[Intracytoplasmic crystals and Fanconi syndrome in a patient with IgA kappa myeloma].
A 45 year old man with IgA-Kappa myeloma had adult Fanconi Syndrome. Examination of renal biopsy revealed lesions in proximal tubules without glomerular lesions and without intratubular casts. By electron microscopy cytoplasmic crystalline inclusions were observed in renal proximal tubular epithelium. Increased plasma cells (28%) in bone marrow aspiration also contained crystalline inclusions. The treatment of myeloma produced partial remission of proliferative disease and Fanconi syndrome. We discuss the pathogenesis of Fanconi syndrome induced by light chains as well as the composition of crystalline deposits and the effects of treatment on Fanconi Syndrome.
Martín Reyes
G
G
Servicio de Nefrología, Hospital Regional Universitario Carlos Haya, Avda. Carlos Haya, 82, 29010 Málaga.
García González
I
I
Alférez
M J
MJ
Martínez González
J M
JM
Frutos
M A
MA
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology; D002330:Carmustine / Q000008:administration & dosage; D003460:Crystallization; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005198:Fanconi Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000652:urine; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis*; D007135:Immunoglobulin Variable Region / Q000032:analysis; D007145:Immunoglobulin kappa-Chains / Q000032:analysis*; D002479:Inclusion Bodies / Q000737:chemistry / Q000648:ultrastructure*; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000652:urine; D007687:Kidney Tubules, Proximal / Q000648:ultrastructure*; D008247:Lysosomes / Q000648:ultrastructure; D008297:Male; D008558:Melphalan / Q000008:administration & dosage; D008854:Microscopy, Electron; D008875:Middle Aged; D009101:Multiple Myeloma / Q000737:chemistry / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D009194:Myeloma Proteins / Q000032:analysis*; D010950:Plasma Cells / Q000648:ultrastructure; D011241:Prednisone / Q000008:administration & dosage; D014617:Vacuoles / Q000648:ultrastructure; D014750:Vincristine / Q000008:administration & dosage
D007070:Immunoglobulin A; D007135:Immunoglobulin Variable Region; D007145:Immunoglobulin kappa-Chains; D009194:Myeloma Proteins; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D008558:Melphalan; D002330:Carmustine; D011241:Prednisone