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Published on: December 28, 2015
[A case report of light and heavy chain deposition disease (IgG2 lambda)]
Y Akashi1, N Yoshizawa, S Oshima
1Second Department of Internal Medicine, National defense Medical College, Saitama, Japan.
Insights
This study details a 73-year-old male diagnosed with plasma cell dyscrasia presenting with kidney issues. The findings highlight IgG lambda light chain deposition in the kidneys, suggesting a link between this condition and renal disease.
Area of Science:
- Nephrology
- Hematology
- Immunology
Background:
- A 73-year-old male presented with hypertension, proteinuria, and edema, with a history of interstitial pneumonia.
- Laboratory findings revealed renal dysfunction, anemia, and elevated IgG2 levels, alongside M-protein and Bence Jones protein (lambda type).
Observation:
- Renal biopsy showed mesangial proliferation, nodular glomerulonephritis, and deposition of PAS-positive, Congo red-negative material.
- Immunofluorescence confirmed IgG and lambda light chain deposition along glomerular and tubular basement membranes and in the mesangium.
- Electron microscopy revealed electron-dense deposits and microfibrillar structures (14 nm) in the mesangium.
Findings:
- The patient was diagnosed with plasma cell dyscrasia.
- Renal pathology demonstrated features consistent with immune complex deposition, specifically IgG lambda light chains.
- The presence of IgG2 and lambda light chain restriction in both serum and kidney tissue suggests a monoclonal gammopathy-related nephropathy.
Implications:
- This case underscores the importance of investigating plasma cell dyscrasias in patients with unexplained renal abnormalities.
- Understanding the specific immunoglobulin deposition patterns can aid in diagnosing and potentially managing such renal complications.
- Further research into the role of IgG2 and lambda light chains in kidney disease is warranted.
Abstract:
A 73-year-old male was admitted to the renal division of our hospital because of hypertension, proteinuria and bilateral pretibial edema. Eight years previously, he was diagnosed as being afflicted with interstitial pneumonia on the basis of a chest X-ray examination. Laboratory tests conducted during the current admission showed normocytic normochromic anemia, renal dysfunction and mild proteinuria. Total IgG was normal, but a high proportion of IgG2 was observed. M-protein in the serum was positive for both IgG lambda and Bence Jones protein (lambda type). A bone marrow biopsy showed the proportion of plasma cells to be 10.6%, but atypical cells were not found. We diagnosed the patient's condition as plasma cell dyscrasia. Light microscopy examination of a renal biopsy specimen showed moderate mesangial proliferation with a deposition of PAS-positive and Congo red negative materials in the mesangial area: nodular gomerulonephritis was seen in some glomeruli. Immunofluorescence revealed IgG and lambda light chains, strong linear staining along the glomerular basement membrane and tubular basement membrane and positivity in the mesangial area. Results of staining for IgA, IgM, fibrinogen and C3 were weakly positive in the mesangium area, while those for C4, Clq and free kappa were negative. Positive staining of IgG2 was seen by immunoperoxidase study, but the tissue was negative for IgG1, IgG3, IgG4. Electron microscopy demonstrated a dense granular deposition in the mesangial, subendothelial and peritubular area and a microfibrillar structure in the mesangial area. The diameter of the microfibrillar structure was 14 nm on the average.(ABSTRACT TRUNCATED AT 250 WORDS)
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