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Waldenström’s Macroglobulinemia

A 57-year-old white male presents with fatigue, mild proteinuria, and a creatinine of 1.5 mg/dL. He was diagnosed with lymphoplasmacytic lymphoma 6 months ago, but refused treatment. Figures 1 & 2 show prominent pseudothrombi and a membranoproliferative pattern of glomerular injury. Image 3 shows moderate interstitial fibrosis. Image 4, 5, 6, & 7 show IgG, IgM, kappa, and lambda, respectively. This is a case of cryoglobulinemic glomerulonephritis secondary to lymphoplasmacytic lymphoma (Waldenström's macroglobulinemia). Waldenström's macroglobulinemia is a rare B-cell lymphoma characterized by lymphoplasmacytic cell infiltration in the bone marrow and other organs and the presence of a monoclonal immunoglobulin M...

Light Chain Cast Nephropathy

A 75-year-old female is found to have a creatinine of 4.0 mg/dL on a routine laboratory examination. At the hospital, a CT scan shows lytic lesions within the skull. A SPEP shows an IgG kappa monoclonal protein. A bone marrow biopsy shows 5% plasma cells. A kidney biopsy is requested to look for monoclonal gammopathy of renal significance. Figure 1 shows a normal glomerulus. Figure 2 shows severe interstitial fibrosis. Figures 3 & 4 show abnormal, PAS negative "stiffened" casts with cellular reaction and fracture. Figures 5 & 6 show kappa and lambda immunofluorescence, respectively. This is a case of...

Disease Week: C3 Glomerulonephritis

C3 glomerulonephritis is a recently described entity which is due to dysregulation in the alternative complement pathway. Patients typically present with hematuria and/or proteinuria in the face of persistently low serum levels of C3. The annual incidence of biopsy-proven disease is 1 to 2 per million with both sexes affected equally. The median age of diagnosis is 21 years of age, but there is a second spike after the age of 50 due to paraprotein-associated disease. The most common glomerular disease pattern is a membranoproliferative pattern. The hallmark of the disease is dominant C3 staining on immunofluorescence which is defined...

Membranous Nephropathy After Transplantation

The biopsy shows a recurrent membranous nephropathy within a transplant. Figure 1 shows "spikes" and "holes." Figure 2 shows mild interstitial fibrosis. Figures 3, 4, 5, and 6 shows IgG, kappa, lambda, and PLA2r, respectively. Figure 7 shows subepithelial and intramembranous deposits. Membranous nephropathy can arise in the graft from recurrence of the original disease, a de novo disease, and rarely as a donor-derived disease. Recurrence of the original disease can be seen as early as one week post transplant. Most cases of recurrent membranous nephropathy occur within the first few months following transplantation, whereas de novo membranous nephropathy usually...

Crescentic IgA nephropathy

The patient is a 17-year-old white female who presents with 2.85 grams/24 hr proteinuria, microscopic hematuria, and a creatinine of 3.2 mg/dl. She was in her normal state of health and was incidentally found to have abnormal lab values and urinalysis at a routine sport's physical. She reports that she had noticed a little more fatigue the last few months, but had blamed this on being busy at her job after school. Figure 1 shows focal fibrinoid necrosis. Figure 2 shows moderate tubular atrophy and interstitial fibrosis. Figures 3 & 4 show a segmental cellular crescent. Figure 5 shows several...

Scleroderma

The patient is a 32-year-old African American female who presents with hypertensive urgency, positive ANA, positive anti-RNA polymerase III antibody, schistocytes on the peripheral smear, forgetfulness, and a creatinine of 2. On physical examination, the skin is tight and thickened along the face ("masked face"), back, and proximal arms. Urinalysis shows 250 mg of proteinuria and no active urine sediment. Serologies for c-ANCA, p-ANCA, dsDNA, and anti-GBM are negative. The patient has scleroderma renal crisis. Figure 1 shows an ischemic glomerulus. Figure 2 shows moderate interstitial fibrosis. From low power, the intimal edema and intimal proliferation within the arteries is...

Cryoglobulinemic Glomerulonephritis

The patient is a 60-year-old male who presents with proteinuria, hematuria, and a creatinine of 2.8 mg/dL. He was in his normal state of health until 2 weeks ago when he noticed he was having trouble putting on his shoes. Serologies show low complements and an albumin of 2.3 g/dL. Serologies for ANA, c-ANCA, p-ANCA, hepatitis B, and hepatitis C are negative. No monoclonal spike is found on serum electrophoresis. Figure 1 shows accentuation of lobulation with both mesangial and endocapillary hypercellularity. This is a membranoproliferative pattern. Figure 2 shows a small, hyaline thrombus. Figure 3 shows moderate interstitial fibrosis....

VEGF Inhibitor

The patient is a 70-year-old white female who presents with 4 gm/24 hr proteinuria and a creatinine of 1.2 mg/dl. She has colon cancer with metastases to the liver, treated with chemotherapy and Avastin. The patient has recently had anemia, thrombocytopenia, ascites, and a urinary tract infection treated with nitrofurantoin. Her blood pressure has been under good control at 120/80. Figure 1 shows acellular closure of the capillary lumens and a "cotton-candy" appearance of the mesangium. Figures 2 and 3 shows capillary microaneurysms and segmental capillary hyalinosis. Figure 3 additionally shows double contours. Figure 4 shows moderate interstitial fibrosis. Figure...

Hemosiderosis

An 80-year-old white male presents with a creatinine of 4.2 (baseline 1.1) on routine lab examination. His medical history is significant for coronary artery disease, aortic valve replacement, hypertension, and peripheral artery disease. He reports no episodes of dehydration and reports that he has been in his normal state of health. Figure 1 shows a normal glomerulus. Figure 2 shows a mild interstitial fibrosis. Figure 3 shows brown spherules within the tubular epithelial cells. Figure 4 confirms the presence of iron within these brown spherules. This is a case of renal hemosiderosis. The presence of iron deposits speaks towards intravascular...

AL Amyloidosis

A 70 year-old female presents with nephrotic range proteinuria and a creatinine of 1.2. She was in her normal level of health until about 6 months ago when she noticed foamy urine and swelling in her ankles. She has lost 30 lbs unintentionally during the last few months. Her cardiologist said that her proteinuria does not appear to be related to the heart. A kidney biopsy is performed. The glomerulus in figure 1 is distorted and has a “washed-out” or pale appearance on PAS stain. A silver stain shows material which is non-argyrophilic (figure 2). A Congo red stain shows...