PT  - JOURNAL ARTICLE
AU  - Liang, Shaoshan
AU  - Liang, Dongmei
AU  - Zhu, Xiaodong
AU  - Liang, Dandan
AU  - Xu, Feng
AU  - Tu, Yuanmao
AU  - Zeng, Caihong
TI  - Light chain only variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits: a clinicopathological study of three cases
AID  - 10.1136/jcp-2023-208795
DP  - 2024 Aug 01
TA  - Journal of Clinical Pathology
PG  - 551--556
VI  - 77
IP  - 8
4099  - http://jcp.bmj.com/content/77/8/551.short
4100  - http://jcp.bmj.com/content/77/8/551.full
SO  - J Clin Pathol2024 Aug 01; 77
AB  - Aims To explore the clinical and pathological features of light chain only variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID-LC).Methods From January 2010 to December 2022, patients who were diagnosed with PGNMID-LC were selected, and their clinical and pathological features were retrospectively analysed.Results 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.Conclusions 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.All data relevant to the study are included in the article or uploaded as online supplemental information.