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Splenic Marginal Zone Lymphoma and Concurrent Membranoproliferative Glomerulonephritis With IgMKappa Deposits in a HCV-Seropositive Patient.

Chelioti E, Efthimiou E, Sotiraki M, Papalexandrou A, Tsilivigkou M - Nephrourol Mon (2014)

Bottom Line: The patient presented with hepatosplenomegaly and a uremic state that necessitated dialysis without any clinical signs of systemic disease apart from the chronic infection with hepatitis C virus.The bone marrow biopsy findings were consistent with splenic marginal zone lymphoma, a rare lymphoproliferative disorder with a rare association with MPGN.Our case indicates high diagnostic value of renal biopsy for rare lymphoplasmacytic neoplasms with renal dysfunction as their predominant clinical manifestation.

View Article: PubMed Central - PubMed

Affiliation: Department of Nephrology, General Hospital of Piraeus, Athens, Greece.

ABSTRACT
We report a case of membranoproliferative glomerulonephritis (MPGN) with IgMκ light chain deposits in a patient with chronic hepatitis C infection and simultaneous onset of monoclonal IgMκ gammopathy with concurrent small B-cell lymphoproliferative disease. The patient presented with hepatosplenomegaly and a uremic state that necessitated dialysis without any clinical signs of systemic disease apart from the chronic infection with hepatitis C virus. The diagnostic approach led to a renal biopsy that revealed MPGN with dominant IgMκ deposits and interstitium infiltration by the lymphoid cells. The bone marrow biopsy findings were consistent with splenic marginal zone lymphoma, a rare lymphoproliferative disorder with a rare association with MPGN. Our case indicates high diagnostic value of renal biopsy for rare lymphoplasmacytic neoplasms with renal dysfunction as their predominant clinical manifestation.

No MeSH data available.


Related in: MedlinePlus

A, On light microscopy, lesions ofmembranoproliferative glomerulonephritis (MPGN), increase in lobular appearance of the glomerular tuft, and significant increase in cellularity are seen (HE × 100 magnification in A-B, Interstitium Infiltrated by strongly IgM-positive lymphoid cells (HE × 40 magnification in B).Abbeviation: HE,Hematoxylin, Eosin.
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fig12050: A, On light microscopy, lesions ofmembranoproliferative glomerulonephritis (MPGN), increase in lobular appearance of the glomerular tuft, and significant increase in cellularity are seen (HE × 100 magnification in A-B, Interstitium Infiltrated by strongly IgM-positive lymphoid cells (HE × 40 magnification in B).Abbeviation: HE,Hematoxylin, Eosin.

Mentions: The clinical and laboratory findings on admission necessitated the immediate start of hemodialysis. Subsequent ultrasound studies revealed hepatosplenomegaly, two kidneys of normal size (right kidney, 11.7 cm; and left kidney, 11.5 cm), and parenchymal thickness (right kidney, 10 mm; and left kidney, 9.9 mm). There was no clinical or radiological sign of lymph nodes involvement. Further laboratory testing by immunological assay (ANA, anti-dsDNA, ASMA, AMA, C-ANCA, P-ANCA, and C3 complement levels) had negative results except for serum proteins electrophoresis, which showed a monoclonal gammopathy of IgM. At the same time, urine immunofixation revealed the presence of albumin, IgM, and kappa light chains, type I cryoglobulinemia, and low C4 complement levels. Considering the history of the patient and after excluding and treating all other causes of renal function deterioration, our diagnostic approach pointed towards an HCV-related renal offence in the context of cryoglobulinemia. However, the rapid decline of renal function and the absence of mixed cryoglobulinemia suggested performing the renal biopsy. While the histopathological examination of the specimen was consistent with the initial suspicion, indicating MPGN (Figure 1 A), the immunofluorescence study revealed two unusual findings: dominant monoclonal IgMκ deposits in glomerular basement membrane and infiltration of the interstitium by abundant strongly IgM-positive lymphoid cells (Figure 1 B). The presence of CD20 cell marker was distinctive while CD5 was absent. According to these findings, a bone marrow biopsy was performed. The histopathological examination revealed plasmacytic differentiation of small B cells and the immunophenotyping results were positive for CD20 and negative for CD5. These findings were consistent with SMZL. The patient became hemodialysis dependent and received no treatment for the lymphoproliferative disease and was monitored regularly for possible transformation to a high-differentiated lymphoma.


