Vnitr Lek 2012, 58(1):38-43

Light chain deposition disease

V. Ščudla^*, J. Minařík, T. Pika

III. interní nefrologická, revmatologická a endokrinologická klinika Lékařské fakulty UP a FN Olomouc, přednosta prof. MUDr. Vlastimil Ščudla, CSc.

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.

Keywords: light chain deposition disease; light chain κ, λ index; bortezomib; autologous stem cell transplantation

Received: February 24, 2011; Accepted: May 13, 2011; Published: January 1, 2012  Show citation