Renal Pathology

Renal Amyloidosis

Maria Picken
Loyola Medical School
Chicago IL


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Clinical History A 55-year-old Caucasian female presented with transient peri-orbital and ankle edema and weight gain.

Past medical history:
She has had no recent routine medical care but she states that she has been in overall good health. Except for urinary tract infections in the past, there was no evidence of chronic inflammatory disease. She denies systemic symptoms such as fever, chills, nausea, vomiting, joint pains, rash or redness upon sun exposure. She denies frequency, urgency or pain on urination. She noticed that her urine became foamy but did not notice blood in her urine. She has not traveled, and has no pets or recent insect bites. She had never smoked tobacco and did not drink alcohol.

Medication:
none on admission

Family history:
Northern European ancestry, both parents are deceased. Her mother died in her forties of breast cancer, her father died of lung cancer in his sixties. Her younger sister and two children are alive and well. There is no known family history of kidney disease.

Physical examination revealed:
weight 155 lb, blood pressure of 165/95mm Hg, mild peri-orbital and ankle edema, heart rate of 72 bpm, regular sinus rhythm, no cardiac murmurs.

ROS:
negative except for the above.

Laboratory findings:
Serum creatinine – 2.1 mg/dl, glucose 109 mg/dl

Urinalysis: 4+ proteinuria, 5-10 RBC, no RBC or cellular casts

24-hr urine protein 5.3 gm, serum albumin 2.4 gm/dl, cholesterol 260 mg/dl, triglycerides 240 mg/dl, hemoglobin 10.4 g/dL, moderately decreased creatinine clearance (62.0 mL/min/1.73 m2). C3/C4 – normal, ANA – negative, ANCA – negative, anti-GBM antibody – negative. Liver function tests were normal, Hepatitis B and C, HIV were all negative. Serum immunoelectrophoresis with immunofixation showed monoclonal immunoglobulin (IgG-kappa).

Cardiac ECHO showed mild to moderate left ventricular hypertrophy. US – normal size kidneys without hydronephrosis or stones. Kidney biopsy was performed.

Despite supportive treatment, the patient developed renal failure within 2 years post kidney biopsy. Subsequently, she underwent a combined liver and kidney transplant. Currently, 2 years post-transplant, the patient is well and has normal kidney function.


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Figure 1
Paraffin section, H&E stain.

Figure 2
Paraffin section, PAS stain.

Figure 3
Paraffin section, Congo red stain.

Figure 4
Paraffin section photographed under polarized light, same stain as fig 3.

Figure 5
Paraffin section, immunoperoxidase stain, low magnification.

Figure 6
Paraffin section, immunoperoxidase stain, showing a positive reaction with a single antibody from the panel of antibodies tested. Immunofluorescence stains with a panel of antibodies including IgG, IgA, IgM, kappa and lambda light chain, C3, C1q, fibrinogen, albumin, amyloid A protein and transthyretin were noncontributory - medulla only.