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Nephrotic syndrome and kidney failure due to immunocomplex-mediated renal damage in a patient with Waldenström's Macroglobulinemia: a case report.

Castro H, Valenzuela R, Ruiz P, Lenz O, Monrroy M - Cases J (2008)

Bottom Line: Nephrotic range proteinuria is a very unusual manifestation of renal injury in these patients and when present it is due to amyloid light-chain deposition most of the times.A 60-year-old male patient presented to the hospital with nephrotic syndrome, renal insufficiency, hypertension and lymphadenopathy.The investigations led to the diagnosis of Waldenström's Macroglobulinemia with associated nephrotic syndrome and chronic kidney disease due to an unusual form of hypocomplementemic glomerulopathy with histopathological features similar to those seen in mesangiocapillary glomerulonephritis type III, but lacking proliferative changes.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Medicine, Division of General Internal Medicine, University of Miami/Jackson Memorial Medical Center, Miami, Florida, USA. hcastro2@med.miami.edu.

ABSTRACT

Introduction: Unlike the quite frequent renal involvement seen in cases of Multiple Myeloma, the kidney is hardly ever compromised in patients with Waldenström's Macroglobulinemia. Nephrotic range proteinuria is a very unusual manifestation of renal injury in these patients and when present it is due to amyloid light-chain deposition most of the times.

Case presentation: A 60-year-old male patient presented to the hospital with nephrotic syndrome, renal insufficiency, hypertension and lymphadenopathy. The investigations led to the diagnosis of Waldenström's Macroglobulinemia with associated nephrotic syndrome and chronic kidney disease due to an unusual form of hypocomplementemic glomerulopathy with histopathological features similar to those seen in mesangiocapillary glomerulonephritis type III, but lacking proliferative changes.

Conclusion: We present an unusual case of immunologically-mediated renal damage in a patient with Waldenström's Macroglobulinemia, leading to non-amyloid nephrotic syndrome and chronic renal insufficiency.

No MeSH data available.


Related in: MedlinePlus

Light Microscopy. There is marked, global, homogeneous, eosinophilic thickening of the glomerular basement membrane with segmental accentuation. Homogeneous, eosinophilic globules are seen in the lumen of occasional capillary loops. The capillary lumina appear reduced in diameter but no inflammatory or proliferative changes are observed. The periglomerular interstitial space shows lymphocytic infiltration. Focal interstitial deposition of homogeneous eosinophilic material is present in the right upper corner of the picture (H&E × 400).
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Figure 1: Light Microscopy. There is marked, global, homogeneous, eosinophilic thickening of the glomerular basement membrane with segmental accentuation. Homogeneous, eosinophilic globules are seen in the lumen of occasional capillary loops. The capillary lumina appear reduced in diameter but no inflammatory or proliferative changes are observed. The periglomerular interstitial space shows lymphocytic infiltration. Focal interstitial deposition of homogeneous eosinophilic material is present in the right upper corner of the picture (H&E × 400).

Mentions: On light microscopy 11 of 18 glomeruli were globally sclerosed and more than 50% of the specimen showed interstitial fibrosis. Marked, global and homogeneous thickening of the glomerular basement membrane, with segmental accentuation due to a strongly PAS positive eosinophilic material was seen and it was Congo red negative (Figure 1). There were no proliferative or inflammatory changes. Focal nodular collections of the same material were present in the tubulointerstitial areas and lymphocytic infiltration was present in the periglomerular interstitial space. Immunofluorescence showed a diffuse global, granular to homogenous deposition of IgG (3+), IgA (3+), IgM (2+), C3 (3+), C4 (3+), C1q (3+), albumin (3+), kappa (3+), lambda (3+) and fibrinogen (1+) involving principally the basement membranes (Figure 2). Focal homogenous deposition of the same immunoreactants with similar fluorescence intensity was seen in the tubulointerstitial areas. No vascular fluorescence was apparent. Finally, electron microscopy showed extensive glomerular sclerosis and basement membrane thickening with significant reduction of the capillary lumen in the remaining glomeruli (Figure 3). There were numerous electron-dense deposits present in both mesangium and glomerular membranes, but principally in the latter. Some of them clearly showed a subendothelial or subepithelial location (Figure 4). The deposits did not exhibit an organized substructure (microtubular or fibrillary). No fibrin thrombi were found. The interstitial areas were expanded due to the presence of mononuclear inflammatory cells (monocytes and plasma cells), increased collagen, and electron dense deposits similar to those identified in glomeruli.


