Light chain deposition disease
Authors:
V. Ščudla; J. Minařík; T. Pika
Authors‘ workplace:
III. interní nefrologická, revmatologická a endokrinologická klinika Lékařské fakulty UP a FN Olomouc, přednosta prof. MUDr. Vlastimil Ščudla, CSc.
Published in:
Vnitř Lék 2012; 58(1): 38-43
Category:
Reviews
Overview
The aim of the study is to put forward the recent knowledge about a relatively rare clinical condition caused by the deposition of immunoglobulin light chains κ or λ into the parenchyme of kidneys and other vital organs, leading to a progressive loss of their function with terminal organ failure. The paper focuses on the etiopathogenesis of light chain deposition disease, and the differentiation of idiopatic form of the disease from multiple myeloma associated conditions and other B lymphoproliferative disorders. We concentrate on the issue of clinical manifestation, contemporary diagnostic possibilities and differential diagnosis of the disease. Finally, we summarize recent therapeutic approaches using chemo-immunotherapy (bortezomib) and high-dosed chemotherapy with support of autologous peripheral stem cell transplantation that lead to a substantial improvement of the prognosis of this prognostically unfavorable disorder.
Key words:
light chain deposition disease – light chain κ/λ index – bortezomib – autologous stem cell transplantation
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Diabetology Endocrinology Internal medicineArticle was published in
Internal Medicine
2012 Issue 1
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