸希夫染色和琼斯银染色呈阳性,在马松三色染色中呈嗜品红色,在刚果红染色的偏振光下显示苹果绿双折射。全面的血液学评估证实存在潜在的浆细胞骨髓瘤。肾小管内淀粉样管型的意义在于,这可能代表轻链管型肾病相关系统性淀粉样变发展过程中的早期现象,可能先于肾脏或肾外部位轻链淀粉样蛋白的形成。