Liang Shaoshan, Liang Dongmei, Zhu Xiaodong, Liang Dandan, Xu Feng, Tu Yuanmao, Zeng Caihong
National Clinical Research Center of Kidney Diseases, Affiliated Jinling Hospital, Medical School, Nanjing University, Nanjing, China.
National Clinical Research Center of Kidney Diseases, Affiliated Jinling Hospital, Medical School, Nanjing University, Nanjing, China
J Clin Pathol. 2024 Jul 18;77(8):551-556. doi: 10.1136/jcp-2023-208795.
To explore the clinical and pathological features of light chain only variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID-LC).
From January 2010 to December 2022, patients who were diagnosed with PGNMID-LC were selected, and their clinical and pathological features were retrospectively analysed.
Three males aged 42-61 years old were enrolled. Hypertension was present in three patients, oedema in three patients, anaemia in two patients, proteinuria in three patients, nephrotic syndrome in one patient, microscopic haematuria in three patients, renal insufficiency in two patients and hypocomplementaemia of C3 in one patient. Elevated serum-free LC ratios and plasmacytosis on bone marrow smears were observed in three patients, and κ was identified by serum protein immunofixation electrophoresis in one patient. Renal biopsy showed membranoproliferative glomerulonephritis in two patients and endocapillary proliferative glomerulonephritis in one patient on light microscopy. Immunofluorescence indicated restricted κ LC and C3 distributed in glomeruli. By electron microscopy, electron-dense deposits without substructure were identified predominantly in the mesangial and subendothelial regions and were variable in the subepithelial region. Two patients were treated with plasma cell-directed chemotherapy and achieved haematological complete response or very good partial response, and one of them achieved a renal status of complete remission. One patient treated with immunosuppressive therapy only did not achieve haematological or renal remission.
PGNMID-LC is a rare and uniform disease with a high frequency of a detectable pathogenic plasma cell clone and is characterised by glomerular deposition of restricted LC and C3 in renal pathology. Plasma cell-directed chemotherapy may improve haematological and renal prognosis.
探讨仅伴轻链变异的单克隆免疫球蛋白沉积性增殖性肾小球肾炎（PGNMID-LC）的临床和病理特征。
选取2010年1月至2022年12月期间诊断为PGNMID-LC的患者，回顾性分析其临床和病理特征。
纳入3例年龄在42至61岁的男性患者。3例患者有高血压，3例有水肿，2例有贫血，3例有蛋白尿，1例有肾病综合征，3例有镜下血尿，2例有肾功能不全，1例有C3补体降低。3例患者血清游离轻链比值升高且骨髓涂片可见浆细胞增多，1例患者血清蛋白免疫固定电泳鉴定为κ型。光镜下，2例患者肾活检显示为膜增生性肾小球肾炎，1例为毛细血管内增生性肾小球肾炎。免疫荧光显示κ轻链和C3在肾小球内呈局限性分布。电镜下，主要在系膜区和内皮下区域发现无亚结构的电子致密沉积物，上皮下区域沉积物情况不一。2例患者接受了针对浆细胞的化疗，血液学达到完全缓解或非常好的部分缓解，其中1例患者肾脏状态完全缓解。1例仅接受免疫抑制治疗的患者未达到血液学或肾脏缓解。
PGNMID-LC是一种罕见且具有一致性的疾病，可检测到致病性浆细胞克隆的频率较高，肾脏病理特征为局限性轻链和C3在肾小球沉积。针对浆细胞的化疗可能改善血液学和肾脏预后。
