Abstract：
UNASSIGNED: Crystal-storing histiocytosis (CSH), light chain proximal tubulopathy (LCPT), and light chain crystalline podocytopathy (LCCP) are rare complications of multiple myeloma (MM) or monoclonal gammopathy of renal significance, and their diagnoses are challenging.
UNASSIGNED: In this case, a 69-year-old Chinese woman presented with suspicious Fanconi syndrome with renal insufficiency. Immunofixation electrophoresis of both serum and urine revealed elevated immunoglobulin G kappa (IgGkappa) and kappa light chain. Bone marrow aspirate revealed 15% plasma cells with considerable cytoplasmic granular inclusions and needle-shaped crystals. Renal biopsy confirmed the final pathologic diagnosis of kappa-restricted CSH, combined LCPT and LCCP by immunoelectron microscopy. A number of special casts were present which could easily be misdiagnosed as light chain cast nephropathy. Immunofluorescence on frozen tissue presented false negative for kappa light chain, as ultimately proven by paraffin-embedded tissue after pronase digestion. MM and CSH were diagnosed, and two cycles of chemotherapy were given. The patient subsequently refused further chemotherapy, and her renal function remained relatively stable during a 2.5-year follow-up period.
UNASSIGNED: In conclusion, we report a rare case of generalized kappa-restricted CSH involving bone marrow and kidney, combined with LCPT and LCCP, provide a comprehensive summary of renal CSH, and propose a new nomenclature of monoclonal immunoglobulin-induced crystalline nephrology. The presentation of monoclonal immunoglobulin and Fanconi syndrome should suggest the presence of monoclonal immunoglobulin-induced crystalline nephrology. Use of paraffin-embedded tissue after pronase digestion and immunoelectron microscopy is beneficial to improve the sensitivity of diagnosis.
UNASSIGNED: In this case, a 69-year-old Chinese woman presented with suspicious Fanconi syndrome with renal insufficiency. Immunofixation electrophoresis of both serum and urine revealed elevated immunoglobulin G kappa (IgGkappa) and kappa light chain. Bone marrow aspirate revealed 15% plasma cells with considerable cytoplasmic granular inclusions and needle-shaped crystals. Renal biopsy confirmed the final pathologic diagnosis of kappa-restricted CSH, combined LCPT and LCCP by immunoelectron microscopy. A number of special casts were present which could easily be misdiagnosed as light chain cast nephropathy. Immunofluorescence on frozen tissue presented false negative for kappa light chain, as ultimately proven by paraffin-embedded tissue after pronase digestion. MM and CSH were diagnosed, and two cycles of chemotherapy were given. The patient subsequently refused further chemotherapy, and her renal function remained relatively stable during a 2.5-year follow-up period.
UNASSIGNED: In conclusion, we report a rare case of generalized kappa-restricted CSH involving bone marrow and kidney, combined with LCPT and LCCP, provide a comprehensive summary of renal CSH, and propose a new nomenclature of monoclonal immunoglobulin-induced crystalline nephrology. The presentation of monoclonal immunoglobulin and Fanconi syndrome should suggest the presence of monoclonal immunoglobulin-induced crystalline nephrology. Use of paraffin-embedded tissue after pronase digestion and immunoelectron microscopy is beneficial to improve the sensitivity of diagnosis.
摘要：
未经批准：储存晶体的组织细胞增生症（CSH），轻链近端肾小管病（LCPT），轻链晶体性足细胞病（LCCP）是多发性骨髓瘤（MM）或具有肾脏意义的单克隆丙种球蛋白病的罕见并发症，他们的诊断具有挑战性。
UNASSIGNED：在这种情况下，一名69岁的中国妇女出现可疑的Fanconi综合征并伴有肾功能不全。血清和尿液的免疫固定电泳显示免疫球蛋白Gκ（IgGkappa）和κ轻链升高。骨髓穿刺液显示有15％的浆细胞，具有大量的细胞质颗粒内含物和针状晶体。肾活检证实了kappa限制性CSH的最终病理诊断，联合LCPT和LCCP通过免疫电子显微镜。存在许多特殊的管型，很容易被误诊为轻链管型肾病。冷冻组织免疫荧光显示κ轻链假阴性，链霉蛋白酶消化后，石蜡包埋的组织最终证明了这一点。诊断为MM和CSH，给予2个周期的化疗。患者随后拒绝进一步化疗，在2.5年的随访期间，她的肾功能保持相对稳定。
未经批准：总而言之，我们报道了一例罕见的广泛性Kappa限制性CSH，涉及骨髓和肾脏，结合LCPT和LCCP，提供肾脏CSH的全面总结，并提出了单克隆免疫球蛋白诱导的晶体肾病的新命名法。单克隆免疫球蛋白和Fanconi综合征的出现应提示存在单克隆免疫球蛋白诱导的晶体肾病。使用链霉蛋白酶消化后的石蜡包埋组织和免疫电子显微镜有利于提高诊断的敏感性。
UNASSIGNED：在这种情况下，一名69岁的中国妇女出现可疑的Fanconi综合征并伴有肾功能不全。血清和尿液的免疫固定电泳显示免疫球蛋白Gκ（IgGkappa）和κ轻链升高。骨髓穿刺液显示有15％的浆细胞，具有大量的细胞质颗粒内含物和针状晶体。肾活检证实了kappa限制性CSH的最终病理诊断，联合LCPT和LCCP通过免疫电子显微镜。存在许多特殊的管型，很容易被误诊为轻链管型肾病。冷冻组织免疫荧光显示κ轻链假阴性，链霉蛋白酶消化后，石蜡包埋的组织最终证明了这一点。诊断为MM和CSH，给予2个周期的化疗。患者随后拒绝进一步化疗，在2.5年的随访期间，她的肾功能保持相对稳定。
未经批准：总而言之，我们报道了一例罕见的广泛性Kappa限制性CSH，涉及骨髓和肾脏，结合LCPT和LCCP，提供肾脏CSH的全面总结，并提出了单克隆免疫球蛋白诱导的晶体肾病的新命名法。单克隆免疫球蛋白和Fanconi综合征的出现应提示存在单克隆免疫球蛋白诱导的晶体肾病。使用链霉蛋白酶消化后的石蜡包埋组织和免疫电子显微镜有利于提高诊断的敏感性。
