Department of Haematology and Oncology, Leicester Royal Infirmary, Leicester, UK.
Published: June 2005
AI Article Synopsis
Article 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.
Waldenström macroglobulinemia is a rare cancer of plasma cells characterized by the excessive production of immunoglobulin M (IgM). IgM-associated systemic amyloid light chain (AL) amyloidosis is a rare complication of Waldenström macroglobulinemia, characterized by the misfolding of lambda light chains that deposit in various organs, including the heart. We describe a case of progressive nonischemic cardiomyopathy secondary to Waldenström macroglobulinemia and IgM-associated AL amyloidosis that was refractory to medical therapy and highlight the challenges in diagnosis and management.
Amyloidosis is a rare disease that can affect genitourinary organs but the involvement of the prostate has been documented in a limited number of cases. We have reviewed morphologic and immunohistochemical features of prostate biopsies or surgical specimens in which an initial diagnosis of amyloidosis was made. Prostatic amyloidosis was diagnosed in 25 patients, 21 of them were needle biopsies (1.
Light chain deposition disease is a rare condition associated with plasma cell dyscrasia and other lymphoproliferative disorders in which there is overproduction and deposition of non-amyloid light chains in various organs, leading to organ dysfunction. It is well-established that the majority of patients with light chain deposition disease exhibit renal involvement. Although awareness of extrarenal manifestations is increasing, cutaneous involvement has rarely been reported.
A 67-year-old male presented with plaques around the orbit and ecchymosis on the neck and back of hands for 2 years. Physical examination showed seborrheic-keratosis-like plaques around the orbit, ecchymosis on the neck and back of hands, as well as nail dystrophy. Serum λ light chain was positive.
