Light chain deposition disease novel biological insights and…轻链沉积疾病的新颖生物学见解和.pdfVIP

International Journal of Laboratory Hematology The Official journal of the International Society for Laboratory Hematology REVIEW ARTICLE INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY Light chain deposition disease: novel biological insights and treatment advances V. H. JIMENEZ-ZEPEDA Department of Medical Oncology SUMMARY and Hematology, Princess Margaret Hospital, Toronto, ON, Canada Light chain deposition disease (LCDD) is a monoclonal gammopathy characterized by nonamyloid deposition of immunoglobulin light Correspondence: chains in various organs. Most cases present with renal dysfunction, Dr Victor H Jimenez-Zepeda, Department of Medical Oncology a ubiquitous feature of this disease, and in some instances, it may and Hematology, Princess Margaret progress to end-stage renal disease. Unfortunately, until now, no Hospital, Suite 7-702, Toronto, ON, standard treatment has been established. The use of alkylating agents Canada M5G 2M9. Tel.: +1 416 946 4501; and steroids has been extensively reported. However, conventional Fax: +1 480 301 8387; chemotherapy response is generally limited with minor effects on E-mail: victor.zepeda@uhn.ca kidney function. The use of novel agents such as bortezomib has shown a more rapid response with a dramatically important reduction doi:10.1111/j.1751-553X.2012.01419.x of light chains in serum and/or urine in small series of cases. Received 14 December 2011; Furthermore, autologous stem cell transplantat