Vnitr Lek 2014, 60(10):821-826

Bortezomib-based therapy in patients with light chain deposition disease

Jiří Minařík1,*, Tomáš Tichý2, Tomáš Pika1, Jaroslav Bačovský1, Dagmar Adamová3, Karel Srovnalík4, Karel Krejčí1, Josef Zadražil1, Vlastimil Ščudla1
1 III. interní klinika - nefrologická, revmatologická, endokrinologická LF UP a FN Olomouc, přednosta prof. MUDr. Josef Zadražil, CSc.
2 Ústav klinické a molekulární patologie LF UP a FN Olomouc, přednosta prof. MUDr. Zdeněk Kolář, CSc.
3 Hematologicko-transfuzní oddělení Slezské nemocnice Opava, primářka MUDr. Dagmar Adamová
4 Hematologické a transfuzní oddělení Nemocnice Vsetín, primář MUDr. Karel Srovnalík

Light chain deposition disease (LCDD) is a rare systemic condition caused by monoclonal proliferation of terminally differentiated B-lymphocytes with production of free light chains and their deposition in kidneys or other organs. The aim of our study is to show the pitfalls of the diagnostics, and to demonstrate the effect of bortezomib-based therapy on a series of 4 patients with LCDD, from the point of hematological and organ therapeutic response. We include that bortezomib based treatment provides rapid and effective hematological response. It is, however, often accompanied by adverse events, especially within intensive treatment schedules. The most serious adverse effects includes peripheral neuropathy, which might be dose or treatment-limiting. Less intensive regimens ("bortezomib weekly") suggest an alternative with expectation of lower incidence of adverse effects. Autologous stem cell transplantation is a recommended and relatively safe approach in convenient candidates. Organ response is significantly delayed after hematological response, and organ damage by light chain deposits might not be fully reversible.

Keywords: autologous stem cell transplantation; bortezomib; hematological treatment response; light chain deposition disease; organ treatment response

Received: May 2, 2014; Accepted: June 23, 2014; Published: October 1, 2014  Show citation

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Minařík J, Tichý T, Pika T, Bačovský J, Adamová D, Srovnalík K, et al.. Bortezomib-based therapy in patients with light chain deposition disease. Vnitr Lek. 2014;60(10):821-826.
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