A case of heavy-chain deposition disease with good long-term renal survival and a literature review

Monoclonal immunoglobulin deposition disease (MIDD) is characterized by the deposition of nonamyloid monoclonal immunoglobulin and its free fragment light chain and/or heavy chain in systemic tissues and organs, and the kidney is most vulnerable organs. MIDD can be divided into three types: light-ch...

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Published in:	BMC nephrology Vol. 25; no. 1; pp. 312 - 8
Main Authors:	Cai, Xiaoqing, Zou, Wenli, Chen, Huaihuai, Xing, Chaonian, Yu, Xuguang
Format:	Journal Article
Language:	English
Published:	England BioMed Central Ltd 19-09-2024 BioMed Central BMC
Subjects:	Abdomen Acute renal failure Adult Anemia Antibodies Basement membranes Biopsy Blood pressure Bortezomib Bortezomib - therapeutic use Case report Case reports Case studies Catheters Chemotherapy Creatinine Dexamethasone Edema Health aspects Heavy Chain Disease - complications Heavy Chain Disease - diagnosis Heavy-chain deposition disease Hematuria Hemodialysis Hemoglobin Humans Hypertension Immunofluorescence Immunoglobulin G Immunoglobulins Kidney - pathology Kidneys Light Light microscopy Literature reviews Male Medical prognosis Medical research Medicine, Experimental Microscopy Monoclonal immunoglobulin deposition disease Patients Proteins Proteinuria Renal function Skin laxity Survival Thalidomide Tubular basement membrane Urine China Case report Skin laxity Heavy-chain deposition disease Monoclonal immunoglobulin deposition disease Acute renal failure
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Summary:	Monoclonal immunoglobulin deposition disease (MIDD) is characterized by the deposition of nonamyloid monoclonal immunoglobulin and its free fragment light chain and/or heavy chain in systemic tissues and organs, and the kidney is most vulnerable organs. MIDD can be divided into three types: light-chain deposition disease (LCDD), light and heavy chain deposition disease (LHCDD), and heavy-chain deposition disease (HCDD), of which LHCDD and HCDD are rarer (Bridoux et al. in Kidney Int 2015;87:698-711; Preud'homme et al. in Kidney Int 1994;46:965-72). Poor outcome in most HCDD, but in this paper, we will report a case of HCDD with good long-term renal survival and review the literature for reference. A 32-year-old man presented to our department with skin laxity and nephritic syndrome, accompanied by an significant increase of serum creatinine and received short-term hemodialysis treatment. Both the blood and urine free light chain ratio increased significantly. Renal biopsy showed mesangial nodular glomerulosclerosis on light microscopy, and immunofluorescence staining showed positivity for γ-heavy chain (HC), with negative light chain (LC) staining; the diagnosis was considered HCDD. After six courses of bortezomib combined with dexamethasone chemotherapy and thalidomide 100 mg/day, the renal function gradually recovered, while also with proteinuria and hematuria significantly improved. The blood and urine free light chain ratio decreased to normal. Until now, the patient has been followed for four years, and long-term renal survival has been observed. Herein, we report a case presenting with proteinuria, hematuria, renal impairment, and skin laxity, and a renal biopsy showed linear IgG deposition in the glomerular basement membranes and tubular basement membrane. However, they ultimately proved to have HCDD. Bortezomib combined with dexamethasone, and oral thalidomide led to a good long-term renal survival. We also provide a review of currently available literature, and this is the first large-scale review summarizing the characteristics of HCDD up to date.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Review-5 content type line 23 ObjectType-Case Study-4 ObjectType-Feature-2 ObjectType-Report-3
ISSN:	1471-2369 1471-2369
DOI:	10.1186/s12882-024-03755-z