Nephrotic syndrome and kidney failure due to immunocomplex-mediated renal damage in a patient with Waldenström's Macroglobulinemia: a case report.

Castro H, Valenzuela R, Ruiz P, Lenz O, Monrroy M - Cases J (2008)

Light Microscopy. There is marked, global, homogeneous, eosinophilic thickening of the glomerular basement membrane with segmental accentuation. Homogeneous, eosinophilic globules are seen in the lumen of occasional capillary loops. The capillary lumina appear reduced in diameter but no inflammatory or proliferative changes are observed. The periglomerular interstitial space shows lymphocytic infiltration. Focal interstitial deposition of homogeneous eosinophilic material is present in the right upper corner of the picture (H&E × 400).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2600791&req=5

Figure 1: Light Microscopy. There is marked, global, homogeneous, eosinophilic thickening of the glomerular basement membrane with segmental accentuation. Homogeneous, eosinophilic globules are seen in the lumen of occasional capillary loops. The capillary lumina appear reduced in diameter but no inflammatory or proliferative changes are observed. The periglomerular interstitial space shows lymphocytic infiltration. Focal interstitial deposition of homogeneous eosinophilic material is present in the right upper corner of the picture (H&E × 400).
Mentions: On light microscopy 11 of 18 glomeruli were globally sclerosed and more than 50% of the specimen showed interstitial fibrosis. Marked, global and homogeneous thickening of the glomerular basement membrane, with segmental accentuation due to a strongly PAS positive eosinophilic material was seen and it was Congo red negative (Figure 1). There were no proliferative or inflammatory changes. Focal nodular collections of the same material were present in the tubulointerstitial areas and lymphocytic infiltration was present in the periglomerular interstitial space. Immunofluorescence showed a diffuse global, granular to homogenous deposition of IgG (3+), IgA (3+), IgM (2+), C3 (3+), C4 (3+), C1q (3+), albumin (3+), kappa (3+), lambda (3+) and fibrinogen (1+) involving principally the basement membranes (Figure 2). Focal homogenous deposition of the same immunoreactants with similar fluorescence intensity was seen in the tubulointerstitial areas. No vascular fluorescence was apparent. Finally, electron microscopy showed extensive glomerular sclerosis and basement membrane thickening with significant reduction of the capillary lumen in the remaining glomeruli (Figure 3). There were numerous electron-dense deposits present in both mesangium and glomerular membranes, but principally in the latter. Some of them clearly showed a subendothelial or subepithelial location (Figure 4). The deposits did not exhibit an organized substructure (microtubular or fibrillary). No fibrin thrombi were found. The interstitial areas were expanded due to the presence of mononuclear inflammatory cells (monocytes and plasma cells), increased collagen, and electron dense deposits similar to those identified in glomeruli.

Bottom Line: Nephrotic range proteinuria is a very unusual manifestation of renal injury in these patients and when present it is due to amyloid light-chain deposition most of the times.A 60-year-old male patient presented to the hospital with nephrotic syndrome, renal insufficiency, hypertension and lymphadenopathy.The investigations led to the diagnosis of Waldenström's Macroglobulinemia with associated nephrotic syndrome and chronic kidney disease due to an unusual form of hypocomplementemic glomerulopathy with histopathological features similar to those seen in mesangiocapillary glomerulonephritis type III, but lacking proliferative changes.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Medicine, Division of General Internal Medicine, University of Miami/Jackson Memorial Medical Center, Miami, Florida, USA. hcastro2@med.miami.edu.

ABSTRACT

Introduction: Unlike the quite frequent renal involvement seen in cases of Multiple Myeloma, the kidney is hardly ever compromised in patients with Waldenström's Macroglobulinemia. Nephrotic range proteinuria is a very unusual manifestation of renal injury in these patients and when present it is due to amyloid light-chain deposition most of the times.

Case presentation: A 60-year-old male patient presented to the hospital with nephrotic syndrome, renal insufficiency, hypertension and lymphadenopathy. The investigations led to the diagnosis of Waldenström's Macroglobulinemia with associated nephrotic syndrome and chronic kidney disease due to an unusual form of hypocomplementemic glomerulopathy with histopathological features similar to those seen in mesangiocapillary glomerulonephritis type III, but lacking proliferative changes.

Conclusion: We present an unusual case of immunologically-mediated renal damage in a patient with Waldenström's Macroglobulinemia, leading to non-amyloid nephrotic syndrome and chronic renal insufficiency.

No MeSH data available.


Related in: MedlinePlus