A 59-year-old woman with no morbid history.
Twenty-eight months prior to transplantation, she presented edema and foamy urine, with nephrotic syndrome with proteinuria up to 9 g/day and hypoalbuminemia 1.9 g/dL.
Creatinine clearance and blood pressure were normal.
The aetiological study included immunological laboratory tests (antinuclear antibodies, anti-DNA antibodies, antineutrophil cytoplasmic antibodies, viral antibodies (human protein), complemented hepatitis B virus surface antibodies, hepatitis C virus antibodies, and hepatitis C virus
The search for a solid mass was negative, including mammography, chest X-ray, abdominal and pelvic ultrasound, upper digestive endoscopy and neoplasia.
A renal biopsy was performed 24 months prior to transplantation and revealed amyloidosis.
Congo Red staining and Congo Red staining with potassium permanganate were both very tenuously positive in glomeruli and blood vessels.
Electron microscopy revealed pedicelar disappearance parcel and dense deposits of amyloid material in the mesangial zone and in some free capillary loops, with an average thickness of 100 stifle, with fibro.
A subsequent study with serum and urinary immunofixation was positive for monoclonal IgA-lambda.
The quantification of serum immunoglobulins revealed IgA 1000 mg/dL, IgG 852 mg/dL and IgM 93 mg/dL.
Myelogram and bone marrow flow cytometry ruled out multiple myeloma.
She was treated with oral melphalan and prednisone for 6 months. Nephrotic proteinuria and positive serum immunofixation persisted.
Subsequently, only nonspecific therapy was maintained, including enalapril, amiodarone and atorvastatin.
Three months before, the presence of monoclonal protein and nephrotic syndrome with normal glomerular filtration syndrome was maintained.
A transthoracic echocardiogram and an isotopic ventriculography were normal.
In January 2004, medullary conditioning was performed with granulocytic colony stimulating factor for five days, and peripheral blood leukocyte apheresis was performed on 2.6 x 106 hematopoietic stem cells.
She received intravenous melphalan 200 mg/m2 body surface area (day 0) and a single infusion of troples cells was performed the following day.
From day 2 onward she developed oliguric ARF, with excreted fraction of sodium 2%, uricosuria/creatinine index < 1.0 at urinary pH 7.0, normal renal doppler ultrasound, creatinine clearance 20%.
Deterioration of renal function was accompanied by evidence of cellular lysis (lactic-dehydrogenase 6710 U/L and a subsequent decrease in blood lactate to 9.2 mg/dL), uricemia to 13,2 days
Table 1 shows the evolution of some representative laboratory parameters.
The patient required granulocytic colony factor between days 2 and 16, renal replacement therapy with stimulant medication daily intermittent venovenous between days 5 to 14.
On day 13, diuresis occurred and the patient was discharged on day 23 post-melaphalan, with creatininemia 1.2 mg/dL and white blood cell count 3000/mm3.
1.
Clinical follow-up after one year revealed excellent general status, normal glomerular filtration rate, proteinuria <1 g/day, and negative serum immunofixation.
