ABSTRACT
A 59-year-old male was diagnosed with nephrotic syndrome secondary to light-chain deposition disease. There was no other evidence of a B cell clonal disorder or amyloidosis; circulating free light chains were identified using a new immunoassay (Freelite) and used to monitor disease progression. Improvement in renal function and proteinuria following VAMP chemotherapy correlated with a reduction in circulating light-chain levels. This case demonstrates a new tool in monitoring light-chain deposition disease in the kidney.
Subject(s)
Hypergammaglobulinemia/diagnosis , Immunoassay/methods , Immunoglobulin kappa-Chains/blood , Immunoglobulin lambda-Chains/blood , Nephrotic Syndrome/etiology , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Azathioprine/administration & dosage , Basement Membrane/metabolism , Basement Membrane/ultrastructure , Disease Progression , Humans , Hypergammaglobulinemia/blood , Hypergammaglobulinemia/drug therapy , Hypergammaglobulinemia/pathology , Kidney/pathology , Kidney Tubules/metabolism , Kidney Tubules/ultrastructure , Male , Methotrexate/administration & dosage , Middle Aged , Prednisolone/administration & dosage , Vincristine/administration & dosageABSTRACT
The optimal dose of interferon-alfa (IFN) for chronic myeloid leukemia (CML) is unknown. Retrospective analyses suggest that low doses are as effective as high doses, with less toxicity and fewer patients abandoning the drug. The Dutch Hemato-Oncology Association (HOVON) and British Medical Research Council (MRC) cooperative groups jointly performed randomized trials in newly diagnosed CML patients, comparing high-dose IFN (5 MIU/m(2) daily) with low-dose (3 MIU, 5 times a week). Both arms allowed additional hydroxyurea to keep the white blood cell count lower than 5 x 10(9)/L. Quality of life data were collected in a subset of patients. Between 1993 and 2001, 407 patients were randomized. At a median follow-up of 53 months, there were no significant differences in overall survival (odds ratio = 1.09, 95% confidence interval, 0.81-1.46), progression-free survival, and complete hematologic or major cytogenetic responses. Fewer patients in the low-dose group abandoned IFN for reasons other than transplant or progressive disease (P =.002, 58% vs 72% at 5 years). Quality of life data showed comparable results in both arms for most factors. There is no evidence of benefit for high-dose IFN compared with low-dose for the treatment of CML. Therefore, when IFN is combined with other drugs, low-dose IFN is advised, to minimize toxicity and costs.
