Clinicopathological Characteristics of Light and Heavy Chain Deposition Disease: A Case Series

Yujie Wang; Dacheng Chen; Ruimin Hu; Yuan Zhang; Dandan Liang; Feng Xu; Feng Liu; Xiaodong Zhu; Yao Lin; Xue Yang; Xumeng Liu; Guolan Xing; Shaoshan Liang; Caihong Zeng

doi:10.1053/j.ajkd.2024.03.021

Clinicopathological Characteristics of Light and Heavy Chain Deposition Disease: A Case Series

Am J Kidney Dis. 2024 May 13:S0272-6386(24)00757-1. doi: 10.1053/j.ajkd.2024.03.021. Online ahead of print.

Authors

Yujie Wang¹, Dacheng Chen¹, Ruimin Hu², Yuan Zhang³, Dandan Liang¹, Feng Xu¹, Feng Liu¹, Xiaodong Zhu¹, Yao Lin⁴, Xue Yang¹, Xumeng Liu¹, Guolan Xing⁵, Shaoshan Liang¹, Caihong Zeng⁶

Affiliations

¹ National Clinical Research Center for Kidney Diseases, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, China.
² Department of Nephrology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China.
³ Department of Nephrology, Affiliated Hospital of Nantong University, Nantong, China.
⁴ National Clinical Research Center for Kidney Diseases, Jinling Hospital, Nanjing Medical University, Nanjing, China.
⁵ Department of Nephrology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China. Electronic address: xgl@zzu.edu.cn.
⁶ National Clinical Research Center for Kidney Diseases, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, China. Electronic address: zengcaihong@nju.edu.cn.

PMID: 38750878
DOI: 10.1053/j.ajkd.2024.03.021

Abstract

Rationale & objective: Light and heavy chain deposition disease (LHCDD) is a rare form of monoclonal immunoglobulin deposition disease (MIDD), and limited clinical data are available characterizing this condition. We described the clinicopathological characteristics and outcomes of LHCDD.

Study design: Case series.

Setting & participants: 13 patients with biopsy-proven LHCDD, diagnosed between January 2008 to December 2022, at two Chinese medical centers.

Findings: Among the 13 patients described, 6 were men and 7 were women, with a mean age of 52.6 ± 8.0 years. Patients presented with hypertension (76.9%), anemia (84.6%), elevated serum creatinine (84.6%, median serum creatinine 1.7 mg/dL), proteinuria (100%, average urine protein 3.0g/24h), nephrotic syndrome (30.8%) and microscopic hematuria (76.9%). Serum immunofixation electrophoresis showed monoclonal immunoglobulin for 11 (84.6%) patients. Serum free light chain (FLC) ratios were abnormal in 11 (84.6%) patients, and heavy/light chain (HLC) ratios were abnormal in 9 of 10 (90%) patients with available data. Five patients were diagnosed with multiple myeloma. A histological diagnosis of nodular mesangial sclerosis was made in 10 (76.9%) patients. Immunofluorescence demonstrated deposits of IgG subclass (γ-κ/γ-λ:4/3) in 7 patients, and IgA (α-κ/α-λ:2/3) in 5 patients. Six patients underwent IgG subclass staining (γ1/γ2/γ3:3/2/1). The deposits of IgD-κ were confirmed by mass spectrometry in 1 patient. Among 12 patients for whom data were available over a median of 26.5 months, 11 received chemotherapy, and 1 received conservative treatment. One patient died. Three (25%) patients progressed to kidney failure. Among the 9 patients evaluable for hematological and kidney disease progression, five (56%) had a hematologic response and one (11%) achieved improvement in kidney disease.

Limitations: Retrospective descriptive study, limited number of patients, UPEP or UIFE missing for most patients.

Conclusions: In this case series of LHCDD, light and heavy chain deposition in kidney tissues were most frequent with monoclonal IgG1-κ. Among patients with evaluable data, more than half had hematologic response but a kidney response was uncommon.

Keywords: case series; heavy/light chain assay; light and heavy chain deposition disease; monoclonal gammopathy of renal significance; monoclonal immunoglobulin deposition disease; outcome.