Splenic Marginal Zone Lymphoma and Concurrent Membranoproliferative Glomerulonephritis With IgMKappa Deposits in a HCV-Seropositive Patient.

Chelioti E, Efthimiou E, Sotiraki M, Papalexandrou A, Tsilivigkou M - Nephrourol Mon (2014)

A, On light microscopy, lesions ofmembranoproliferative glomerulonephritis (MPGN), increase in lobular appearance of the glomerular tuft, and significant increase in cellularity are seen (HE × 100 magnification in A-B, Interstitium Infiltrated by strongly IgM-positive lymphoid cells (HE × 40 magnification in B).Abbeviation: HE,Hematoxylin, Eosin.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4317725&req=5

fig12050: A, On light microscopy, lesions ofmembranoproliferative glomerulonephritis (MPGN), increase in lobular appearance of the glomerular tuft, and significant increase in cellularity are seen (HE × 100 magnification in A-B, Interstitium Infiltrated by strongly IgM-positive lymphoid cells (HE × 40 magnification in B).Abbeviation: HE,Hematoxylin, Eosin.
Mentions: The clinical and laboratory findings on admission necessitated the immediate start of hemodialysis. Subsequent ultrasound studies revealed hepatosplenomegaly, two kidneys of normal size (right kidney, 11.7 cm; and left kidney, 11.5 cm), and parenchymal thickness (right kidney, 10 mm; and left kidney, 9.9 mm). There was no clinical or radiological sign of lymph nodes involvement. Further laboratory testing by immunological assay (ANA, anti-dsDNA, ASMA, AMA, C-ANCA, P-ANCA, and C3 complement levels) had negative results except for serum proteins electrophoresis, which showed a monoclonal gammopathy of IgM. At the same time, urine immunofixation revealed the presence of albumin, IgM, and kappa light chains, type I cryoglobulinemia, and low C4 complement levels. Considering the history of the patient and after excluding and treating all other causes of renal function deterioration, our diagnostic approach pointed towards an HCV-related renal offence in the context of cryoglobulinemia. However, the rapid decline of renal function and the absence of mixed cryoglobulinemia suggested performing the renal biopsy. While the histopathological examination of the specimen was consistent with the initial suspicion, indicating MPGN (Figure 1 A), the immunofluorescence study revealed two unusual findings: dominant monoclonal IgMκ deposits in glomerular basement membrane and infiltration of the interstitium by abundant strongly IgM-positive lymphoid cells (Figure 1 B). The presence of CD20 cell marker was distinctive while CD5 was absent. According to these findings, a bone marrow biopsy was performed. The histopathological examination revealed plasmacytic differentiation of small B cells and the immunophenotyping results were positive for CD20 and negative for CD5. These findings were consistent with SMZL. The patient became hemodialysis dependent and received no treatment for the lymphoproliferative disease and was monitored regularly for possible transformation to a high-differentiated lymphoma.

Bottom Line: The patient presented with hepatosplenomegaly and a uremic state that necessitated dialysis without any clinical signs of systemic disease apart from the chronic infection with hepatitis C virus.The bone marrow biopsy findings were consistent with splenic marginal zone lymphoma, a rare lymphoproliferative disorder with a rare association with MPGN.Our case indicates high diagnostic value of renal biopsy for rare lymphoplasmacytic neoplasms with renal dysfunction as their predominant clinical manifestation.

View Article: PubMed Central - PubMed

Affiliation: Department of Nephrology, General Hospital of Piraeus, Athens, Greece.

ABSTRACT
We report a case of membranoproliferative glomerulonephritis (MPGN) with IgMκ light chain deposits in a patient with chronic hepatitis C infection and simultaneous onset of monoclonal IgMκ gammopathy with concurrent small B-cell lymphoproliferative disease. The patient presented with hepatosplenomegaly and a uremic state that necessitated dialysis without any clinical signs of systemic disease apart from the chronic infection with hepatitis C virus. The diagnostic approach led to a renal biopsy that revealed MPGN with dominant IgMκ deposits and interstitium infiltration by the lymphoid cells. The bone marrow biopsy findings were consistent with splenic marginal zone lymphoma, a rare lymphoproliferative disorder with a rare association with MPGN. Our case indicates high diagnostic value of renal biopsy for rare lymphoplasmacytic neoplasms with renal dysfunction as their predominant clinical manifestation.

No MeSH data available.


Related in: MedlinePlus