Cast nephropathy in mu heavy chain disease.
The occurrence of kidney diseases was very rarely reported in heavy chain diseases (HCD). At variance with gamma and alpha HCD in which there is no free light chain secretion, about two-thirds of mu HCD patients have urinary Bence Jones (BJ) proteins. We report on a 66 year-old man affected with typical mu HCD who developed renal failure after a 3-year follow-up. He had presented with chronic lymphocytic leukemia with bone marrow vacuolated plasma cells, serum mu HCD protein and serum and urine BJ protein. After an apparent hematological remission following fludarabine therapy, anemia and blood hyperlymphocytosis recurred together with microscopic hematuria, proteinuria and increased creatininemia. Kidney biopsy showed numerous tubular eosinophilic casts which stained for kappa chain determinants by immunofluorescence and an interstitial infiltration by lymphocytes and plasma cells. The hematological and renal condition improved after reinitiation of chemotherapy. This appears to be the first documented report of a light chain-dependent visceral complication in HCD.
Preud'homme
J L
JL
Laboratory of Immunology and Immunopathology (CNRS URA 1172 Immunology and Molecular Interactions, Poitiers, France.
Bauwens
M
M
Dumont
G
G
Goujon
J M
JM
Dreyfus
B
B
Touchard
G
G
D000368:Aged; D001536:Bence Jones Protein / Q000378:metabolism; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D005500:Follow-Up Studies; D006362:Heavy Chain Disease / Q000150:complications* / Q000276:immunology / Q000378:metabolism; D006801:Humans; D007122:Immunoelectrophoresis; D007148:Immunoglobulin mu-Chains / Q000097:blood / Q000652:urine; D007674:Kidney Diseases / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications / Q000378:metabolism / Q000473:pathology; D008297:Male
D007148:Immunoglobulin mu-Chains; D001536:Bence Jones Protein