RT Journal Article
SR Electronic
T1 Light chain only variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits: a clinicopathological study of three cases
JF Journal of Clinical Pathology
JO J Clin Pathol
FD BMJ Publishing Group Ltd and Association of Clinical Pathologists
SP 551
OP 556
DO 10.1136/jcp-2023-208795
VO 77
IS 8
A1 Liang, Shaoshan
A1 Liang, Dongmei
A1 Zhu, Xiaodong
A1 Liang, Dandan
A1 Xu, Feng
A1 Tu, Yuanmao
A1 Zeng, Caihong
YR 2024
UL http://jcp.bmj.com/content/77/8/551.abstract
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.