A 51-year-old male patient was admitted for chronic anasarca.
Physical examination showed no other relevant findings such as lymphadenopathy or organomegaly.
Body weight: 90 kg, blood pressure 110/70 mmHg, diuresis preserved.
Laboratory: haematocrit 36%, leukocytes 6,500 mm3, erythrocyte sedimentation rate globular (SGA) 120 mm in 1 h (normal < 20), creatinine 1.4 mg/dl {measured creatinine clearance albumin 128 dl: 69 ml
Light chains with predominance of monoclonal component were identified by electrophoretic uroproteinogram and kappa chains by immunofixation, with negative results in peripheral blood.
Serology of HBV, HCV, HIV, antinuclear antibodies and antineutrophil cytoplasmic antibodies negative.
Normal complement levels.
Criterion < 1%.
Doppler echocardiography: normal.
Renal biopsy revealed diffuse nodular nodular fibromerulopathy with 35% tubular and interstitial pigmentation, with red pigmentation diffuse, the morphology of Wilson de la glm similar to that of la glm
The immunofluorescence was positive for kappa chains and lymphocytes authenticated the existence of light chain deposits of globulin or other fibroids that suggested amyloid, cryogenic, globulin-related disorders.
Bone marrow biopsy: myeloid hyperplasia.
The patient was treated with meprednisone 0.5 mg/kg and plasmapheresis, achieving an increase in creatinine to 2.2 mg/dl.
After ten consecutive sessions, and with albumin as replacement, albumin was increased to 3.1 g/dl, creatinine decreased to 1.5 mg/dl and the monoclonal kappa component urine almost disappeared albumin.
She started treatment with monthly pulses of cyclophosphamide 1 g/iv with six applications.
The patient had an MIS (malnutrition and inflammation score) of 26 (range 0 to 30), which implies severe malnutrition, coupled with increased inflammatory markers, with a previously reported ESR < 0.3 mg).
Although elevated inflammatory markers could be due to the underlying pathology and hypoalbuminemia is mainly attributable to nephrotic syndrome, creatinine clearance due to near-normal haemodialysis has started secondary to nephrotic syndrome.
A hypercaloric diet was prescribed (35 calories/kg/day).
His clinical status improved, with a loss of 23 kg of body weight, without edema and a significant improvement in nutritional status.
Creatinine decreased to 1.6 mg/dl (measured creatinine clearance: 52 ml/min).
After the third monthly infusion of cyclophosphamide, the monoclonal component of the urinary kappa chain disappeared completely without presenting a monoclonal component in blood.
Enalapril, furosemide and low doses of indomethacin were used in order to achieve chemical nephrectomy and reduce massive proteinuria with acceptable results.
The latter gradually decreased to an average level of 7 g/day in 1 liter of daily diuresis and albuminemia increased to 2.6 g/dl. The patient continued on hemodialysis for a period of six months, and continued receiving 10 mg/dl.
