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Kidney Week

Abstract: SA-PO0917

IgG Heavy-Chain Deposition Disease: An Overlooked Cause of ESRD

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Naeem, Shanzae, Piedmont Macon Medical, Macon, Georgia, United States
  • Kasumu, Khadijat, Piedmont Macon Medical, Macon, Georgia, United States
  • Memon, Sidra, Piedmont Macon Medical, Macon, Georgia, United States
  • Zaggout, Ihab Z., Piedmont Macon Medical, Macon, Georgia, United States
Introduction

Heavy Chain Deposition Disease (HCDD) is a rare type of Monoclonal Immunoglobulin Deposition Disease (MIDD) in which structurally altered monoclonal immunoglobulin heavy chains are deposited in multiple organs, including kidneys - without light chain deposition. Classified under Monoclonal Gammopathy of Renal Significance, HCDD presents as nephrotic-range proteinuria, hypertension & rapid progression to ESRD.

Case Description

A 55 y/o man with T-cell lymphoma s/p allogeneic stem cell transplant, with worsening anasarca, renal dysfunction & sob.

Labs: Cr 6.95 mg/dL, UPCR 7.7, Albumin 2.7 g/dL
Immunofixation: Faint free lambda chains. SPEP lacked a definitive M-spike
Renal biopsy: Nodular glomerulosclerosis with linear deposition of IgG heavy chains on immunofluorescence
Bone marrow biopsy: 5–10% lambda-restricted plasma cells
FISH: IGH rearrangement & gain of chromosome 1q

Urgent dialysis, chemotherapy, & dexamethasone were initiated to target the plasma cell clone, but he remained dialysis-dependent, reflecting the fulminant course of HCDD.

Discussion

HCDD can progress to ESRD within 3 months, requiring kidney biopsy due to low paraprotein burden. Chromosomal abnormalities 1q gain/IGH rearrangement predict poor outcomes despite minimal clonal burden, supporting an underlying monoclonal disorder. Patients are at increased risk for amyloidosis & multiple myeloma, necessitating lifelong follow-up.

FISH panel

Flow cytometry

Digital Object Identifier (DOI)